Autoimmunity reviews
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Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis (SSc) and mainly encountered in patients with diffuse disease and/or anti-topoisomerase 1 antibodies. ILD develops in up to 75% of patients with SSc overall. However, SSc-ILD evolves to end-stage respiratory insufficiency in only a few patients. ⋯ Cyclophosphamide, which has been used for 20 years, has recently been evaluated in two prospective randomized studies that failed to demonstrate a major benefit for lung function. Open studies reported mycophenolate mofetil, azathioprine and rituximab as alternatives to cyclophosphamide. On failure of immunosuppressive agent treatment, lung transplantation can be proposed in the absence of other major organ involvement or severe gastro-esophageal reflux.
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Autoimmunity reviews · Dec 2010
ReviewCatastrophic antiphospholipid syndrome: updated diagnostic algorithms.
The catastrophic antiphospholipid syndrome (APS) is the most severe form of APS with acute multiple organ involvement and small vessel thrombosis. During the 13th International Congress on Antiphospholipid Antibodies (aPL) a "Catastrophic APS Task Force" was developed to discuss the challenges in the diagnosis and management of catastrophic APS. The purpose of this paper is to summarize the diagnostic challenges (false-positive/negative aPL results and overlap with other thrombotic microangiopathies) and propose updated diagnostic algorithms for catastrophic APS. Important steps of the diagnostic algorithms include: a) history of APS or persistent aPL-positivity; b) three or more organ new thrombosis developing in less than a week; c) biopsy diagnosis of microthrombosis; and d) other explanations for multiple organ thromboses and/or microthrombosis.
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Autoimmunity reviews · Sep 2010
ReviewMolecular pathways involved in synovial cell inflammation and tumoral proliferation in diffuse pigmented villonodular synovitis.
Diffuse-type tenosynovial giant cell tumors, also known as pigmented villonodular synovitis, are unique mesenchymal lesions that arise from the synovial tissue of the joints. They are predominantly intraarticular, aggressive, infiltrative processes, characterized by both inflammatory or neoplastic properties and local destructive progression. The pattern of synovial gene and protein expressions in pigmented villonodular synovitis, similar to those in activated macrophages in rheumatoid arthritis, and the phenotype of multinucleated giant cells, characteristic of osteoclasts, suggest that there is a common autocrine mechanism in osteoclast differentiation in both diseases and indicate the potential utility of tumor necrosis factor (TNF)-alpha blockade. ⋯ Knee injections produced a rapid reduction in clinical and sonographic indexes and immunohistological alterations, confirmed by arthroscopic synovectomy. A delayed relapse in one of the four knees and unaltered synovial CSF1 expression were other important findings. In the light of these observations, CSF1/CSF1R interaction probably represents a more sensible therapeutic target than TNF-alpha blockade in the diffuse form of pigmented villonodular synovitis.
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The term pulmonary renal vasculitis syndrome describes a clinical syndrome of diffuse alveolar haemorrhage (DAH) complicating acute glomerulonephritis that often heralds severe, life-threatening systemic vasculitis requiring urgent, aggressive therapy. "Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis" and glomerular basement membrane ("Goodpasture's") disease are the commonest causes but other pathologies including systemic lupus erythematosus and the anti-phospholipid syndrome are also implicated. Such patients hence present most commonly to rheumatologists and nephrologists but usually require input from a number of specialties, respiratory and critical care medicine in particular. Such care is typically provided in specialist centres able to offer plasma exchange and experience in the use of immunosuppressants. We review clinical features, advances in therapy and understanding of pathophysiology.
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Vitamin D levels depend on many variables, including sun exposure, age, ethnicity, body mass index, use of medications and supplements. A much higher oral vitamin D intake than the current guidelines is necessary to maintain adequate circulating 25(OH)D levels in the absence of UVB radiation of the skin. ⋯ Disorders such as systemic lupus erythematosus, rheumatoid arthritis, Behçet's, polymyositis/dermatomyositis and systemic scleroderma have all been associated to some extent to vitamin D deficiency. If vitamin D deficiency occurs at a higher rate in patients with autoimmune disorders, then appropriate supplementation may be indicated.