INTRODUCTION:Microscopic Polyangiitis (MPA) can be difficult to diagnose even for the most experienced clinicians. It is the most common cause of pulmonary-renal syndrome. We are reporting a case of massive hemoptysis as an initial presentation of microscopic polyangiitis in a previously asymptomatic patient.
CASE PRESENTATION:An 87 year old female was brought in to the Emergency Room with the complaint of coughing up a large volume of fresh blood. She began to bleed profusely upon arrival to the emergency room and went into hypovolemic shock within 1 hour. She denied any prior history of similar complaints or past medical history. She had a smoking history but ceased smoking 20 years prior. She denied any illicit drug use or alcohol intake. Significant findings were confined to the Respiratory System which revealed bilateral coarse crackles. Admitting blood work disclosed anemia with hemoglobin 8 mg/dl and renal insufficiency serum creatinine 1.3 with no other abnormalities. She remained in shock despite aggressive fluid management, blood transfusion and pressor support. Emergent bronchoscopy was attempted with aim of identifying the source of bleeding but was unsuccessful. Double lumen endotracheal tube was inserted by the anesthesiologist. Estimated total blood loss was now over 2 liters. Cardiothoracic Surgery consult was called but patient went into cardiac arrest soon after and expired. Autopsy confirmed extensive bilateral pulmonary hemorrhage with an incidental finding of small nodule of squamous cell lung carcinoma. Sections from bilateral lungs showed parenchymal arterioles, venules and capillaries with acute inflammation, thickening and subintimal edema of their walls. The diagnosis was made of microscopic polyangiitis and squamous cell lung cancer.
DISCUSSIONS:Microscopic polyangiitis is a vasculitis of small vessels. It is a characterized by a pauci-immune, necrotizing, vasculitis of small vessels without clinical or pathological evidence of granulomatous inflammation.Pulmonary manifestations of microscopic polyangiitis range from fleeting focal infiltrates to massive pulmonary hemorrhage. Diffuse alveolar hemorrhage is a potentially fatal complication with a prevalence of 12-29%1 however; the presentation of massive hemoptysis in the mentioned patient who was previously asymptomatic is not often seen. Bleeding is usually refractory to therapy, and the mortality rate is 31 % 1. The relative risk for death is 8.65 times greater in patients who presented with pulmonary hemorrhage and 3.78 times greater in patients with c-ANCA than with p-ANCA.2 There is a five year survival rate of sixty eight 68%.1 Sixty nine (69%) patients had complete resolution of their respiratory function and 24% had persistent alterations.1 The cornerstone of treatment for MPA is medical and includes induction therapy corticosteroids, cyclophosphamide, plasmapharesis and factor VIIa.
CONCLUSION:Massive hemoptysis is a catastrophic initial presentation of Microscopic Polyangiitis. It’s imperative to have it as a differential diagnosis in massive hemoptysis as the treatment modality is medical rather than surgical as in other cases. Initiating the use corticosteroids, factor VIIa and plasmapharesis can make the difference in a successful resuscitation.
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