A 58 year-old male guitarist presented to the hospital with worsening generalized weakness for a week. He also had sore throat and generalized malaise with diarrhea and decreased oral intake. Past medical history is significant for hypertension, type II diabetes mellitus and a recent diagnosis of rheumatoid arthritis (RA). His home medications are lisinopril, metformin and glipizide. He denies any NSAIDs use. Physical examination was unremarkable except for tachycardia and generally unwell in appearance. His initial labs were remarkable for blood glucose of 30 mg/dl and acute kidney injury (AKI) with creatinine of 6.6 mg/dL (baseline 0.8); fractional excretion of sodium of 4.5; urinalysis revealed microscopic hematuria and nephritic range proteinuria; serum complement and protein electrophoresis were normal; anti-streptolysin O antibody was negative. After correcting hypoglycemia, IV fluids were started for the AKI. Vasculitis work up was ordered and was remarkable for myeloperoxidase (MPO)-ANCA. Renal biopsy showed crescentic glomerulonephritis (GN) pauci-immune type suggestive of MPO associated disease. Prednisone, Cyclophosphamide and 7 cycles of plasmapheresis were started. As the renal function wasn’t improving with worsening uremia, he was started on hemodialysis. With initiation of the treatment, his pains and joint stiffness in hands improved significantly and was excited to play guitar again. He was clinically stable and was discharged on outpatient hemodialysis along with the cyclophosphamide and long tapering doses of steroids. Following discharge his renal functions were gradually improving and the hemodialysis was stopped a week later.
Renal limited MPO-vasculitis with simultaneous RA is reported in the literature, but is a rare occurrence. Review of literature suggests that most of these patients had a diagnosis of RA for several years prior to the presentation with renal failure from MPO-vasculitis. Patients with simultaneous diseases usually present in middle age and may have other signs or symptoms of systemic vasculitis. Patients with longstanding RA may develop renal failure commonly due to NSAIDs use and less frequently due to inflammatory amyloidosis, membranous GN or vasculitis. Our patient is unique that he was diagnosed with RA recently, a few months ago, and still presented with MPO-vasculitis limited to the kidneys requiring hemodialysis at this early stage of his RA. Early diagnosis and treatment is the key to avoid permanent renal damage. Treatment is immunosuppression with high dose steroids and Cyclophosphamide or Rituximab. Plasma exchange has benefit in only patients with severe active renal disease, pulmonary hemorrhage and concurrent Anti-GBM antibodies.
We report a unique case of simultaneous presentation of RA and MPO-renal limited vasculitis. We also highlight the distinctive clinical features, modalities of diagnosis and the importance of timely treatment in such cases.