Chetram, Deandra

Abstract Introduction EGPA is diagnosed clinically in patients with asthma, rhinosinusitis, and eosinophilia with end-organ involvement including neuropathy, lung infiltrates, cardiomyopathy, or kidney, gastrointestinal, or skin involvement. The five-factor score (FFS) differentiates non-severe (FFS = 0) and severe (FFS > 0) EGPA. Forme fruste, or partially treated, EGPA occurs when presumed severe eosinophilic asthma is treated with corticosteroids that can mask end organ damage typical of EGPA. We present a case of forme fruste non-severe EGPA that failed established treatments for non-severe and severe EGPA. Case Presentation This is a 38-year-old female presenting to the pulmonology clinic with three years of cough, dyspnea, wheezing, rhinorrhea, and chest heaviness. She was initially given prednisone for adult-onset asthma with improvement but was unable to taper due to symptom recrudescence on doses lower than 20 milligrams. Allergic bronchopulmonary aspergillosis testing was negative, fractional exhaled nitric oxide was elevated, pulmonary function testing showed moderate airway obstruction, and absolute eosinophil count was 11x10^3/microliter despite prednisone use. Chest computed tomography (CT) showed mild bronchial wall thickening. She was given benralizumab followed by mepolizumab, inhaled corticosteroid with a long-acting beta agonist, cetirizine, nasal budesonide, and two cycles of rituximab for possible EGPA at an outside hospital. At the current evaluation, Chest CT showed multiple nodules, patchy ground glass opacities, and bronchial wall thickening. CT sinuses showed paranasal sinus mucosal thickening with sinus outlet obstruction and nasal endoscopy showed polyps. Anti-neutrophil cytoplasmic antibody was negative, urinalysis showed mild hematuria, and bronchoalveolar lavage was negative for infection. Bone marrow did not suggest neoplasm and hematology workup did not identify other causes of hypereosinophilia. EGPA was considered highest on the differential. She was offered sinus surgery and lung biopsy for enrollment in refractory EGPA trials, but no further medical therapy was identified. Discussion This case shows the importance of recognizing potential forme fruste EGPA rather than asthma refractory to systemic glucocorticoids. Early suspicion is crucial to initiation of the proper regimen. Non-severe EGPA warrants treatment with corticosteroids and mepolizumab. Azathioprine and cyclophosphamide have demonstrated a comparable benefit in relapsing non-severe EGPA after steroid treatment, but in a single low power study. In our patient, who was refractory to glucocorticoids with mepolizumab and rituximab, there is no strong data suggesting further treatment not involving high dose steroids. In her case, chronic steroid use led to morbid obesity and obstructive sleep apnea, highlighting the importance of further research in non-severe EGPA treatment.

This is a case of a young woman who developed neurological and psychiatric symptoms 3 days after resection of an immature teratoma. She was diagnosed with anti-NMDA receptor encephalitis via positive serum antibody titres, which was later confirmed with cerebrospinal fluid antibody titres. Given her cancer diagnosis, she underwent treatment with bleomycin, etoposide and cisplatin chemotherapy in addition to 5 days of high-dose steroids (1 g of intravenous methylprednisolone) for the encephalitis. This treatment regimen led to significant clinical improvement 3 weeks after completion of one cycle of chemotherapy.