Acute fibrinous and organizing pneumonia, a recently described form of diffuse acute lung injury, sometimes affects adults with inflammatory myopathy. We describe a child with juvenile dermatomyositis who had development of acute fibrinous and organizing pneumonia. There does not appear to be a successful method of treatment, particularly in severe cases with respiratory failure.

Introduction Juvenile Dermatomyositis (JDM) is an inflammatory myopathy with a predilection for proximal muscles and skin. Current treatment of JDM includes early aggressive use of corticosteroids coupled with immunosuppressive drugs such as methotrexate, cyclosporine and intravenous immune globulins (IVIG). Delay to diagnosis and inadequate treatment increase the risk of developing calcinosis....

Telbivudine, a thymidine nucleoside analog, is a common therapeutic option for chronic hepatitis B infection. While raised serum creatine kinase is common, myopathy associated with telbivudine is rare. Reports on its myopathological features are few and immunohistochemical analyses of inflammatory cell infiltrates have not been previously described. We describe the clinical, myopathological and...

OBJECTIVE To study the clinical and pathological correlations of neuromuscular patients with a high aldolase and normal creatine kinase (CK) in serum at presentation or during a symptomatic exacerbation. METHODS Records and muscle biopsies were retrospectively reviewed in a consecutive series of 12 patients. Pathological results were compared to 75 abnormal muscle biopsies associated with acq...

June-July 2008 www.igliving.com IG Living! Case Study A 26-year-old female was referred to the rheumatology service two years after the insidious onset of “walking funny” that progressed to significant proximal muscle weakness over a 10-month period. Early in the course of her illness, her creatine kinase (CK) was 4,000. Subsequent muscle biopsy revealed muscle fiber degeneration and regenerati...

Myopathy is usually a non-fatal muscle disease involving skeletal muscle weakness, tenderness and pain with the possibility of the plasma creatinine kinase elevation. There are many different types of myopathies, some of which are genetic, inflammatory, or related to endocrine dysfunction. Also, numerous drugs have been reported to possess myotoxic effect. Myopathy is included among the potenti...

Multicore myopathy is a rare congenital myopathy. The multicores consist of numerous small areas of decreased oxidative enzyme activity. The long axis of the lesion is perpendicular or parallel to the long axis of the muscle fiber. These cores are usually smaller than central cores. For this reason they are also called minicores. Although the multicores represent a nonspecific change in that th...

The discovery of unique autoantibodies has informed and altered our approach to the diagnosis and management of the inflammatory myopathies. This study reports the initial clinical experience of use of the Extended Myositis Antibody (EMA) panel in the largest university teaching hospital in Ireland. We conducted a retrospective review of all patients who had serum samples tested for myositis sp...

In the past three decades, not much has changed in the pathophysiologic concepts of dermatomyositis and polymyositis. However, in the past couple of years, many changes have occurred reflecting the extremely complex nature of the immune response in general. New pathophysiologic models are needed, but at present, none of them encompasses all the recent findings. The changing concepts of dermatom...