Abdominal Panniculitis Preceding Presentation of Granulomatosis With Polyangiitis by 2 Years

Abstract

SESSION TITLE: Pulmonary Manifestations of Systemic Disease 4 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Granulomatosis with polyangiitis (GPA) is a multi-vessel vasculitis characterized by disseminated necrotizing inflammation involving small blood vessels and their surrounding tissues. While 30-46% of patients present with dermatological manifestations such as papules, palpable purpura, vesicles, subcutaneous nodules, plaques and ulcers, an initial presentation of panniculitis preceding GPA is rare. Here, we present a patient with GPA who had been diagnosed with panniculitis two years earlier. CASE PRESENTATION: A 67-year-old female nonsmoker with past medical history of diabetes mellitus, hyperlipidemia, hypertension, goiter, and arthritis presented to the emergency department (ED) for acute hypoxemia. SpO2 was 90% on 3L/min. She complained of dyspnea, wheezing, night sweats, epistaxis, hemoptysis, and fevers of 99-101°F. Increased work of breathing was noted on exam with diminished breath sounds diffusely. Chest x-ray showed diffuse bilateral alveolar infiltrates (Fig A) with slightly blood tinged secretions seen on bronchoscospy. Urinalysis revealed proteinuria (1 g/L) with 11-20 RBC/hpf, creatinine 0.97 mg/dL. c-ANCA (1:640 titer) and proteinase-3 antibodies (62.8 AI) were positive. Renal biopsy confirmed GPA with focal necrotizing and crescentic, pauci-immune glomerulonephritis. Two years earlier, this patient was admitted to the hospital with complaints of nonspecific abdominal discomfort, fluctuating fevers, nausea, and vomiting. On physical exam, there was mild tenderness to palpation throughout the abdomen. CT abdomen/pelvis demonstrated subtle haziness in the mesentery (Fig B) with a few slightly prominent mesenteric lymph nodes. Imaging combined with the patient’s complaints and elevated transaminases (AST 68 U/L, ALT 70 U/L) suggested a clinical diagnosis of mesenteric panniculitis. No further workup was done. Treatment was supportive and she was discharged from the hospital. Transaminases had returned to normal three months later. DISCUSSION: While it is common for patients with GPA to have skin manifestations, it is unusual for panniculitis to precede a presentation of GPA. Biopsies of affected regions are diagnostic, demonstrating vasculitis or inflammatory infiltrates. A similar case of a patient with biopsy-proven panniculitis of the breast who then went on to be diagnosed with GPA seven years later had occured. Patients with isolated cutaneous signs of panniculitis may benefit from ANCA testing with close follow-up hence aiding in early diagnosis of GPA in the future. CONCLUSIONS: We stress the rarity of GPA presenting as isolated panniculitis as seen in our patient and raise the question of whether it is justifiable to check ANCA levels in patients' with isolated panniculitis. Reference #1: Chowdhury, M.M.U. and Natarajan, S. (2000), Panniculitis of the breast preceding presentation of Wegener's granulomatosis by 7 years. International Journal of Dermatology, 39: 312-320. doi:10.1046/j.1365-4362.2000.00930-1 DISCLOSURE: The following authors have nothing to disclose: Lakshmi Kallur, Nanette Bentley, Adel El Abbassi No Product/Research Disclosure Information