An Unusual Presentation of Anti Pm/Scl Antibody-positive Idiopathic Inflammatory Myositis

Abstract

ABSTRACT Idiopathic inflammatory myopathies are the heterogeneous group of diseases that affect multiple systems apart from the skeletal muscles, preferentially affecting the proximal skeletal muscle. We present a rare case with quadriparesis, purely involvement of proximal muscles without any extra muscular symptoms with positive Anti Pm/Scl antibodies. Our patients was 52 year old male who presented to be symptomatic with generalized swelling all over the body followed by progressive pure proximal group of muscles motor weakness of all four limbs without sensory involvement. He also developed oropharyngeal weakness, in the form of nasal regurgitation of food and significant weight loss. C reactive protein was 22 mg/L, erthryocyte sedimentation rate was 30 mm after 1 h, and liver enzymes were also increased (aminotransferase was 236 U/L and alanine aminotransferase was 145 U/L). His lactate dehydrogenase was 1175 U/L whereas creatinine kinase was 3290 U/L. His ANA profile was positive for Anti Pm/ Scl antibodies. MRI of both thighs was suggestive of diffuse inflammatory changes in both lower limbs and pelvic girdle and PET CT was suggestive of increased uptake in proximal limb muscles. He was managed with pulse Inj Methylprednisolone and intravenous Immunoglobulin. This followed by oral prednisolone for a few months than slow tapering. In Conclusion that Anti Pm/Scl antibody positive idiopathic inflammatory myositis can also present atypically without any other systemic manifestation and weakness in lower limb more than upper limbs. Idiopathic inflammatory myopathies disease is one of the treatable diseases that repones to immunomodulation therapy.