P-MU005. Myopathy in primary Sjögren’s syndrome in Dr. Moewardi Hospital Indonesia

Abstract

Introduction . Myopathies are disorders affecting skeletal muscle. There is a term 'overlap syndrome' when myopathies are associated with another well-defined connective tissue disease. Sjögren’s syndrome is one of the connective tissue disease characterized by hypo- function of salivary and lacrimal glands and possible systemic multi-organ manifestations. Although muscular manifestations are very common with Sjögren’s syndrome, true myopathy is very rare. Myopathy inflammatory has been reported in less than 3% of patients. The aim of the case report is to report a case of myopathy in primary Sjögren’s syndrome in Dr. Moewardi hospital Surakarta. Results . 22 years old female, with the weakness of all four extremities, arthralgia, myalgia, and shortness of breath due to respiratory failure. The weakness was heavier in the proximal. Schirmer’s test ≤5 mm/5 min in left eye. From the results of laboratory examination obtained increased CK, SGOT, and SGPT. From cerebrospinal fluid examination, no dissociation of cytoalbumin was found. There was a positive (strong) result on La / SS-B antigen. Muscle biopsy showed hyalinized muscle. Plasmapheresis was performed on this patient. The patient received intravenous methylprednisolone, azathioprine, and folic acid therapy. The patient did not improve after the plasmapheresis and immunosuppressant administration. Conclusion . Skeletal muscle involvement in primary Sjögren’s syndrome is a relatively rare complication. Some signs, symptoms, and laboratory results support the appearance of myopathy in this case. Muscle biopsy is the gold standard examination for diagnosed myopathy. In Sjögren’s syndrome, the type of myopathy that is often found is myositis. In this case, there was a hyalinized muscle that supports myopathy but no inflammatory cells were found on the muscle biopsy. Myopathy in Sjögren’s syndrome responded favorably to high- dose steroids. In this case, there was no improvement after immunosuppressant administration.