Zeitschrift für Rheumatologie
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Interstitial lung disease (ILD) is among the most common organ manifestations in collagen vascular diseases and can be a major determinant of the long-term prognosis. Early diagnosis rests mainly on spirometry, including measurement of the CO diffusion capacity, and high-resolution computed tomography (HRCT). ⋯ Therapeutic experience is heterogeneous but evidence is accumulating suggesting that NSIP in the collagen vascular diseases is amenable to immunosuppression. Rapid clinical and/or radiological deterioration, reflecting inflammatory activity, appears to mark those patients who are most likely to benefit from cyclophosphamide therapy.
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To date, the long-term course of fibromyalgia (FM) generally shows little improvement and many patients have problems managing their private, social and professional lives, if at all. Health workers in various specialties, and even in the same specialty, have differing concepts on etiology, diagnosis and treatment of FM. In its work spanning 2 years, an interdisciplinary working group made up from 10 scientific associations reviewed the literature and developed an S3-level consensus guideline. ⋯ A stepwise treatment approach with patient education, endurance training, multi-modal therapy and promotion of significant resources is recommended. Of the psychotherapeutic methods, cognitive behavioural training has proved the most relevant. Only a handful of drug therapies have undergone controlled trials, most however only over a period of a few weeks.
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Muscle and nerve biopsies provide valuable information contributing to the diagnosis of diseases of the peripheral nervous system and skeletal muscle. The application of modern histological, immunohistochemical, electron microscopic and molecular methods establishes a definite diagnosis in many cases and narrows the spectrum of possible entities in most of the remaining cases. Inflammatory myopathies and neuropathies are distinguished from non-inflammatory muscular and peripheral nervous conditions. The latter include muscular dystrophies and congenital myopathies, hereditary neuropathies, metabolic diseases affecting skeletal muscle and peripheral nerves as well as degenerative myopathies and neuropathies including sporadic motor neuron diseases.
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A 61-year-old male patient presented with petechiae on the arms and legs due to a thrombocytopenia of 3 Gpt/l (3000/microl). A butterfly rash on the patient's face suggested a diagnosis of systemic lupus erythematosus (SLE). The thrombocytopenia was due to autoimmune thrombocytopenia. The diagnosis of SLE could be excluded and the butterfly rash attributed to a laminar hemorrhage, an ecchymosis due to the autoimmune thrombocytopenia.
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Promising results in an animal model of multiple sclerosis (MS; experimental autoimmune encephalomyelitis, EAE) have shown that immunosuppression followed by allogeneic bone marrow transplantation has the potential to significantly ameliorate the spontaneous course of the disease. Since 1995, emerging data on autologous hematopoietic stem cell transplantation (AHSCT) has supported a benefit also in patients with multiple sclerosis. To date, results on approximately 500 cases have been consecutively reported by the European Group for Blood and Marrow Transplantation (EBMT). ⋯ However, a number of critical issues remain unresolved. Furthermore, with upcoming new treatment compounds that to some extent act via lymphoablative properties, it remains essential to better select those patients who might profit most from stem cell therapy based on a justifiable benefit-to-risk ratio. Although transplant related mortality has dropped to 1%, mortality combined with concerns about long-term safety remain critical issues in a primarily non-life-threatening disease like MS.