Dupilumab-induced Eosinophilic Granulomatosis with Polyangiitis Complicated by Peripheral Neuropathic Pain: a Case Report and Literature Review

Abstract

Purpose: Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare vasculitis characterized by increased eosinophils in human tissues and peripheral blood. In this case, we present a 53-year-old female patient with EGPA. By this case and literature review, we want to explain the early manifestations, diagnosis, and management of EGPA, which will help clinicians to understand the disease and attach importance to the possibility of dupilumab-induced EGPA, to improve the early diagnosis rate of EGPA, and reduce misdiagnosis and missed diagnosis.

Methods: The diagnostic criteria for EGPA established by the American Rheumatology Association (ACR) in 2022 were used; these criteria encompass clinical presentation, laboratory tests, and pathological biopsy. In addition, we conducted a comprehensive literature review on this case.

Result: We present a 53-year-old female patient who developed severe peripheral neuropathic pain after the administration of dupilumab for the treatment of refractory asthma and sinusitis. The patient's symptoms, laboratory examination findings, and nasopharyngeal biopsy pathology results collectively support the diagnosis of EGPA. When dupilumab was converted to mepolizumab combined with glucocorticoid, her peripheral neuropathic pain and asthma symptoms were dramatically relieved. Our literature review also provides a detailed discussion on the relationship between Dupilumab and EGPA.

Conclusion: We present a case of EGPA with peripheral neuropathic pain induced by Dupilumab, and mepolizumab has a good therapeutic effect on this patient. Our literature review shows that although dupilumab is effective in treating eosinophilic airway inflammatory diseases, clinicians must pay attention to the possibility of dupilumab inducing or aggravating EGPAs.

Keywords: Asthma; Dupilumab; Eosinophilic Granulomatosis with Polyangiitis (EGPA); Mepolizumab; Neuralgia; Sinusitis.

Fig. 1

Cranial computed tomography angiography (CTA) showed that the anterior, middle, and posterior arteries of the double-layer brain were hardened, with sinusitis attached (A, B). Cervical vertebra computed tomography plain scan suggests: 1. Disc herniation at C3/4, C4/5, C5/6, and C6/7 levels and spinal canal stenosis. 2. Degenerative osteoarthritis of the cervical spine (C).

Fig. 2

Chest CT: Slight thickening of the interlobular septa in both lungs, with multiple patchy areas of ground-glass opacity in a map-like pattern, most prominent in the apical segment of the right lung, with indistinct borders. Multiple small solid patches and nodules are adjacent to the bronchi in both lungs. Bilateral bronchial wall thickening and luminal narrowing, with some lumens filled with high-density shadows (A). Superficial ultrasound: localized increased echogenicity in the subcutaneous muscle layer of the left lower limb (B). Electromyography (EMG): Abnormal function of the right facial nerve conduction pathway (C). Laryngoscope: Mild redness and swelling of the bilateral nasal mucosa, with no obvious neoplasm observed (D). Nasopharyngeal tissue pathology biopsy: Chronic mucosal inflammation with extensive lymphoplasmacytic infiltration and eosinophilic infiltration (approximately 40 per high-power field in hotspot areas), small vessel proliferation with no necrosis observed, or granuloma formation. Immunohistochemistry: CD68+, CD163+ (E).