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

 

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

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Yuki
Tsutsumi
Yuki Tsutsumi tsutsumi.yuki.83z@st.kyoto-u.ac.jp Kyoto University Department of Nephrology Kyoto Japan *
Shinya Yamamoto syamamon@kuhp.kyoto-u.ac.jp Graduate School of Medicine, Kyoto University Department of Nephrology Kyoto Japan -
Tatsuaki Kosaka tatsuaki_kosaka@outlook.jp Graduate School of Medicine, Kyoto University Department of Nephrology Kyoto Japan -
Koji Muro muro_kogi@kuhp.kyoto-u.ac.jp Graduate School of Medicine, Kyoto University Department of Nephrology Kyoto Japan -
Naoko Yasugi naoko_yasugi@kuhp.kyoto-u.ac.jp Graduate School of Medicine, Kyoto University Department of Nephrology Kyoto Japan -
Sayaka Sugioka sayaka_113@kuhp.kyoto-u.ac.jp Graduate School of Medicine, Kyoto University Department of Nephrology Kyoto Japan -
Motoko Yanagita motoy@kuhp.kyoto-u.ac.jp Graduate School of Medicine, Kyoto University Department of Nephrology Kyoto Japan -
 
 
 
 
 
 
 
 

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis often preceded by asthma or allergic rhinitis. Although its symptoms overlap with other ANCA-associated vasculitides, central diabetes insipidus (CDI) is rarely reported in EGPA. CDI may arise from various conditions, including other vasculitides such as granulomatosis with polyangiitis (GPA). Here, we present a rare case of EGPA with preexisting CDI and severe kidney impairment, which improved with multidisciplinary treatment.We conducted a literature review of cases of concurrent EGPA and CDI to investigate the clinical course of both conditions and the outcomes of CDI following treatment for EGPA.

A 68-year-old woman with asthma and eosinophilic sinusitis was diagnosed with CDI 17 months before admission, based on hypotonic urine, low AVP response, and loss of posterior pituitary signal on MRI, with no structural abnormalities. She later developed general malaise, weight loss, elevated creatinine (5.21 mg/dL), eosinophilia, and MPO-ANCA positivity. Kidney biopsy revealed eosinophil-rich necrotizing vasculitis with crescents. EGPA was diagnosed and treated with steroids, rituximab, and plasma exchange.  After starting treatment, the creatinine level improved to 2.0 mg/dL, and the patient was withdrawn from hemodialysis. Furthermore,  desmopressin discontinuation was successfully achieved. Follow-up MRI showed slight signal recovery in the posterior pituitary. A repeat hypertonic saline test showed mild improvement in AVP secretion.

This case highlights a rare occurrence of EGPA developing after CDI, suggesting a possible pathophysiological link. While EGPA is known for multiorgan involvement, posterior pituitary dysfunction is extremely uncommon. Among reported cases of EGPA with CDI, clinical courses varied, but all showed improvement with immunosuppressive therapy. In our case, slight recovery of posterior pituitary MRI signal and AVP secretion was observed after treatment. Although the precise mechanism remains unclear, the absence of eosinophilia and pituitary enlargement at CDI onset, along with the subsequent response to immunosuppression, suggests that vasculitic ischemia, rather than eosinophilic infiltration, may have contributed to CDI. A direct pathological link could not be confirmed, and idiopathic CDI cannot be excluded; however, the clinical course raises the possibility that CDI may reflect early central involvement of EGPA. 

Clinicians should suspect EGPA when CDI occurs in patients with asthma or sinusitis, as this may indicate early vasculitic involvement.

Kewords