Tag Archives: autoimmune

Paraneoplastic pemphigus (PNP), a clinically and immunopathologically distinct mucocutaneous blistering dermatosis, is a severe form of autoimmune multiorgan syndrome generally associated with poor therapeutic outcome and high mortality. This IgG-mediated disease is initiated by an obvious or occult lymphoproliferative disorder in most cases. Clinically severe mucositis, and polymorphic blistering skin eruptions, and histologically acantholysis, keratinocyte necrosis and interface dermatitis are its hallmark features. A 58-year-old female presented with recurrent, severe, recalcitrant stomatitis and widespread erosions/blistering lesions of one-year duration. Treatment with repeated courses of systemic corticosteroids at a peripheral center would provide temporary relief. She also had fever, productive cough, odynophagia and poor oral intake, herpes zoster ophthalmicus, pain in the abdomen, and watery diarrhea. An array of investigations revealed chronic lymphocytic leukemia (CLL), mediastinal and para-aortic lymphadenopathy, bronchiolitis obliterans, and vertebral osteoporosis/fractures. With the diagnosis of CLL-associated PNP she was managed with dexamethasone-cyclophosphamide pulse (DCP) therapy for 3 cycles initially, followed by COP regimen (cyclophosphamide, vincristine, and prednisolone) for 5 cycles. Remission is being maintained with chlorambucil and prednisolone pulse therapy once in 3 weeks with complete resolution of skin lesions and adequate control of CLL.

Full article can be viewed here: http://www.hindawi.com/crim/dm/2012/207126/

 

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Pemphigus and pemphigoid are uncommon dermatological entities in domestic animals and of a presumed autoimmune nature. In one form or another, they

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have been reported in the dog, cat, horse and goat. Although these diseases are considered to be bullous dermatoses, the clinical presentation may vary from ulcerative to exfoliative to proliferative depending on the individual condition. Currently, four variants of pemphigus are recognized (vulgaris, vegetans, foliaceus, erythematosus) and two of pemphigoid (bullous, cicatricial) although cicatricial pemphigoid has not yet been conclusively demonstrated in animals. Diagnosis is based on history, clinical signs, histopathology and immunopathology. Therapy must be immunosuppressive to be effective and is palliative rather than curative.

Full article available here: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1680036/

Puppy-Love-puppies-9460996-1600-1200Pemphigus in Dogs

Pemphigus is the general designation for a group of autoimmune skin diseases involving ulceration and crusting of the skin, as well as the formation of fluid-filled sacs and cysts (vesicles), and pus filled lesions (pustules). Some types of pemphigus can also affect the skin tissue of the gums. An autoimmune disease is characterized by the presence of autoantibodies that are produced by the system, but which act against the body’s healthy cells and tissues — just as white blood cells act against infection. In effect, the body is attacking itself. The severity of the disease depends on how deeply the autoantibody deposits into the skin layers. The hallmark sign of pemphigus is a condition called acantholysis, where the skin cells separate and break down because of tissue-bound antibody deposits in the space between cells.

There are four types of pemphigus that affect dogs: pemphigus foliaceus, pemphigus erythematosus, pemphigus vulgaris, and pemphigus vegetans.

In the disease pemphigus foliaceus, the autoantibodies are deposited in the outermost layers of the epidermis, and blisters form on otherwise healthy skin. Pemphigus erythematosus is fairly common, and is a lot like pemphigus foliaceus, but less afflictive. Pemphigus vulgaris, on the other hand, has deeper, and more severe, ulcers because the autoantibody is deposited deep in the skin. Pemphigus vegetans, which affects only dogs, is the rarest form of pemphigus, and seems to be a gentler version of pemphigus vulgaris, with somewhat milder ulcers.

full article can be found here: http://www.petmd.com/dog/conditions/skin/c_dg_pemphigus?page=show#.UQbd3R3WLXA

 

An Epidemic of Absence: A New Way of Understanding Allergies and Autoimmune Diseases could be co-marketed with the Thomas Rockwell’s children’s classic How to Eat Fried Worms. It begins with the author, Moises Velasquez-Manoff, recounting his border-crossing to Tijuana to infect himself with Necator americanus—hookworms—in an attempt to cure the asthma, hay fever, food allergies, and alopecia that had plagued him since childhood. In the next three hundred pages, the author very cogently explains the idea that led him to willingly infect himself with a parasite known to cause severe diarrhea, anemia, and mental retardation in children.

Velasquez-Manoff marshals the reams of evidence researchers have accumulated to support said concept: the hygiene hypothesis, but with an updated, parasitic twist. The ideas he presents haven’t been accepted by many in the medical community, and there’s little high-quality evidence, in the form of well controlled trials, that exposure to parasites could have positive effects on human health. So, even if the author is thorough, it’s important to keep in mind that the evidence he’s presenting is primarily in the form of correlations.

The Hygiene Hypothesis

A simplistic view of the hygiene hypothesis is that in the absence of something dangerous to fight against—the cholera toxin, for example—immune cells get confused, or bored, and fight against harmless stimuli like dust mites and peanuts instead. But there is a more nuanced view. Our immune systems co-evolved with an enormous community of microbes, and were in fact shaped by them. Many became established, long-term, and vital residents in our guts; the importance, and in fact the very existence, of these commensals has only recently been realized.

Constant exposure to all of these bugs, as a unit, enhanced the regulatory arm of the immune system, modulating responses so that we could tolerate the filthy environment in which we lived while at the same time (hopefully) fighting off those pathogens that posed a mortal threat and not destroying our own bodies in that process. In the martial analogy that is inevitable in discussing immunology, ancient human immune cells that were always surrounded by microbes were like battle-hardened old soldiers who have learned the ability to watch warily when encountering something new, waiting to see whether or not it is dangerous; modern immune cells raised in our hyper-sanitized environment are like new recruits just given their first gun, testy and jumpy at the first hint of a threat and liable to blow up their surroundings in inappropriately directed and outsized force. Experience has not taught them moderation.

Seeing worms everywhere

Yes, he includes autism in the list of modern diseases caused by our out-of-whack immune systems. Along with other cases where immune dysfunction hasn’t been established, like obesity, cardiovascular disease, type 2 diabetes, and cancer.

There are some serious problems with blaming all of these on immune dysfunction, but we’ll focus on a single example: autism. Just as the absence of worms’ mediating effects on our immune system causes some people to have an allergic response to harmless ingested proteins and others to attack their own tissues, the argument goes, chronic inflammation in the womb generates fetuses with autism.

The rest of this article can be read here: http://arstechnica.com/science/2012/10/book-review-an-epidemic-of-absence-takes-on-the-worms-youre-missing/

Abstract

BACKGROUND:

Systematic reviews and meta-analysis are essential tools to accurately and reliably summarize evidence, and can be used as a starting point for developing practice guidelines for the diagnosis and treatment of patients.

AIM:

To estimate the diagnostic accuracy of enzyme-linked immunosorbent assays (ELISA) to detect anti-BP180 and anti-desmoglein 3 (Dsg3) autoantibodies in the diagnosis of autoimmune blistering skin diseases.

METHODS:

A Medline search of English written articles, published between 1994 and 2011, reporting data on the sensitivity and specificity of diagnostic tests was conducted using the following search terms: “BP180 autoantibodies”, “Dsg3 autoantibodies”, and “enzyme linked immunosorbent assay”. The selected articles have been evaluated according to the quality of the statistical methods used to calculate diagnostic accuracy (definition of cutoff value, use of ROC curves, and selection of control cases). The meta-analysis was performed using a summary ROC (SROC) curve and a random-effect model to independently combine sensitivity and specificity across studies.

RESULTS:

The search yielded 69 publications on BP180 autoantibodies and 178 on Dsg3 autoantibodies. A total of 30 studies met the inclusion criteria: 17 provided data on the assays to detect autoantibodies to BP180 in a sample of 583 patients with bullous pemphigoid (BP), while 13 studies provided data on the assays to search for anti-Dsg3 autoantibodies in a sample of 1058 patients with pemphigus vulgaris (PV). The 17 studies on BP180 autoantibodies yielded a pooled sensitivity of 0.87 (95% confidence interval (CI) 0.85 to 0.89) and a pooled specificity of 0.98 (CI, 0.98 to 0.99). The area under the curve (AUC) for the SROC curve was 0.988, and the summary diagnostic odds ratio was 374.91 (CI, 249.97 to 562.30). The 13 studies on Dsg3 autoantibodies which met the inclusion criteria, yielded a pooled sensitivity of 0.97 (CI, 0.95 to 0.98), and a pooled specificity of 0.98 (CI, 0.98 to 0.99). The AUC for the SROC curve was 0.995 and the summary diagnostic odds ratio was 1466.11 (95% CI, 750.36 to 2864.61).

CONCLUSIONS:

Results of the meta-analysis demonstrated that ELISA tests for anti-BP180 and anti-Dsg3 autoantibodies have high sensitivity and specificity for BP and PV, respectively, and can be used in daily laboratory practice for the initial diagnosis of autoimmune blistering skin diseases.
PMID: 22781589 [PubMed – as supplied by publisher] (Source: Autoimmunity Reviews)

from MedWorm: Pemphigus http://www.medworm.com/index.php?rid=6303276&cid=c_297_3_f&fid=34528&url=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2FPubMed%2F22781589%3Fdopt%3DAbstract

Paraneoplastic pemphigus (PNP) is a distinct autoimmune blistering disease that can affect multiple organs other than the skin. It occurs in association with certain neoplasms, among which lymphoproliferative diseases are most commonly associated. The clinical presentation of PNP consists typically of painful, severe oral erosions that may be accompanied by a generalised cutaneous eruption and systemic involvement. The eruption may be of different morphology, consisting of lesions that resemble pemphigus, pemphigoid, erythema multiforme or graft versus host disease, as well as lesions resembling lichen planus. Similarly, the histological findings also show considerable variability. PNP is characterised by the presence of autoantibodies against various antigens: desmoplakin I (250 kd), bullous pemphigoid antigen I (230 kd), desmoplakin II (210 kd), envoplakin (210 kd), periplakin (190 kd), plectin (500 kd) and a 170-kd protein. This 170-kd protein has recently been identified as alpha-2-macroglobulin-like-1, a broad range protease inhibitor expressed in stratified epithelia and other tissue damaged in PNP. The prognosis of PNP is poor and the disease is often fatal. Immunosuppressive agents are often required to decrease blistering, and treating the underlying malignancy with chemotherapy may control autoantibody production. The prognosis is better when PNP is associated with benign tumours and these should be surgically excised when possible.

http://onlinelibrary.wiley.com/doi/10.1111/j.1440-0960.2012.00921.x/abstract

Congresswoman Ann Marie Buerkle (R-NY 25th)

On March 28, 2012 in partnership with the National Coalition of Autoimmune Patient Groups (NCAPG), the IPPF co-sponsored a congressional briefing titled, “The Multi-Generational Impact of Autoimmune Disease: America’s Silent Health Crisis” featuring experts on the subject.

The IPPF is pleased to announce that Congresswoman Ann Marie Buerkle (R-NY 25th) is sponsoring a bi-partisan bill that will establish an autoimmune disease interdepartmental coordinating committee — and it has full House leadership support. The bill is excellent and would establish a very high-level committee called the Autoimmune Diseases Interdepartmental Coordinating Committee (ADICC). The Committee would have the responsibility to develop criteria to be used in defining and identifying autoimmune disease, report on the  strategic plan for autoimmune diseases, and make recommendations on education and continued education for health care professionals on autoimmune disease.  Although the funding included is not a lot (just over $1 million), more funding could be added in future years as the ADICC make budgetary recommendations in the follow on years of the bill’s time frame.

I have been told that they will not include any language about a single disease and that the House leadership has also made a decision not to support any single disease (not just autoimmune ones) in any legislative language.  It would, therefore, make real sense to band together behind this bill which will benefit all autoimmune diseases.

The American Autoimmune Related Diseases Association (AARDA) has recommended that the Social Security Administration be included on the ADICC and is waiting to hear back as to whether the Congresswoman agrees. AARDA member organizations would also like to have some language that would support a study of the healthcare costs (now an unknown) of autoimmune diseases and are working together to make this a reality.

The IPPF is a long-time member of AARDA (www.aarda.org) and the NCAPG. This is a great opportunity to work together for the benefit of all autoimmune diseases. The IPPF will keep members posted on the progress of this bill. Feel free to contact your US representatives and let them know you support this bill.

Congress (www.congress.gov)| House of Representatives (www.house.gov)

Although there are no standard guidelines for the treatment of autoimmune blistering diseases, azathioprine has shown good efficacy in acquired autoimmune blistering diseases, and is well tolerated. Side effects of azathioprine normally occur in mild variants. Severe reactions are due to reduced thiopurine S-methyltransferase (TPMT) or inosine triphosphate pyrophosphohydrolase (ITPA) activity. Therefore, screening for TPMT activity should be conducted in white patients and Africans, whereas Japanese should be screened for ITPA activity before therapy with azathioprine is started. Azathioprine is clinically meaningful for the treatment of pemphigus. (Source: Immunology and Allergy Clinics of North America)

http://www.immunology.theclinics.com/article/PIIS0889856112000240/abstract?rss=yes