Rash and Pulmonary Infiltrates in a 37-Year-Old Woman

Abstract

SESSION TITLE: Global Case Report Poster - Miscellaneous SESSION TYPE: Global Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Eosinophilic Granulomatosis with Polyangitis (EGPA, formerly known as Churg Strauss) is a small-medium sized granulomatous vasculitis characterized by late onset asthma, peripheral eosinophilia and systemic vasculitis. It is rare among the vasculitidies, with incidence estimated at approximately 3 new cases per 1 million people per year.1 We present a case of EGPA in a 37-year old woman who presented with a rash and pulmonary infiltrates. CASE PRESENTATION: A 37 year-old woman with a history of asthma presents to the ED with a pruritic rash of approximately 5 days duration. The rash began on her forehead and spread to her extremities. Three weeks prior, the patient had been diagnosed with an upper respiratory tract infection and treated with multiple courses of oral antibiotics without improvement. The patient endorsed fevers, night sweats, cough and mild dyspnea on exertion for approximately 4 weeks. Her past medical history was significant for adut onset asthma, which she had been diagnosed with a year prior and was well controlled on a daily salmeterol-fluticasone inhaler. Her physical examination revealed diffuse rales on lung auscultation and purple papular lesions on forehead and on dorsal hands and feet. On laboratory evaluation, the patient had a leukocytosis to 12.4 ×109 per L, 27.3% of which were eosinophils. A complete metabolic panel was normal. A rheumatologic evaluation was initiated, including ANCA titers and ANA, which was normal. An IgE level was elevated at 6266 IU/mL (normal <100 IU/mL). Chest x-ray demonstrated bilateral infiltrates, and subsequent chest CT demonstrated multifocal ground glass opacities throughout all lobes. A punch biopsy of one of the skin lesions was performed, as well as bronchoscopy. Bronchaolveolar lavage yielded a striking eosinophilia (750 eos), and transbronchial biopsies demonstrated heavy interstitial and perivascular infiltration of eosinophils. Skin biopsy additionally demonstrated fibrinous degeneration and necrosis of medium sized vessels. The patient was subsequently diagnosed with EGPA. Further evaluation for more widespread organ damage, including urinalysis and an echocardiogram, were normal and the patient was discharged home with steroids. Notably, her eosinophil percentage on the day of discharge was 49.6%. DISCUSSION: This case well illustrates EGPA. Patients classically experience a prodromal phase, which can last months to one year, and is characterized by a diagnosis of asthma. This is followed by the eosinophilic phase, as eosinophils begin to infiltrate various tissues. During the eosinophilic phase, patients may experience symptoms akin to sinusitis. Other organs can be affected, including myocardium and the gastrointestinal tract. Lastly, the patient progresses to the vasculitic phase, which is characterized by widespread manifestations of systemic vasculitis such as skin lesions (as in our patient) and neuropathies.1 EGPA is currently diagnosed based on criteria proposed by the American College of Rheumatology; those criteria include 1) asthma, 2) eosinophilia > 10%, 3) neuropathy, 4) pulmonary infiltrates, 5) sinusitis, and 5) eosinophilic infiltration on biopsy. The presence of 4/6 of these criteria carry a sensitivity of 85% and a specificity of 99.7% in diagnosing EGPA.2 Treatment of this condition is based on the presence of end organ damage. Patients without significant end organ damage- myocardial, kidney, central nervous system or gastrointestinal involvement- may be treated with steroids alone. Patients that do have evidence of more widespread disease also require cyclophosphamide therapy.3 Some patients with EGPA may achieve remission following steroids alone, but many relapse and treatment of these patients is controversial. CONCLUSIONS: Eosinophilic Granulomatosis with Polyangitis (EGPA) is a granulomatous vasculitis characterized by late onset asthma, eosinophilia and systemic vasculitis. Reference #1: Greco A, Rizzo MI, De Virgilio A, et al. Churg-Strauss syndrome. Autoimmun Rev. 2015;14(4):341-348. Reference #2: Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA AW. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome. Arthritis Rheum. 1990;33:1094-1100. Reference #3: Gayraud M, Guillevin L, Cohen P, et al. Treatment of good-prognosis polyarteritis nodosa and Churg-Strauss syndrome: comparison of steroids and oral or pulse cyclophosphamide in 25 patients. French Cooperative Study Group for Vasculitides. Br J Rheumatol. 1997;36:1290-1297. DISCLOSURE: The following authors have nothing to disclose: Bethany Weiler-Lisowski, Janaki Deepak, William Twaddell, Allen Burke No Product/Research Disclosure Information