Paraspinal and scapular myopathy associated with scleroderma.
ABSTRACT To describe a form of inflammatory myopathy with prominent involvement of the paraspinal and scapular muscles in patients with scleroderma.
Review of clinical records, laboratory investigations, and muscle biopsies.
Patients presented with a "dropped head" resulting from weakness of the posterior cervical muscles (three cases) or camptocormia ("bent spine") resulting from weakness of the paraspinal muscles (two cases) and variable weakness and atrophy of shoulder girdle muscles with mild or absent pelvic girdle involvement. Biopsies from the deltoid or paraspinal muscles showed myositis of variable severity and scleroderma vasculopathy in all cases. The response to prednisolone and cytotoxic agents was poor, but there was a good response to intravenous immunoglobulin therapy in one case.
Patients with scleroderma may develop a restricted form of immune-mediated inflammatory myopathy with a predilection for the paraspinal and scapular muscles, which is poorly responsive to treatment with glucocorticoids and immunosuppressive agents and may require consideration of other treatment modalities.
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ABSTRACT: In this case, we describe an unusual presentation of a young woman with a rash typical of morphoea (confirmed on biopsy), who went on to develop myositis in an atypical distribution. Although the association of myositis with diffuse systemic sclerosis is well described, the link with localised scleroderma (morphoea) and myositis has not been described.Case reports in rheumatology. 01/2011; 2011:134705.
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ABSTRACT: This review discusses the characterization of myopathy in scleroderma with a focus on new developments in imaging, biomarkers, and therapy, and details several current reports and several seminal reports prior to 2012. In the past year, studies have shown that MRI techniques highlight the importance of muscle edema in scleroderma, and that aldolase may be a useful biomarker to predict incident myopathy. When compared to studies preceding 2012, both the current and prior reports too often fail to account for the full spectrum of muscle disease in scleroderma. There remain no uniform classification criteria that are routinely integrated into clinical research reports. Thus, important questions remain to be answered, including risk factors for developing myopathy, optimal screening and diagnostic strategies, and efficacious therapies. But, just as important is the priority to systematically define what the true entity(ies) of myopathy is in scleroderma. Scleroderma myopathy is a heterogeneous group of muscle disorders among patients with underlying scleroderma which requires robust studies to clarify the full spectrum of disease.Current opinion in rheumatology 01/2014; · 5.07 Impact Factor
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ABSTRACT: Camptocormia, an abnormal truncal flexion posture that occurs while walking or standing, is usually caused by various hypokinetic movement disorders, mainly Parkinson disease. We describe the case of a man with subacute onset of camptocormia. Quadriceps muscle biopsy showed significant rhabdomyolysis, few isolated inflammatory cells and mild expression of type I MHC in few fibers, a pattern usually found in immune-mediated necrotizing myopathies. Myositis was associated with Raynaud's phenomenon, mild sclerodactyly, and anti-Ku antibodies leading to the diagnosis of polymyositis/systemic sclerosis overlap myositis. The posture showed modest improvement in response to treatment.Rheumatology International 03/2012; · 1.63 Impact Factor