PubMed · 3396977
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B Schindel, M Lapidot, R Weinstein, Y Rusecki, S Leiba. 1988-05-15. [Polyglandular autoimmune failure].. https://pubmed.ncbi.nlm.nih.gov/3396977/
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Autoimmune pancreatitis is a recently described chronic inflammatory disease of the pancreas which appears to be part of a systemic disorder characterized by a lymphoplasmacytic infiltrate in affected organs that is rich in IgG4 positive cells. The inflammatory component of the disease appears to resolve with steroid treatment, though relapses do occur. Here we briefly discuss the role of steroid therapy in the management of autoimmune pancreatitis.
We treated a 5-year-old boy, in our hospital in south India, who had a history of recurrent respiratory infections, tuberculosis, and severe varicella infection. He was short in build and a radiological examination revealed evidence of spondylometaphyseal dysplasia. Investigation of the immune system was suggestive of compromised cellular immunity. Immunofluorescence and immunoblot assay for antibodies detected underlying multiple disorders such as systemic lupus erythematosus (SLE), autoimmune thrombocytopenia, and juvenile rheumatoid arthritis (JRA). Roifman et al. described a similar syndrome in 2000 and 2003, which was characterized by spondylometaphyseal dyplasia, combined immunodeficiency, and autoimmunity and called it Roifman-Costa syndrome (OMIM 607944). Hence a diagnosis of Roifman-Costa syndrome was made. Ours shall be the first report of such a condition from the Indian subcontinent and hence the communication.
Our understanding of the multiple physiological and pathological functions of B-cells continues to expand at a fascinating rate. A critical part of this expanding knowledge is the realization that B-cells can be responsible, at least in part, for diseases in which they had not been previously suspected and that their pathogenic influence can be mediated by multiple mechanisms. In turn, the availability of effective agents capable of inducing profound and long-lasting B-cell depletion and the safety and efficacy of Rituximab in non-Hodgkin lymphoma has prompted investigators to use this therapeutic approach in a large number of autoimmune diseases. Thus far, the results have been very promising, and in some cases nothing short of spectacular. In this review, we shall discuss the roles of B-cells in health and disease and the available evidence regarding the efficacy of B-cell depletion in human autoimmunity. Finally, we will discuss some of the many challenges and opportunities that the medical and scientific community should address in the foreseeable future.