La Revue de médecine interne
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Case Reports
[Inflammatory myopathy following acute meningoccemia in a properdin-deficient patient: A case report].
Myalgia is a classical sign in invasive meningococcal diseases (IMD), but severe and persistent myalgia following an IMD have never been reported to date. ⋯ Post-infectious inflammatory myopathy should be suspected in case of severe and persistent myalgia associated with rhabdomyolysis following an IMD, after exclusion of pyomyositis especially. A short course of corticosteroids seems to be effective.
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Avascular necrosis is an ischemic or cytotoxic necrosis of epiphyseal bone, responsible for joint pain, altered life quality and frequently affecting young patients. Avascular necrosis can be unifocal or multifocal, underlining the possibility of a systemic origin. Avascular necrosis involves the femoral head in more than 75% of cases. ⋯ In the earlier stages of the disease (stage I and II of the Arlet and Ficat classification), joint surface is preserved, and conservative treatment is recommended. In the more advanced stages (III and IV of the Arlet and Ficat classification), the articular surface collapses and joint arthroplasty is the main treatment. However, there are some recent therapeutic advances, based on mesenchymal stem cells, which may contribute, in the future, to improve the bad functional prognosis of the disease.
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Anti-glomerular basement membrane (anti-GBM) disease or Goodpasture's syndrome is a small vessel vasculitis affecting the capillary beds of kidneys and lungs. It is an autoimmune disease mediated by autoantibodies targeting the glomerular and alveolar basement membranes, leading to pneumorenal syndrome. ⋯ Management should be prompt and combine plasma exchange with systemic corticosteroids and immunosuppressive therapy by cyclophosphamide. The objective of this review is: 1) to describe the pathogenesis, clinical and histological features of the disease; 2) to characterize double-positive anti-GBM/ANCA patients; 3) to highlight the prognostic factors of renal and global survival, and 4) to focus on the treatment of anti-GBM disease.
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Double-positive vasculitis with anti-polynuclear cytoplasm (ANCA) and anti-glomerular basement membrane (GBM) antibodies is a rare entity of systemic vasculitis defined by the presence of ANCA and anti-GBM antibodies. The gradual accumulation of clinical and therapeutic data shows the usefulness of identifying and differentiating this entity from the two vasculitis respectively associated with the isolated presence of each of these two antibodies. Indeed, the double-positive ANCA and anti-GBM vasculitis appears to associate the characteristics of the demography and the extra-renal and pulmonary involvement of the ANCA-associated vasculitis on the one hand, and of the histological type and severe renal prognosis of the anti-MBG vasculitis on the other hand, with the renal involvement which is the only involvement consistently observed in double-positive vasculitis. The aim of this focus is to describe the epidemiological, clinico-biological, histological and prognostic characteristics of this entity, in light of recent literature and ongoing therapeutic changes in the two eponymous vasculitis.