1/7. Autologous peripheral blood stem cell transplantation in a patient with severe mixed connective tissue disease.A 65-year old man with mixed connective tissue disease (MCTD) and severe therapy resistant polymyositis was considered for high-dose cyclophosphamide (200 mg/kg) supported by autologous stem cell transplantation (ASCT). During a 21 months follow-up there has been a significant subjective, but objectively only a slight improvement in muscle strength. Initially the levels of serum creatine kinase and serum aldolase normalised, but are at 21 months at about the same level as before ASCT. Based on histopathological examination there is still active myositis. Our case would suggests that this treatment may have some efficacy in MCTD with severe polymyositis although longer follow-up is needed.- - - - - - - - - - ranking = 1keywords = mixed connective, connective (Clic here for more details about this article) |
2/7. polymyositis as a cause of total gut failure.BACKGROUND: Gastrointestinal manifestations are seen in systemic sclerosis and mixed connective tissue disorders but are rare in pure polymyositis. CASE REPORT: A 44 year old woman with polymyositis who developed total gut failure requiring treatment with total parenteral nutrition is described. RESULTS: The patient's polymyositis is now fully controlled biochemically, but her gastrointestinal symptoms persist.- - - - - - - - - - ranking = 0.2keywords = mixed connective, connective (Clic here for more details about this article) |
3/7. erythema elevatum diutinum in the setting of connective tissue disease and chronic bacterial infection.erythema elevatum diutinum (EED) is a rare and chronic cutaneous leukocytoclastic vasculitis. It is predominantly seen on the extensor surfaces of the extremities. Although a specific cause is largely unknown, EED has been noted to occur in association with a wide variety of diseases. A 28-year-old man with systemic lupus erythematosus (SLE) and a 53-year-old woman with an overlap syndrome of rheumatoid arthritis and polymyositis are presented. Both patients developed EED in the setting of chronic recurrent bacterial infections. patients with a connective tissue disease are at increased risk for such infections secondary to immunosuppression, either from the disease itself or secondary to immunosuppressive therapy. EED has been independently reported to occur in the setting of connective tissue disease as well as in the setting of chronic infection. Our patients had both of these underlying conditions, which are known to predispose patients to immune complex-mediated vasculitides, in this case EED. One patient's EED responded to treatment of the SLE and the other improved, as has been previously reported with dapsone.- - - - - - - - - - ranking = 0.11045101232056keywords = connective (Clic here for more details about this article) |
4/7. A localised morphoea/idiopathic polymyositis overlap.A 54-year-old female presented with a three-year history of proximal muscle weakness and arthralgia. EMG, muscle enzymes and biopsy and other tests were consistent with idiopathic polymyositis. At a later stage, she developed plaques of morphoea involving the dorsum of hands and forearms. There was no evidence of systemic sclerosis throughout the course of the disease. In our opinion, such association appeared as an unusual overlap syndrome that provided a new constellation of features in connective tissue disease.- - - - - - - - - - ranking = 0.018408502053427keywords = connective (Clic here for more details about this article) |
5/7. bronchiolitis obliterans organizing pneumonia as the first manifestation of polymyositis.bronchiolitis obliterans organizing pneumonia (BOOP) preceding polymyositis is rare. In this report, a 51-year-old patient with fever, nonproductive cough, and dyspnea had bilateral basal interstitial infiltrates on chest roentgenogram. Open lung biopsy was consistent with BOOP. prednisone therapy led to improvement, but 8 weeks later, fever, cough, and weakness of the arms and legs developed because the patient had not been compliant with the prednisone regimen. The creatine kinase (CK), the macrophage inflammatory protein (MIP-1), and the tumor necrosis factor (TNF-alpha) were elevated. Anti-Jo-1 antibody was not present. Quadriceps femoris muscle biopsy was compatible with polymyositis. After a second course of corticosteroid therapy, the patient became afebrile, the dyspnea resolved, the pulmonary infiltrates decreased, and the muscle strength improved. The serum CK, MIP-1, and TNF-alpha levels declined significantly. This is only the second reported case of BOOP preceding polymyositis. patients with idiopathic BOOP should have follow-up for the possible development of connective tissue disorders including polymyositis.- - - - - - - - - - ranking = 0.018408502053427keywords = connective (Clic here for more details about this article) |
6/7. Fatal alveolar hemorrhage in a patient with mixed connective tissue disease presenting polymyositis features.A 22-year-old female with Raynaud's phenomenon, swollen hands and a high titer of anti-RNP antibodies developed fever and myositis. Prednisolone (40 mg/day) was considered effective for myositis since circulating myogenic enzymes rapidly decreased. However, she suddenly developed respiratory distress with bilateral pulmonary infiltrates and bloody sputum. Under the diagnosis of alveolar hemorrhage (AH), intravenous methylprednisolone pulse therapy was given, but she died of respiratory failure. autopsy findings demonstrated massive AH with hematoma formation, and myositis in the iliopsoas muscle. Depositions of immune complex and vasculitic lesions were not recognized in her lungs.- - - - - - - - - - ranking = 0.8keywords = mixed connective, connective (Clic here for more details about this article) |
7/7. Acute sympathetic hyperfunction in overlapping syndromes of systemic lupus erythematosus and polymyositis.A 20-year-old woman with arthralgia, serositis, thrombocytopenia, proteinuria, muscle weakness, elevated creatinine kinase, and positive anti-Sm antibody was diagnosed as having polymyositis and systemic lupus erythematosus (SLE). She had persistent high temperature, sinus tachycardia, hyperhidrosis, mydriasis, visual disturbance, hallucination, and loss of consciousness. Levels of plasma adrenaline, noradrenaline, and dopamine and cerebrospinal fluid interleukin (IL)-6 and IL-8 were all high. A diagnosis of sympathetic hyperfunction accompanied by central nervous system (CNS) involvement in SLE was made parenteral. pulse administration of high dose corticosteroid therapy was effective. This is the first reported case of a connective tissue disease with CNS involvement manifesting as sympathetic hyperfunction with high plasma catecholamine levels.- - - - - - - - - - ranking = 0.018408502053427keywords = connective (Clic here for more details about this article) |