Eosinophilic granulomatosis polyangiitis (egpa) presenting as eosinophilic myositis in a patient with fibrotic interstitial lung disease

SESSION TITLE: Pulmonary Manifestations of Systemic Disease Case Report Posters 8 SESSION TYPE: Case Report Posters PRESENTED ON: 10/09/2023 12:00 pm - 12:45 pm INTRODUCTION: Eosinophilic granulomatosis polyangiitis (EGPA) is a rare small vessel necrotizing vasculitis which can cause multiorgan disease. Reports of myositis associated with EGPA are rare. Numerous case reports have described and association between fibrotic interstitial lung disease (fILD) and ANCA-associated vasculitis; however, to our knowledge none of these cases have been EGPA. CASE PRESENTATION: A 57-year-old male former smoker, with a history of idiopathic thrombocytopenic purpura (ITP), and a diagnosis of pulmonary fibrosis presented to advanced lung disease clinic for worsening shortness of breath. The patient's fILD was diagnosed in 2014 by open lung biopsy which revealed a usual interstitial pneumonia pattern. Serologic evaluation demonstrated a positive ANA at 1:160 and a weakly positive SSA. No systemic manifestations of connective tissue disease were present. Although the patient had interstitial pneumonitis with autoimmune features, a diagnosis of idiopathic pulmonary fibrosis was made and pirfenidone therapy was started. The patient had stable pulmonary function testing (PFT) and symptoms until the summer of 2022 when he reported worsening dyspnea, fatigue, and leg pain. Repeat PFT and CT revealed stable fILD; however, six-minute walk testing revealed a marked decline in distance and elevated heart rate. The patient reported swelling, tenderness, and weakness in the legs. Vascular studies were negative for lower extremity clots or arterial insufficiency. Creatine Kinase was normal, but aldolase was elevated. EMG testing was consistent with a diffuse bilateral proximal myopathy. Muscle biopsy of the left thigh revealed necrotizing arteritis with eosinophils, but no evidence of endomysial inflammation. Anti-MPO and p-ANCA (1:320) were elevated, supporting a diagnosis of EGPA and eosinophilic myositis. Myositis specific antibody panel was negative. The patient denied wheezing or asthma symptoms. The patient had significant improvement in myositis related symptoms with high dose prednisone. Meplolizumab was initiated with rapid tapering of the prednisone. The patient's respiratory symptoms remained stable. DISCUSSION: EGPA is a multiorgan disease that typically presents with asthma, eosinophilic infiltration of blood and tissue, and vasculitis. The clinical presentation most often involves the lung and the skin but can affect any organ system. Symptoms generally include prodromic wheezing, shortness of breath, pulmonary opacities, asymmetrical neuropathy (mononeuritis multiplex), and sinusitis. Clinically EGPA may be suspected when asthma is difficult to control, associated with chronic rhino sinusitis, and eosinophilia > 1500/uL. Laboratory testing is associated with anti-neutrophil cytoplasmic antibodies in nearly half of patients with EGPA, the majority of which are directed against myeloperoxidase with a perinuclear staining pattern (MPO-ANCA, P-ANCA). Antibodies to proteinase-3 are not typical in EGPA. While asthma is often a cardinal symptom of EGPA, it is not essential to diagnosis. Myositis has also previously reported albeit rare presentation of EGPA. In our case, numerous features were highly atypical including the absence of asthma symptoms, associated myositis, and the associated fibrotic ILD. CONCLUSIONS: EGPA may rarely present without asthma symptoms and may be associated with myositis. fILD has been reported in association with other ANCA-associated vasculitides; however, this is the first reported case of fILD and EGPA to our knowledge. The relationship between fILD and EGPA remains uncertain. REFERENCE #1: Woo K, Park YE, Jeon D, Shin JH. A Case of Myositis Preceding Neuropathy in Eosinophilic Granulomatosis with Polyangiitis. J Clin Neurol. 2020 Oct;16(4):717-719. doi: 10.3988/jcn.2020.16.4.717. PMID: 33029986; PMCID: PMC7542004. 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