Retroperitoneal fibrosis.

Retroperitoneal fibrosis (RPF) is a rare disease that is marked by systemic inflammation and the development of a periaortic fibroinflammatory mass. The fibroinflammatory infiltration can encase the abdominal aorta, ureters, and other abdominal organs. The clinical presentation often includes constitutional symptoms, abdominal pain, and signs of renal insufficiency or renal failure related to ureteral obstruction. Less frequently, RPF may present with vascular complications, such as venous thrombosis or claudication. The idiopathic form of RPF is most common but secondary forms have been described and are associated with malignancy and a variety of different medications. The pathophysiology is uncertain, but RPF has been linked with periaortitis and IgG4-related disease. Treatment centers on the relief of symptoms and complications associated with mass effects. Corticosteroids and other immunosuppressant therapies can improve constitutional symptoms, reduce infiltrate mass, and achieve disease remission, but a chronic relapsing course is not uncommon.

Erosive osteoarthritis: A systematic analysis of definitions used in the literature.

BACKGROUND: Erosive osteoarthritis (EOA) is a commonly invoked diagnosis representing an important variant of hand osteoarthritis (OA). There is increasing literature on the prevalence, risk factors, etiology, and management of EOA. METHODS: We systematically reviewed the literature to assess variability in the diagnostic definitions used to define EOA in these studies. RESULTS: We reviewed 336 articles and found 62 articles citing diagnostic definitions for EOA. Radiographic appearance was the most commonly used criterion, but there was little agreement on the details or extent of the radiographic changes. Overall, 56 of the 62 studies included clinical features in the diagnostic definitions, yet these features varied considerably. Exclusion criteria were mentioned in 43 of the studies. CONCLUSION: Based on the widely disparate definitions of EOA, we urge caution in interpretation of this literature, and propose that further understanding of EOA will require consensus on its definition.

Diagnosis and treatment of the idiopathic inflammatory myopathies.

The idiopathic inflammatory myopathies (IIMs) are rare disorders with the unifying feature of proximal muscle weakness. These diseases include polymyositis(PM), dermatomyositis (DM) and inclusion body myositis (IBM) as the most common. The diagnosis is based on the finding of weakness on exam, elevated muscles enzymes, characteristic histopathology of muscle biopsies, electromyography abnormalities and rash in DM. Myositis-specific antibodies have been helpful in defining subsets of patients with different responses to treatment and prognosis. The cornerstone of therapy is corticosteroids with the addition of other immunosuppressives in severe or refractory disease or patients with intolerable side effects. IBM is particularly difficult to treat but is more slowly progressive as compared with PM or DM. There is still a great need to find more effective and less-toxic therapies.