J Rheum Dis.  2015 Jun;22(3):195-199. 10.4078/jrd.2015.22.3.195.

Pituitary Granulomatosis with Polyangiitis Presenting with Central Diabetes Insipidus

• Kim IY
• Lee S
• Kwon HJ
• Lee K
• Yoo KH
• Cha HS
• Koh EM
• Lee J

• KMID: 2222887
• DOI: http://doi.org/10.4078/jrd.2015.22.3.195

Abstract

We report on a case of limited form of granulomatosis with polyangiitis (GPA) with pituitary involvement which presented with central diabetes insipidus. This rare form of GPA has not been reported in Korea. The patient presented with fever, headache, productive cough, nasal symptoms, and polyuria. Laboratory data and imaging studies demonstrated inflammatory lesions in nasal sinus and lungs. Pituitary stalk thickening and enhancement were observed on brain magnetic resonance imaging. The histopathology of the lung lesions showed chronic active granulomatous inflammation. Polyuria, hyperosmolar hypernatremia, and decreased urine osmolality which responded to synthetic vasopressin analog were consistent with central diabetes insipidus. Based on the clinical findings and histopathological results, a diagnosis of GPA with pituitary involvement was established. Treatment with desmopressin as well as concurrent glucocorticoids and immunosuppressant resulted in clinical improvement.

Keyword

Granulomatosis with polyangiitis; Neurogenic diabetes insipidus; Pituitary gland

MeSH Terms

Brain
Cough
Deamino Arginine Vasopressin
Diabetes Insipidus, Neurogenic*
Diagnosis
Fever
Glucocorticoids
Headache
Humans
Hypernatremia
Inflammation
Korea
Lung
Magnetic Resonance Imaging
Osmolar Concentration
Pituitary Gland
Polyuria
Vasopressins
Deamino Arginine Vasopressin
Glucocorticoids
Vasopressins

Figure

• 

  Figure 1. (A) Chest radiograph shows multifocal patchy consolidations in both lungs. (B) Chest computed tomography demonstrated bilateral infiltrations along the bronchovascular bundles (arrow).

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  Figure 2. (A, B) Baseline brain magnetic resonance imaging (MRI) of T1 weighted image with contrast showing thickening and enhancement of pituitary stalk (arrows). Leptomeningeal and pa-chymeningeal involvement of left hemisphere are also seen (arrow head). (C, D) Follow-up MRI shows resolustion of pituitary stalk thickening and enhancement (arrows), as well as pachy-meningeal enhancement.

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  Figure 3. Histopathological finding of the lung biopsy. Microscopic necrosis and ill-defined granuloma formations are demonstrated (H&E, ×400).

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