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Abstract Details

Acute Overlap Syndrome with Myopathy Associated with Anti-signal Recognition Particle Antibodies and Sarcoidosis
Neuromuscular and Clinical Neurophysiology (EMG)
P16 - Poster Session 16 (5:30 PM-6:30 PM)
1-008

To present an acute overlap syndrome of anti-signal recognition particle antibody (anti-SRP) myopathy and systemic sarcoidosis
NA

Case report

A 61-year-old African-American man presented with a 3 week history of progressive dyspnea and generalized weakness. Two years earlier he had been diagnosed with systolic heart failure of unknown etiology (ejection fraction of 20%). Examination was notable for generalized proximal to distal weakness. Diagnostic evaluation revealed non-caseating granulomas (NCG) on cardiac biopsy and creatine kinase of 8,889 IU/L. Renal function, electrolytes, complete blood count, thyroid hormone, erythrocyte sedimentation rate, and antinuclear antibodies were normal. Electromyography revealed a severe generalized irritative myopathy. Serologies for HIV and 3-hydroxy-3-methylglutaryl coenzyme A reductase antibody were negative but the myositis panel showed high-titer anti-SRP antibodies (>100 SI). Subsequent muscle biopsy revealed scattered necrotic and regenerating fibers with a paucity of inflammation and no NCG. Patient was treated with pulse dose IV methylprednisolone followed by IV immunoglobulin and steroid taper. Within days of treatment, the patient showed significant improvement in strength and mobility. 
The patient’s clinical phenotype, autoantibodies, and histological data are characteristic of SRP myopathy, which is characterized by rapidly progressive proximal muscle weakness, scattered necrotic muscle fibers and scant inflammation. In contrast, sarcoidosis myopathy typically presents with insidious muscle weakness along with NCG and diffuse inflammation on biopsy. Although asymptomatic muscular involvement in sarcoidosis is common (50% of cases at post-mortem), clinically apparent myopathy is seen in only 1% of cases. This is the first reported case of overlap syndrome with systemic sarcoidosis and SRP myopathy, and highlights the importance of considering other potential etiologies of rapidly progressive myopathy despite histologically-proven systemic sarcoidosis.
Authors/Disclosures
Catherine Daley, MD (Northwestern Medicine)
PRESENTER 		Dr. Daley has nothing to disclose.
No disclosure on file
Jinny O. Tavee, MD (National Jewish Health) Dr. Tavee has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for CSL Behring. Dr. Tavee has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Johnson and Johnson. Dr. Tavee has received personal compensation in the range of $0-$499 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Dynamed. The institution of Dr. Tavee has received research support from Woolsey. The institution of Dr. Tavee has received research support from Milken Foundation. The institution of Dr. Tavee has received research support from CSL Behring. Dr. Tavee has received personal compensation in the range of $0-$499 for serving as a Article author with Medlink.