Granulomatous Myositis Associated with Myasthenia Gravis: A Rare Case

Abstract

Myasthenia gravis (MG) is an immune-mediated disease of the neuromuscular junction mediated by anti-acetylcholine receptor (AChR) antibodies (Ab). Granulomatous Myositis (GrM) is a histological diagnosis characterized by the presence of epithelioid granuloma in striated muscles. Few cases describing the presence of concomitant thymoma and non-thymoma-related MG with GrM have been reported. This present case is an addition to the literature describing the presence of concomitant thymoma and non-thymoma-related MG with GrM. The patient described is a 77-year-old male who started developing weakness and atrophy involving the musculature of the bilateral lower and upper extremities. Initial laboratory workup showed an elevated level of serum creatine phosphokinase (CPK) of 1,231 U/ L (reference range: 22 to 198 U/L). The right quadriceps muscle biopsy performed showed inflammatory infiltrates containing eosinophils, plasma cells, and lymphocytes forming multinucleate giant cells consistent with a diagnosis of GrM. Detailed laboratory and imaging work conducted to rule out an underlying cause of GrM showed elevated titers of AChR Ab (79.50 nmol/L, reference range: <0.02 nmol/L) and striational Ab (titer: 1:320, reference range < 1:120). A positive repetitive nerve stimulation test for the left ulnar nerve (decrement in the amplitude of muscle action potential by 13%) further confirmed the diagnosis of MG concomitant with GrM. Computed tomography of the chest was negative for the presence of a thymoma. The patient was started on treatment with oral prednisone and mycophenolate mofetil, which resulted in an improvement of symptoms and the downward trending of serum CPK level.