A Rare Case of Eosinophilic Granulomatosis with Polyangiitis Following Development of Central Diabetes Insipidus: A Case Report and Literature Review

Abstract

Central diabetes insipidus (CDI) can develop as an acquired systemic disease. However, cases of eosinophilic granulomatosis with polyangiitis (EGPA) with concurrent CDI are rare. We herein report a case of EGPA following CDI. A 68-year-old woman with a 30-year history of sinusitis and asthma developed CDI, followed by severe renal dysfunction with eosinophilia and elevated myeloperoxidase-antineutrophil cytoplasmic antibody levels. A renal biopsy confirmed EGPA. Intensive treatment resulted in dialysis discontinuation, while partial improvement in arginine vasopressin secretion suggested a potential link between EGPA and CDI. We also conducted a literature review on concurrent EGPA and CDI. Clinicians should be aware of EGPA in CDI patients with asthma or sinusitis.

Keywords: CDI; EGPA; acute kidney injury; central diabetes insipidus; dialysis; eosinophilic granulomatosis with polyangiitis.

Figure 1.

Plain chest computed tomography (CT) findings. Newly identified diffuse consolidations were observed in both lungs on admission (left panel, circles). CT findings on day 37 (post-admission) demonstrating improvement in lung consolidation (right panel).

Figure 2.

Light microscopic findings for the renal biopsy specimen. (a) Cellular crescent with fibrinoid necrosis and rupture of Bowman’s capsule (periodic acid-methenamine-silver stain, ×40). (b) Approximately 50% of the interstitium showed inflammatory cell infiltration, with prominent eosinophilic infiltrates (Hematoxylin and Eosin staining, ×20). (c) Fibrinoid necrosis in the wall of interlobular artery (periodic acid-methenamine-silver stain, ×20). b and c are images of the same location.

Figure 3.

Clinical course. After the diagnosis of eosinophilic granulomatosis with polyangiitis by a renal biopsy, creatinine and C-reactive protein levels decreased following steroid pulse therapy, rituximab, and plasma exchange. Hemodialysis was withdrawn on day 30 of admission.

Figure 4.

Magnetic resonance imaging (MRI) findings. Brain MRI revealed slight signal recovery in the posterior pituitary lobe after treatment (circles).

Figure 5.

Hypertonic saline infusion test conducted five months post-discharge showing mild improvement in arginine vasopressin (AVP) secretion. The 95% prediction intervals for plasma AVP concentrations during a hypertonic saline test are shown by the dotted lines in the control and central diabetes insipidus groups.