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Abstract

Case Reports: We represent a case of Microscopic Polyangitis in elderly female who presented with generalized weakness, dry cough and unintentional weight loss. 79 year old female with PMHx of NIDDM, Hypertension, Hyerlipidemia, CKD, Anemia presented to ED with chronic intermittent dry cough and chest pain for 2 months, gradual onset of Shortness of breath and fatigue for almost 1 week. Patient endorses decreased appetite and 14 pound unintentional weight loss within one year. Patient had normal age appropriate cancer screening. She was found to be acutely declining renal function and anemia in last 1 year which was not explainable by her well controlled Diabetes and hypertension. On chest X-ray showed bilateral multiple pulmonary nodules which were confirmed by CT scan. Vasculitis workup was initiated and P-ANCA came back positive. Transbronchial Lung Biopsy showed focal acute vasculitis of medium size without granuloma and Renal biopsy with special stained confirmed the diagnosis. She was started on Cyclophosphamide and prednisone. Her symptoms improved dramatically and tolerating medication without any adverse events. Discussion: MPA is characterized by pauci-immune, necrotizing, and small vessel vasculitis without clinical or pathological evidence of granulomatous inflammation. It was initially considered as a microscopic form of PAN untill 1994. Wegner’s granulomatosis, MPA, and Churg-Strauss Syndrome comprise a category of small vessel ANCA associated pauceimmune vasculitis. However, an absence of granuloma formation and sparing of the upper respiratory tract are features of MPA. Renal failure and pulmonary involvement are the major causes of morbidity and mortality. With treatment, Falk and Guillevin (1990) reported 2- and 5-year survival rates of 75% and 74%, respectively.It is more common in Whites and Males. Average reported age is 50 years. Most common symptoms are fever(55%), malaise, myalgia, weight loss, rash(50%), cough and dyspnea which can be confused with any other common disorders. Treatment of microscopic polyangiitis (MPA) consists of 3 phases. A) Induction phase with oral cyclophosphamide and oral prednisone. In 2011 FDA approved rituximab for the induction as an alternative to Cyclophosphamide. Rituximab at 6 months has better remission rate than cyclophosphamide. B) Methotraxate/Azathioprine with prednisone. Rituximab is being studied currently being studied in randomized trial for the maintainance therapy. C) Treatment of relapse is the same as of the induction phase. Conclusion: Vasculitis is not uncommon diagnosis that is overlooked in the common day practice. This case shows reemphasis the same fact that and vasculitis should always in differential when patients have multisystem involvement which can not be explained by the chronic diseases like Diabetes and Hypertension.