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A3073 - Granulomatosis with Polyangiitis in a Patient with Non Specific Systemic Signs
Author Block: R. Samannan1, Z. Smith2, J. Keddissi3; 1Internal Medicine/Pediatrics, University of Oklahoma Health Sciences Center, Oklahoma City, OK, United States, 2Internal Medicine, University of Oklahoma Health Sciences Center, Oklahoma City, OK, United States, 3Pulmonary and Critical Care, University of Oklahoma Health Sciences Center, Oklahoma City, OK, United States.
Introduction: Granulomatosis with Polyangiitis (GPA), an ANCA associated vasculitis (AAV) variant, is a rare autoimmune disease of unknown cause, predominantly affecting upper and lower respiratory tracts and Kidney. We present a patient who presented nonspecific systemic signs and weight loss who was diagnosed and successfully treated for GPA. Case Description: 68-year-old Caucasian male, a heavy smoker, presented with unintentional weight loss of 20 lbs. severe back pain. Symptoms started 3 months prior with progressive fatigue, night sweats, appetite loss and swelling of extremities. Vitals signs were normal. Positive findings on physical exam included nasal bleeding, distended jugular vein and decreased breath sounds on his left lower lung base, distant heart sounds and 2+ pitting edema up to legs with no focal neurological findings. Labs showed leukocytosis at 21000 cells/cu.mm, normochromic, normocytic anemia with hemoglobin at 12 gm/dl, thrombocytosis at 470000 cells/cu.mm and elevated inflammatory markers. Urinalysis was normal. Metabolic profile showed mild hyponatremia with otherwise normal parameters. CT chest which showed multiple sub centimeter nodules on bilateral lung fields, moderate sized left pleural effusion and moderate pericardial effusion. Pericardial and thoracentesis was done draining about 700 cc of sanguineous fluid in each cavity. Effusion analysis showed exudative effusion with no malignant cells. Pleural biopsy was negative for granulomatous inflammation/infections or malignancy. Extensive evaluation for infectious etiology including fungal, tubercular etiologies were negative. Autoimmune work up revealed positive ANA and CANCA (1: 320 dilution) and elevated PR3 Ab. (> 100 U/mL). Ophthalmological exam was normal. He was diagnosed with Granulomatosis with polyangiitis and was stared on pulse dose steroids and Rituximab with resolution of symptoms and no recurrence of pleural or pericardial effusion. Since discharge he has been on Rituximab infusion every 6 months with complete resolution of pulmonary and normalization of PR3. Discussion: GPA is a necrotizing granulomatous inflammation of small-medium sized blood vessels with absence of immune deposition. It is associated with anti MPO or PR3 ANCA. The incidence is estimated at about 2.1/million population with increased risk in elderly males. The use of newer therapies has improved the dire prognosis of untreated AAV (>80% mortality at 1 year) to one of where long-term remission was possible. Treatment is long term and consists of induction and maintenance phase with glucocorticoids and Rituximab. Conclusion:The relative rarity and non-specific presentation of the AAV pose diagnostic challenges, therefore, a very careful and systematic approach is required to make the diagnosis