Top 5 Deathbed Regrets

People grow a lot when they are faced with their own mortality. I learnt never to underestimate someone’s capacity for growth. Some changes were phenomenal. Each experienced a variety of emotions, as expected, denial, fear, anger, remorse, more denial and eventually acceptance. Every single patient found their peace before they departed though, every one of them. When questioned about any regrets they had or anything they would do differently, common themes surfaced again and again. Here are the most common five: 1. I wish I’d had the courage to live a life true to myself, not the life others expected of me. This was the most common regret of all. When people realise that their life is almost over and look back clearly on it, it is easy to see how many dreams have gone unfulfilled. Most people had not honoured even a half of their dreams and had to die knowing that it was due to choices they had made, or not made. It is very important to try and honour at least some of your dreams along the way. From the moment that you lose your health, it is too late. Health brings a freedom very few realise, until they no longer have it. 2. I wish I didn’t work so hard. This came from every male patient that I nursed. They missed their children’s youth and their partner’s companionship. Women also spoke of this regret. But as most were from an older generation, many of the female patients had not been breadwinners. All of the men I nursed deeply regretted spending so much of their lives on the treadmill of a work existence. By simplifying your lifestyle and making conscious choices along the way, it is possible to not need the income that you think you do. And by creating more space in your life, you become happier and more open to new opportunities, ones more suited to your new lifestyle. 3. I wish I’d had the courage to express my feelings. Many people suppressed their feelings in order to keep peace with others. As a result, they settled for a mediocre existence and never became who they were truly capable of becoming. Many developed illnesses relating to the bitterness and resentment they carried as a result. We cannot control the reactions of others. However, although people may initially react when you change the way you are by speaking honestly, in the end it raises the relationship to a whole new and healthier level. Either that or it releases the unhealthy relationship from your life. Either way, you win. 4. I wish I had stayed in touch with my friends. Often they would not truly realise the full benefits of old friends until their dying weeks and it was not always possible to track them down. Many had become so caught up in their own lives that they had let golden friendships slip by over the years. There were many deep regrets about not giving friendships the time and effort that they deserved. Everyone misses their friends when they are dying. It is common for anyone in a busy lifestyle to let friendships slip. But when you are faced with your approaching death, the physical details of life fall away. People do want to get their financial affairs in order if possible. But it is not money or status that holds the true importance for them. They want to get things in order more for the benefit of those they love. Usually though, they are too ill and weary to ever manage this task. It is all comes down to love and relationships in the end. That is all that remains in the final weeks, love and relationships. 5. I wish that I had let myself be happier. This is a surprisingly common one. Many did not realise until the end that happiness is a choice. They had stayed stuck in old patterns and habits. The so-called ‘comfort’ of familiarity overflowed into their emotions, as well as their physical lives. Fear of change had them pretending to others, and to their selves, that they were content. When deep within, they longed to laugh properly and have silliness in their life again. When you are on your deathbed, what others think of you is a long way from your mind. How wonderful to be able to let go and smile again, long before you are dying. Life is a choice. It is YOUR life. Choose consciously, choose wisely, choose honestly. Choose happiness. Credits: This article first appeared on inspirationandchai.com via Real Farmacy

MCTD- mixed connective / CREST

Mixed Connective Tissue Disorder

CREST
Calcinosis
Raynaud's
Esophageal
Sclerdactyl
Teneglascia

http://emedicine.medscape.com/article/1064663-clinical#showall

http://www.merckmanuals.com/home/bone_joint_and_muscle_disorders/autoimmune_disorders_of_connective_tissue/systemic_sclerosis.html

Put me on the front porch...

with a cocktail & I'll tell you stories past sunset! That's how we do it in the south!  Call me crazy but, I come by it honest ~ I'm just willing to share a little more than others.

Which is unusual when there is supposed to be so much shame attached to our skeletons, that we keep them crammed in the back of the closet: well hidden!

It must be the tribal talking gene that came along in my DNA basket of goodies.

At this point, I guess I'm just hoping there's a comfy porch or rocking chair with my name on it somewhere with an interesting view, someone who wants to listen & hopefully I'm not a burden.

So I adopted the mantra...
IF YOU FIND YOUR WOUND `
YOU CAN BEGIN TO HEAL
and am willing to take the skeletons out of the closet & dance with them (how very UN-Southern, of me!) ...

I have -by necessity, due to physical ailments- had to start picking at proverbial scabs. I had been focusing on figuring out the signs & symptoms of my condition when it became apparent that all my world's were colliding ....
~ physical manifestations & emotional scaring ~
whoaa the motherload I have uncovered is a conspiracy theorists dream.... Man, this goes deep!

In fact, I still take pause over some of the things I have been enlightened with... Especially when I listen to things I am sharing & how they might be interpreted/heard by the listener of said tale (s).

So with much appreciation and copious amounts of humility I am ever so grateful and thankful to the scant few who took time to listen & show non-judgmental compassion/empathy.  Thanks to their support & understanding I have been able to work my way through some intensive therapies -mental, physical, genetic-!!

Seronegative Uveitis / PubMed

Opthalmologist. Dr Brenda Lee 11/2014

Uveitis in seronegative arthritis.
Authors Wendling D1.
Author information Journal Curr Rheumatol Rep. 2012 Oct;14(5):402-8. doi: 10.1007/s11926-012-0267-1.
Affiliation Abstract

Uveitis is the most frequent extra-articular feature in seronegative arthritis and is part of the classification criteria for spondyloarthritis. Recent studies confirm a prevalence of up to one third of patients with spondyloarthritis and a relationship with HLA-B27 and disease duration. In recent forms of the disease, a relation with infectious episodes, inflammatory bowel disease, and cervical spine involvement has been reported. Many data argue for the implication of the IL23/IL17 pathway in uveitis, already obvious in spondyloarthritis. Studies with NSAIDs, DMARDs, and anti-TNF agents demonstrated the ability of these treatments to reduce the incidence of flares of uveitis in spondyloarthritis. Only a few data are available with other biologic agents. New onset of uveitis during anti-TNF therapy has been reported. The Il-17 blockade may be a new option for treating both uveitis and the underlying rheumatic condition. PMID 22736066 [PubMed - indexed for MEDLINE] Springer: Full text

Dorso-Lumbar junction

The dorso-lumbar junction is a very important limit between areas where vertebrae have very different morphology and therefore function.

Everyone stresses the shape and the orientation of articular faces in the dorsal and lumbar spine.
In the dorsal spine, the instant rotation centres of the articular processes are in the middle of the intervertebral disc, which makes rotation easier.
At the lumbar level, the instant rotation centres project themselves on the spinous process more or less near its extremity.

In the lumbar moving segments there are thus two rotation centres, the first corresponding to discal rotation, the second to rotation of articular facets. Rotation is only possible by a clipping movement inside the disc.

However, the lumbar spine is the privileged site of flexion extension, due to the thickness of the disc being greater at this level. The morphological transition may be only on one vertebra, usually T12, or on two or three.

3yr DX neuro/panama city
BONE CYST T12 - L1 transverse process

AutoImmune Disease (mtDNA dysfunction)

http://arizonaadvancedmedicine.com/autoimmune-diseases/

Eyes & Ears of Auto Immune

http://www.ufrgs.br/imunovet/molecular_immunology/pathohomotissueimmunity.html

Autoimmune diseases They are caused by autoimmune reactions and affect ~ 3-5% of the population with 2/3 of the patients being women. The site of attack is organ- or tissue-specific or more systemic : as the self-antigen(s) is usually expressed in more than one cell type, from an anatomical point of view autoimmune diseases should more properly be enclosed among the multi-organ failures (MOF). Anyway, due to common pathogenesis, I prefer to group them globally among the diseases affecting the immune system : a link to this page is provided in every page regarding involved organs. They can be classified as follows according to the main targeted organ(s) / apparatus(es) : sense organs eye autoimmune uveitis Aetiology : 5 < RR < 15 in individuals with HLA-B27 Pathogenesis : autoimmune anterior uveitis / iridocyclitis IgA nephropathy / Berger mesangial glomerulonephritis spondyloarthropathies (SpA) / spondyloarthritides-enthesoarthritides pauciarticular Still disease lens-induced uveitis / phacogenic uveitis phacoantigenic uveitis / phacoanaphylactic panophthalmitis occurs 2 weeks (required for sensitization) or months after lens capsular rupture (including surgery : expecially severe if patient had undergone extracapsular lens surgery) phacoanaphylaxis : hypersensitivity to the protein of the crystalline lens of the eye, induced by escape of material from the lens capsule phacotoxic uveitis is a low-grade phacoanaphylactic panophthalmitis phacolytic glaucoma : lens proteins leak through the capsule due to hypermature cataract => macrophages obstruct trabeculate, but no lymphocyte infiltration occurs Pathogenesis : autoimmune response to lens protein leaking through the capsule sympathetic ophthalmia (SO) / sympathetic uveitis / migratory ophthalmia Aetiology : 6-7 weeks after traumatic eye lesions intraocular surgery intraocular silicone oil ... in the contralateral eye causing antigen dissequestration : primary or exciting eye : the eye that is primarily injured and from which the influences start which involve the other eye in sympathetic ophthalmia secondary or sympathizing eye : the uninjured eye which becomes secondarily involved in sympathetic ophthalmia Pathogenesis : antibodies directed against retinal photoreceptors and pigment epithelium => bilateral progressive subretinal fibrosis and blindness with multifocal granulomatous chorioretinitis, preservation of the choriocapillaris (thanks to anti-inflammatory products secreted by the retinal pigment epithelium, including TGF-b and cystatin C / retinal pigment epithelial protective protein that is known to suppress the phagocyte generation of superoxide) and retina despite extensive inflammatory cell infiltration in the choroid Therapy : enucleation of the injured eye, otherwise blindness may occur. autoimmune posterior uveitis / choroiditis or panuveitis Aetiology : Behçet syndrome Heerfordt-Waldenström syndrome Wegener's granulomatosis rheumatoid arthritis diabetes mellitus nephropathies uveomeningitis / Vogt-Koyanagi-Harada (VKH) syndrome Aetiology : HLA-DR4, HLA-DRw53, and HLA-Bw54 Pathogenesis : Ab against the outer segments of photoreceptors and Muller cells Symptoms & signs : severe headache, vertigo, nausea and vomiting, very deep pain in the eyes => uveitis (first one eye and in a couple of weeks the other eye may become affected) with preservation of the choriocapillaris (thanks to anti-inflammatory products secreted by the retinal pigment epithelium, including TGF-b and cystatin C / retinal pigment epithelial protective protein that is known to suppress the phagocyte generation of superoxide) and retina (despite extensive inflammatory cell infiltration in the choroid) => retinal detachment => rapid vision loss, drowsiness, alopecia areata, autoimmune vitiligo, poliosis, hearing loss, facial nerve palsies, rigidity, walking (gait) disturbance Prognosis : after treatment sight and hearing usually return. However, there may be some permanent problems; hair loss with associated loss of color of the hair, eyelashes, and skin may remain. Lasting visual effects may include the development of secondary glaucoma and cataract. Experimental models : experimental autoimmune uveoretinitis (EAU) is a T cell mediated disease of the eye induced by immunization with S-arrestin / S antigen or interphotoreceptor retinoid-binding protein (IRBP). Mooren's ulcer Epidemiology : in elderly individuals Aetiology : trauma infections HHV-1 / HSV-1 HCV Salmonella spp. parasites Symptoms & signs : chronic serpiginous ulceration of cornea central to the most obvious crescent of epithelial defect and stromal melting developing in the corneal periphery, typically progressive centrally, centrifugally, and posteriorly, sometimes progressing to full corneal thickness and perforation. Therapy & prognosis : benign variant : unilateral form which often responds to intensive topical steroid therapy and/or conjunctival resection, malignant variant : bilateral form which progresses despite all attempted local treatments, and responds only to systemic immunosuppresssive chemotherapy. Web resources : Ocular immunology at Massachusetts Eye and Ear Infirmary ear Meniere's or Ménière disease / autoimmune inner ear disease (AIED) / labyrinthine vertigo / recurrent aural vertigo Epidemiology : prevalence 0.4% in USA, onset after age 50. Ménière’s disease, first described in 1861, is idiopathic endolymphatic hydropsref (excess fluid in the inner ear, first revealed through temporal bone studies in 1938), causing deafness, tinnitus, and vertigo. The median time of onset is the fourth decade; Ménière’s disease is rare in childrenref1, ref2, ref2. It is also probably underdiagnosed. Children who complain of dizziness tend to be overlookedref, perhaps partly because they have difficulty describing it, and because vaguely defined “dizziness” has many possible causes. Therefore, children with Ménière’s disease often present with vomiting—which can easily be misdiagnosed as cyclic vomiting or migraine (including abdominal migraine). Hearing loss sometimes develops after onset of the diseaseref1, ref2. Audiometry can be useful in identifying the cause of unexplained vomiting or “dizziness”; regrettably, it is not always widely availableref. Pathogenesis : autoantibodies against COCH5B2, stress and emotional disturbancesref seem to contribute to attacks. Elevation of plasma vasopressin levels (probably as a result of stress) may present as a matter of consequence, but susceptibility of the V2R-overexpressed and cAMP-hypersensitized inner ear to plasma vasopressin elevation might be essential as the basis of this diseaseref Symptoms & signs : unilateral (90%) or bilateral (10%) episodic turning or whirling vertigo (absent if endolymphatic distention is limited to the cochlea) that may lead to nausea and vomiting, and even prostration, tinnitus (varying from a whistle to a roar), and deafness. The onset of symptoms is insidious, usually with a sensation of dullness or fullness in the ear, and an initial fluctuation in hearing of 10 to 30 dB, usually in the low tones. The hearing improves somewhat between attacks, but it continues to deteriorate as time goes on. There may be increased sensitivity to sound, or music may sound distorted. Any head movement aggravates the condition, with the vertigo lasting several hours. Some patients can have fleeting attacks lasting several minutes, and still others have attacks lasting a week or longer and may take months to regain normal equilibrium. Laboratory examinations : spontaneous nystagmus, usually rotary and often direction-changing, and a direction-fixed, positional nystagmus are the most common findings no nystagmus at rest; reactive nystagmus elicted from caloric reflex testing (with warm and cold water) in the contralateral ear. Aside from the hearing loss, Meniere's patients frequently have recruitment and diplacusis, low threshold discomfort, and low discrimination scores. Tone decay and a type II Bekesy are present. Pure-tone audiometry showed mild sensorineural hearing loss in the affected ear glycerin test, where a patient ingests 1.5 gm/kg body weight of glycerol mixed with equal parts of normal saline and a few drops of lemon juice. Audiograms are taken immediately and at 1, 2, and 3 hr after ingestion. A positive test is said to be an improvement in hearing of 15 dB in any one frequency from 250 to 4000 Hz or 12% improvement in the discriminating score. Differential diagnosis : Ménière-like syndromes Therapyref : neutral-ash, sodium-free diet, supplemented with diuretics (isosorbide, an osmotic diuretic), regimen of bed rest, no smoking, plus inhalation of 5% CO2 + 95% O2 for 30' q.i.d. and 2.75 mg of histamine diphosphate in 250 cc of lactated Ringer's solution I.V. b.i.d. Other drugs, given individually, that are reported to be effective for an acute attack are 1/150 grain atropine I.V., diazepam 10 mgm I.V., and fentanyl citrate + droperidol, which must be administered in the hospital or by an anesthesiologist. Vasodilators are usually ineffective in Meniere's, as are the antivertiginous drugs. office-based intratympanic inner ear steroid perfusion (ITPs) treatmentref Meniett pulse generatorref vestibular ablation chemical intratympanic high-concentration gentamycinref1, ref2 destructive labyrinthotomy endolymphatic sac to mastoid shuntsref provided improvement in major spells of vertigo in 77% of patients at 24 months after surgery. Revision surgery provided improvement in 65% of cases. Results of revision surgery were better in those patients who developed recurrent symptoms more than 24 months after their original procedure compared with those of patients who failed treatment earlier triple semicircular canal occlusion (TSCO)ref low-level laser therapy (LLLT)ref Web resources : Autoimmune inner ear disease by Timothy C.Hain ulcerative colitis relapsing polychondritis systemic lupus erythematosus polyarteritis nodosa Cogan’s syndrome Wegener’s granulomatosis

Post infection arthritis

http://m.crisbertcualteros.page.tl/Postinfectious-Arthritis-and-Related-Conditions.htm

Chapter 147 - Postinfectious Arthritis and Related Conditions

Michael L. Miller
James T. Cassidy

Infections have been associated with arthritis during their course and as a postinfectious reaction observed several weeks or months afterward. Although certain infectious organisms have been suspected but not proved to trigger juvenile rheumatoid arthritis (JRA), other agents are often associated with a transient arthritis that does not satisfy the classification criteria for JRA. Reactive arthritis follows an infection outside the joint, particularly the gastrointestinal or genitourinary tract. The course of reactive arthritis is variable and may progress to chronic spondyloarthropathy (see Chapter 146 ). Postinfectious arthritis implies arthritis that follows infections that are usually viral in origin, with shorter duration than reactive arthritis.
Pathogenesis.
Reactive arthritis may follow enteric infection with non-typhoidal Salmonella, Shigella, Yersinia enterocolitica, Campylobacter jejuni, Cryptosporidium parvum, or Giardia intestinalis, or genitourinary tract infection with Chlamydia trachomatis. Reactive arthritis may represent an autoimmune response involving T lymphocytes that cross-react to antigens in joints (molecular mimicry). A study of synovial fluid from patients with reactive arthritis suggested that T cells may be more engaged in promoting inflammation than in eliminating bacteria through cytotoxic mechanisms. However, the origin of these abnormal cells may be from outside the joint. Another possible mechanism is lymphocytic reactivity to bacterial DNA found in synovium, perhaps as a by-product of otherwise successfully cleared infection. One study of joint fluid from patients with reactive arthritis, using polymerase chain reaction amplification, demonstrated bacterial DNA. However, a relationship to specific clinical characteristics was not found.
Several viruses (rubella, varicella-zoster, herpes simplex, and cytomegalovirus) have been isolated from the joint space. Antigens from other viruses (hepatitis B, adenovirus 7) have been identified in immune complexes from joint tissue. Postinfectious arthritis following viral infections appears to involve the deposition in joints of immune complexes containing viral antigens.
Certain HLA types may predispose to development of reactive arthritis, possibly by triggering autoreactive T lymphocytes. Adolescents and adults with reactive arthritis following enteric infections have shown persistent gut inflammation even after resolution of gastrointestinal manifestations, particularly in HLA-B27-positive individuals. Uveitis complicating reactive arthritis to Y. enteritis has also been associated with HLA-B27. Some children, often HLA-B27 positive, with reactive arthritis eventually develop spondyloarthropathy, further suggesting that HLA type predisposes to reactive arthritis.
Clinical Manifestations.
Bacterial enteritis caused by Shigella, Salmonella, Yersinia, and Campylobacter can be followed within days to several weeks by the development of arthritis and sometimes enthesitis, or inflammation of the muscular or tendinous attachment to bone, in a syndrome similar to and overlapping spondyloarthropathy (see Chapter 146 ). Although the erythrocyte sedimentation rate (ESR) may be elevated, fever and leukocytosis are often absent. Urethritis and conjunctivitis develop occasionally. Postinfectious arthritis following less apparent illness, such as viral upper respiratory tract infections, may precede arthralgia and arthritis by 1–2 mo. Symptoms of arthralgia and joint swelling are transient, usually lasting less than 6 wk.
Certain viruses associated with arthritis ( Box 147–1 ) may result in particular patterns of joint involvement. Rubella and hepatitis B virus typically affect the small joints, and mumps and varicella often involve large joints, especially the knees. The hepatitis B arthritis-dermatitis syndrome is characterized by rash and arthritis resembling serum sickness. Rubella-associated arthropathy follows natural rubella infection and, infrequently, rubella immunization. It typically occurs in young women, with increased incidence with advancing age, and is uncommon in preadolescent children and in males. Arthralgias of the knees and hands usually begin within 7 days of onset of the rash or 10–28 days after immunization. Parvovirus B19, responsible for erythema infectiosum (fifth disease), can cause arthralgia, symmetric joint swelling, and morning stiffness in adults, particularly women, and less frequently in children. Arthritis occurs occasionally during cytomegalovirus infection and may occur during varicella infections, and is rare after Epstein-Barr virus infection. Varicella may also be complicated by suppurative arthritis, usually due to group A Streptococcus.
Arthritis has been reported in children with severe truncal acne, usually in male adolescents. Patients often have fever and superficial infection of skin lesions. Recurrent episodes may be associated with myopathy and may last for several months. Infective endocarditis can be associated with arthralgia, arthritis, or signs suggestive of vasculitis, such as Osler nodes, Janeway lesions, and Roth spots. Arthritis also occurs in children with Mycoplasma pneumoniae infections.
Poststreptococcal arthritis may follow infection with either group A or group G Streptococcus. Because valvular lesions have been documented by echocardiography after the acute illness in some of these children, some clinicians consider this to be an incomplete form of acute rheumatic fever (see Chapter 168.1 ). Certain HLA-DRB1 types may predispose children to develop either poststreptococcal arthritis or classic rheumatic fever. Poststreptococcal arthritis is pauciarticular, may affect the small and large joints, and persists for months, compared with the

Box 147-1. Viruses Associated with Arthritis
Togaviruses

Rubivirus

Rubella

Alphaviruses

Ross River

Chikungunya

O'nyong-nyong

Mayaro

Sindbis

Ockelbo

Pogosta

Parvovirus

B19

Hepadnavirus

Hepatitis B

Adenoviruses

Adenovirus 7

Herpesviruses

Herpes simplex

Cytomegalovirus

Epstein-Barr

Varicella-zoster

Paramyxoviruses

Mumps

Enteroviruses

Echovirus

Coxsackievirus B

Orthopoxviruses

Variola virus (smallpox)

Vaccinia virus

Adapted from Cassidy JT, Petty RE: Arthritis related to infection. In Textbook of Pediatric Rheumatology. Philadelphia, WB Saunders, 1995, p 503.

 

809
typical course of migratory polyarthritis of rheumatic fever. The symptoms are usually mild and tend to resolve completely. 
Transient synovitis (toxic synovitis) typically affects the hip joints, often after an upper respiratory tract infection. Boys from 3–10 yr of age are most commonly affected and have complaints of pain in the hip, thigh, or knee. The ESR and white blood cell count are usually normal. Radiologic or ultrasound examination may reveal widening of the joint space of the hip. Aspiration of the hip may be necessary to exclude suppurative arthritis. The cause of this relatively common syndrome is not known, but it is presumed to be a viral or postinfectious arthritis.
Diagnosis.
The diagnosis of reactive postinfectious arthritis is usually established by exclusion only after the arthritis has resolved. Acute arthritis affecting a single joint suggests suppurative arthritis; osteomyelitis may cause joint pain but is associated more often with focal bone pain over the site of infection. Arthritis associated with gastrointestinal symptoms or elevated liver function test results may be caused by infectious or autoimmune hepatitis. Arthritis or spondylarthritis may occur in some children with inflammatory bowel disease, either Crohn disease or chronic ulcerative colitis (see Chapter 317 ). When two or more blood cell lines show a progressive decrease in a child with arthritis, parvovirus infection, macrophage activation syndrome, and leukemia should be considered. Persistent arthritis suggests the possibility of rheumatic disease, including JRA, spondyloarthropathy, and systemic lupus erythematosus.
Treatment.
No specific treatment is necessary for reactive arthritis, except for relief of pain and the functional limitations of arthritis with nonsteroidal anti-inflammatory agents. If swelling or arthralgia recurs, further evaluation may be necessary to preclude active infection or evolving rheumatic disease, such as spondyloarthropathy, JRA, or systemic lupus erythematosus.
Complications and Prognosis.
Postinfectious arthritis following viral infections usually resolves without complications unless it is part of a severe viral infection affecting other organs, as with encephalomyelitis. Reactive arthritis, especially after bacterial enteric infection or genitourinary tract infection with C. trachomatis, has the potential for evolving to a chronic arthritis and spondyloarthropathy (see Chapter 146 ). Children with reactive arthritis following enteric infections occasionally develop inflammatory bowel disease months to years after onset. Both uveitis and carditis have been reported in some children diagnosed with reactive arthritis.

Pub-med: b27 + mesenychemal (Josh)

Microbial antigens mediate HLA-B27 diseases via TLRs. Authors Pöllänen R1, Sillat T, Pajarinen J, Levón J, Kaivosoja E, Konttinen YT. Author information Journal J Autoimmun. 2009 May-Jun;32(3-4):172-7. doi: 10.1016/j.jaut.2009.02.010. Epub 2009 Mar 18.

Affiliation Abstract
HLA-B27 positive individuals are predisposed to reactive arthritis developing 1-3 weeks after urogenital and gastrointestinal infections. Also ankylosing spondylitis (AS) associates strongly to HLA-B27, but no specific infection, Klebsiella pneumoniae excluded, has been linked to it.
Before the discovery of its HLA-B27 association there were many reports suggesting a link between chronic prostatitis in men or pelvic inflammatory disease in women and AS. They have since been forgotten although HLA-B27 did not help to understand, why this disease has an axial and ascending nature.
It is proposed that the urogenital organs form a source of damage (or danger)-associated molecular patterns (DAMPs), either exogenous pathogen-associated molecular patterns (PAMPs) from microbes or endogenous alarmins, such as uric acid, released from necrotic cells or urate deposits. DAMPs are slowly seeded from low-down upwards via the pelvic and spinal lymphatic pathways.
They reach Toll-like receptors (TLRs) in their target mesenchymal stem cells, which are stimulated to ectopic enchondral bone formation leading to syndesmophytes and bamboo spine. At the same time inflammatory cytokines induce secondary osteoporosis of the spine.
This new paradigm places microbes, HLA-B27 and TLRs in the pathogenic centre stage, but without pinpointing any (one) specific pathogen; instead, shared microbial patterns are indicated. PMID 19299108 [PubMed - indexed for MEDLINE] Elsevier Science: Full text
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4110540/

Mesenchymal hamartoma

After hemangiomas, mesenchymal hamartoma of the liver (MHL) is the second commonest benign hepatic tumor in childhood, but these tumors are relatively rare. Most MHLs are large benign multicystic masses that present in the first 2 years of life[28]. Prenatal diagnosis of MHL has been reported, most often in the last trimester of pregnancy and it may be a cause of severe hydrops. An early prenatal diagnosis and a subsequent follow-up could help to establish the best time for delivery. Fetal intervention may be beneficial in selected cases. If the fetus is becoming hydropic, early delivery or fetal treatment (particularly if the tumor is composed of a few large cysts) should be considered. Most affected fetuses have been successfully delivered vaginally[29].

Postnatal presentation is more common with abdominal distension and/or an upper abdominal mass. Liver function tests are usually normal. AFP is occasionally elevated though not to the degree that occurs in hepatoblastoma. About 75% of MHL occur in the right lobe of the liver. In the newborn, the tumor may expand rapidly and cause life-threatening abdominal distension with respiratory distress[30]. Diagnostic imaging studies demonstrate a multiloculated cystic tumor with a variable amount of solid tissue[31]. This may be seen in undifferentiated embryonal sarcoma of the liver (UESL), but rarely in hepatoblastoma. Intratumor calcification, which can be frequently detected in hepatoblastoma or hepatic hemangioma, has been reported very rarely for a MHL.

Ultrasound demonstrates the presence of thin mobile septate and/or round hyperechoic parietal nodules within the cysts, but rarely containing debris. The hepatic architecture is normal beyond the outer rim of compressed liver. On CT-contrast the solid component, septate, and the peripheral rim may enhance. On MRI, MHL has a low signal intensity on T1-weighted magnetic resonance sequences and a variable signal intensity on T2-weighted sequences (Figure ​(Figure44)[32]. In most patients, the diagnosis of MHL is suggested by imaging and confirmed by histological examination of the resected specimen. If radiological diagnosis is not clear, a percutaneous or open tumor biopsy can be performed[33].

*see preceding link for image

Figure 4

Abdominal computed tomography and magnetic resonance imaging. A: Abdominal computed tomography-contrast shows enhancement of the solid component, septate, and the peripheral rim; B: Abdominal magnetic resonance imaging-contrast shows a high signal intensity ...

Although a laparoscopic or open surgical biopsy is considered by some authors, SIOPEL (International Childhood Liver Tumor Study Group of the International Society of Paediatric Oncology) currently recommends image-guided coaxial plugged needle biopsy for liver tumors (obtaining numerous cores)[34]. Fine needle aspiration cytology is of limited value because hepatoblastoma or a malignant mesenchymal tumor is difficult to exclude. MHL has been considered a focal tumor, but small satellite lesions at the tumor margin have been described, which could explain tumor recurrence after apparent complete resection. Clinical and histological evidence suggest that UESL can develop within a preexisting MHL[28,30]. Both tumors share similar features on gross pathology (cystic and solid components, sometimes pedunculated), histology (mesenchymal elements with benign bile duct epithelial structures), and immunohistochemistry (positive staining for vimentin, desmin, a-1-antitrypsin, actin, cytokeratins). Flow cytometry studies have shown that although most MHLs are diploid, some are aneuploid and cytogenetic studies have demonstrated a balanced translocation involving the same breakpoint on chromosome 19 (band 19q13.4) and chromosome 11. These abnormalities have been found in both, UESL and MHL[28].

The management of MHL remains still controversial. MHL has the potential to involute spontaneously, especially for those tumors with a prominent angiomatous component. Nonoperative management may be appropriate in selected cases (e.g., infants with a biopsy-proven MHL and a prominent vascular component). Percutaneous aspiration or drainage of larger cysts may temporarily control tumor size in life-threatening lesions and it may helpful for the definitive surgical resection. The standard of care is complete resection with the goal of achieving negative margins to avoid the risks of local recurrence and long-term malignant transformation. Enucleation may be adequate in case of very large tumors that replace most of the liver parenchyma. Liver infiltration by MHL is rarely seen and a surgical plain is normally found for resection (Figure ​(Figure5).5). Pedunculated lesions are amenable to laparoscopic resection. Marsupialization or partial resection are suboptimal because of the risk of tumor recurrence. Liver transplantation can be considered for unresectable tumors[28,30].

* see preceding link for original article & images

Figure 5

Surgical resection of mesenchymal hamartoma of the liver.

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http://www.ncbi.nlm.nih.gov/pubmed/16365828

Mesenchymalhamartoma of the liver in adulthood: immunohistochemical profiles, clinical and histopathological features in two patients.

    Mesenchymal hamartoma is an uncommon cystic mass of the liver which occurs primarily in children. There are a few reports of its occurrence in adulthood. Here, we present two cases infemale patients, 54 and 51 years old. Radiological examinations in both patients showed multiple cystic lesions in the liver. Surgically, totalcystectomy was performed in the first patient, while an unroofing procedure was done in the second patient (due to misdiagnosis of the lesion as a simple cyst of the liver). On microscopic examinations of the lesion in each patient, a multilocular cyst was observed, lined by flattenedepithelium and surrounded by a mesenchymal component composed of mature connective tissue, arterial and venous vascular structures, peripheral nerve bundles, and ductal structures. An immunohistochemical panel consisting ofdesmin, smooth-muscle actin, S-100, vimentin, CD34, carcinoembryonic antigen, pancytokeratin, cytokeratin 7, cytokeratin 8, cytokeratin 17, cytokeratin 18, cytokeratin 19, and cytokeratin 20 was applied to paraffin sections. Immunoreactivity for cytokeratin 7 and cytokeratin 19 was observed in cystic epitheliumand ductal structures. Focal and patchy desminimmunoreactivity was observed in connective tissue. S-100 was positive only in peripheral nerve bundles. In conclusion, mesenchymal hamartomaof the liver in adulthood is a localized tumoral abnormality that precedes birth, and which has delayed clinical presentation. These lesions seems to be related to a maturation process. During this period of maturation, immature edematous stroma rich in mucopolysaccharides may convert to mature paucicellular hyalinizedconnective tissue. This maturation process may be also related to loss of premalignant potential of these tumors.

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Opthalmologist sees cells 11/3

Follow up 1 week RE; iritis/uevitis.

Interesting video on DNA Pinterest board...

 The uveal tract is the highly vascular and densely pigmented layer of the eyeball, lying between the sclera (superficial to it) and the retina (deep to it).

The anterior, visible portion is the iris. This extends back into the ciliary body (at the level of the lens) and then extends round to the posterior pole, where it is known as the choroid.

Uveitis means inflammation at any point of the uveal tract, with or without inflammation of neighbouring structures (eg, the retina or vitreous).^[1]

About 50% of patients with acute anterior uveitis are HLA-B27 positive. Nongranulomatous disease can be associated with:

• Sacroiliitis
• Ankylosing spondylitis
• Reiter's syndrome
• Psoriasis
• Inflammatory bowel disease
• Sarcoidosis
• Behçet's disease
• Juvenile idiopathic arthritis

The only group of patients currently screened for uveitis is children with juvenile idiopathic arthritis.

Management^[2][7] 

If uveitis is suspected, immediate specialist referral is appropriate to confirm diagnosis and facilitate treatment. Delay in appropriate management can lead to the development of significant complications and irreversible loss of vision. Exact treatment is determined by the type of uveitis (anterior versus posterior), whether the uveitis is secondary to an infection or not and whether it is likely to threaten sight. All patients with anterior disease and most with intermediate or posterior disease require treatment. For patients with systemic disease, a collaborative approach between ophthalmologists and other specialities is important.

Refer people with suspected uveitis for an assessment by an ophthalmologist within 24 hours. Also refer people who have had uveitis before and feel the symptoms coming on before the signs are present. Do not initiate treatment for recurrent uveitis in primary care, unless asked to do so by an ophthalmologist.

• Corticosteroids are used to reduce inflammation and prevent adhesions in the eye. They may be given topically, orally, intravenously, intramuscularly, or by peri-ocular or intra-ocular injection, depending on the severity of the uveitis. Corticosteroids are reduced slowly, as withdrawing them too quickly may lead to rebound inflammation.
• A cycloplegic-mydriatic drug (for example, cyclopentolate 1% or atropine 1%) is also given to paralyse the ciliary body. This relieves pain and prevents adhesions between the iris and lens.
• Infectious uveitis (bacterial, viral, fungal, or parasitic) is treated with an appropriate antimicrobial drug as well as corticosteroids and cycloplegics.
• People with severe or chronic uveitis may also be given systemic (non-corticosteroid) immunosuppressive drugs, laser phototherapy, or cryotherapy, or have the vitreous removed surgically (vitrectomy).

Adjunctive therapy

• Secondary causes should be treated as appropriate. Where there is an infection, this needs to be treated as a priority.^[2] (See the separate article on Chorioretinal Inflammation for more details about treatment of ocular toxoplasmosis.)
• Ongoing research is looking at the use of immune modulators such as tumour necrosis factor alpha-blockers (eg, etanercept, infliximab) and the interleukin-2 receptor blockers. A number of studies on less invasive sustained ocular drug delivery systems, including episcleral implants, nanospheres, and cyclodextrin particles are being conducted.^[9] Interferon alfa may also have potential in treating refractory sight-threatening uveitis from a variety of causes.^[10]
• Azathioprine has been found to be useful in steroid-resistant autoimmune uveitis.^[11] Ciclosporin and tacrolimus have also been investigated and have been shown to be associated with control of ocular inflammation and preservation or restoration of sight. However, currently immunosuppressive treatment has not been licensed for uveitis.
• There may be a role for the family of anti-vascular endothelial growth factor drugs (eg, ranibizumab, Lucentis®, which is currently used for 'wet' age-related macular degeneration) in managing chronic, non-infective uveitis but these drugs are not yet licensed for this use.^[2]Furthermore, any intravitreal injection carries a small inherent risk of complications (including sight loss) in itself.

Surgery

Surgery is only considered in a small proportion of patients with severe or intractable disease or where a diagnostic vitreous sample is required (eg, to diagnose infection or malignancy). Other reasons for surgery include removal of a secondary cataract or for preservation of vision in uveitic glaucoma.^[2]

Monitoring the disease^[3]

• This is almost entirely by clinical examination but some investigations provide a useful adjunct.
• Optical coherence tomography (OCT) is very helpful in looking for macular causes of worsening vision, particularly where slit-lamp view is not good.
• Fluorescein angiography is helpful where retinal vascular involvement needs to be assessed. Visual fields are also helpful for monitoring optic nerve damage.

Complications

Visual loss as a result of:^[2]

• Cystoid macular oedema (swelling of the macula) - this is the leading cause of visual loss in the UK along with secondary cataract formation.
• Secondary cataract.
• Acute rise in intraocular pressure, with or without glaucoma:
  • This may occur either as a direct consequence of the inflammatory process or secondary to steroid treatment.
  • Some patients, especially those with a history of glaucoma, are prone to developing high intraocular pressure as a result of steroid treatment and require treatment to reduce the intraocular pressure. Treatment to reduce intraocular pressure can be stopped when the steroid treatment is stopped if the patient doesn't have pre-existing glaucoma.
• Vitreous opacities (inflammatory debris or haemorrhage).
• Retinal detachment.
• Neovascularisation of the retina, optic nerve, or iris.
• Macular ischaemia, vascular occlusions and optic neuropathy - can be complications of posterior uveitis.

Posterior synechiae are a common complication of anterior uveitis; if numerous they can cause blockage of the aqueous flow, leading to a rise in intra-ocular pressure, and can complicate cataract operations

Prognosis

• Anterior uveitis is sometimes a self-limiting condition.
• With prompt and effective treatment, there is usually a good visual outcome (a study found that 91% of these patients retain normal vision).^[2]
• The factors which cause spontaneous resolution in some patients and complications in others are unclear.^[12]
• Relapse after a first episode of acute anterior uveitis is probably more common than previously thought - a recent study suggested an incidence of about 24% per person-year, this occurring more commonly in the younger patient age group (18-35 year-olds).^[13]
• The prognosis of chronic granulomatous uveitis depends on the cause and whether the underlying condition is recognised and treated early enough.
• Outcomes have been improved by the use of immune modulators in previously refractory cases.^[14]

http://www.patient.co.uk/doctor/uveitis#nav-6

Going forward with diagnosis

I will continue to explore the Erase AS (ankylosing spondylitis) site...

http://www.eraseas.com/research.htm

Flares (acute symptomatic episodes followed by remission) occur chronically since AS is a life-long disease. Once the early stages of inflammation are behind you, the severity of these flare-ups may subside to a mild or moderate level. Of course the intensity of the disease’s activity varies from person to person without regard to age, race or gender.

The following list includes the range of different symptoms commonly associated with AS. Symptoms are arranged in the general order of occurrence during AS progression.

Sacroiliitis: Inflammation of the sacroiliac joints – where the spine meets the pelvis (SI).

Enthesitis: Inflammation of the enthesis (where the joint capsules, ligaments and tendons attach to the bone). This includes swelling and tenderness along joints like the back, pelvis, chest and heel.Inflammation of the heel can have a serious impact on person’s mobility. Inflammatory spots include the Achilles tendon and plantar fascia.

Spinal Conditions: The repair process following inflammation leads to scarring of the tissue and extra bone formation along the spine. During severe stages of AS, incessant healing and inflammation can cause fusion of the spine and sometimes other joints as the disease progresses. This leads to an increased possibility of spinal fractures since the spine has little flexibility, and bone formed during the fusion is weak. It also causes great discomfort to the person since he or she may be forced into a stooped position – which is thankfully far less common now with treatment advances and information on posture.

Hip and Shoulder Conditions: One-third of the AS population experiences hip and shoulder problems. Hip strain comes on gradually and is often felt in the groin area, but can also be mistakenly felt in the knees or thighs – otherwise known as “referred pain”. This is sometimes misleading during an examination of inflamed areas. Hip discomfort is more common in younger people while shoulder involvement is mild.

Neck Pain: The cervical section of the spine is the most mobile area on the back and stiffness in this area can greatly affect a person’s mobility such as turning the head and looking up and down.

Knee Pain: Although knee pain and stiffness is usually associated with pain in other areas of the body like the hips, about 20 percent of people experience inflammation in the knee capsule as a separate symptom.

Jaw Pain: Approximately ten percent of people have inflammation of the jaw – making it hard to fully open their mouth when chewing.

Chest Pain: Chest pain can imitate cardiac angina – pain caused by deep breathing when the outer lining of the lungs is inflamed. Over time the joints between the ribs and spine, and where the ribs meet the breastbone, develop decreased chest expansion due to scarring of the tissue from inflammation.

Iritis (Inflammation of the eye): Thirty to forty percent of people experience iritis. Symptoms can occur in each eye at different times and include redness, pain, sensitivity to light and blurred vision. See a doctor immediately if you think you have irits.

Heart Conditions: Sometimes a rare chronic inflammation at the base of the heart around the aortic valve will occur. Long-term inflammation at these sites can lead to heart blockage and valve leakage. This problem is seen in fewer than two percent of people with AS and is detectable.

Lung Conditions: Due to chest pain and poor chest wall movement comes the possibility of fibrosis scarring at the top of the lungs. This condition can make it longer for colds and respiratory infections to heal. Smoking is out of the question.

Kidney Conditions: Kidney problems are caused by long-term treatment with nonsteroidal anti-inflammatories. This condition is extremely rare in the U.S.

Neurological Complications: Very rarely, advanced AS patients experience problems from the scarring of nerves at the base of the spine. This can cause urinary retention, loss of bowel control, sexual dysfunction and pain or weakness in the legs. It is necessary to see a neurologist at this point.

Potential Cures
Currently there is no cure for AS – but that just means one hasn’t been discovered yet. There are holistic treatments, medications and daily exercises to reduce and manage the pain. Biologic medicines called Tumor-Necrosis-Factor blockers (TNF-blockers) can potentially slow down or stop the progression of AS. Some remedies may be disappointing and others surprising. Simple strategies like daily exercise and attention to posture make a world of difference. Continue to educate yourself about different treatments, side effects and activity in medical research.

Following their outline ... I have experienced each of these with a focus on right or midline (neck to neuroma/face to foot)

Oct 2014 Dr.Nami Rheumatologist
uspa, mctd, hla-b27 (w/preexisting Dercums/atd) autoimmune driven