IgG4-related hypophysitis diagnosed by retroperitoneal mass biopsy in a patient presenting with abducens nerve palsy: A case report (CARE-compliant article).

Abstract

RATIONALE:

Immunoglobulin G4 (IgG4)-related hypophysitis is a rare disorder which often requires invasive pituitary gland biopsy to confirm its diagnosis. We present a case whereby peripheral organ lesion biopsy and imaging findings were sufficient for the diagnosis.

PATIENT CONCERNS:

A 77-year-old man with diplopia was referred to our department by an opthomologist who had diagnosed the patient with right abducens nerve palsy.

DIAGNOSES:

Head magnetic resonance imaging revealed enlargement of the pituitary gland and pituitary stalk, while hormonal analysis revealed panhypopituitarism, thereby indicating a diagnosis of hypophysitis. Abdominal computed tomography imaging revealed a solid mass that encompassed the left kidney ureter. Although the patient did not have an increase in serum IgG4, a biopsy of the periureteral mass revealed infiltrating plasma cells that were positive when stained for IgG4.

INTERVENTIONS:

The patient was given corticosteroid pulse therapy (methylprednisolone: 1 g × 3 days), followed by oral corticosteroids (prednisolone, 0.5 mg/kg/d).

OUTCOMES:

The right abducens nerve palsy improved and the pituitary lesion shrank after the initiation of corticosteroid treatment.

CONCLUSION:

Based on the diagnosis of IgG4-related disease in the retroperitoneal organ and response to corticosteroid treatment, this patient was diagnosed with IgG4-related hypophysitis. This hypophysitis caused enlargement of the pituitary gland with resulting nerve compression, causing abducens nerve palsy. When IgG4-related hypophysitis is suspected, a thorough examination of other organ lesions and biopsy should be considered.

• Authors: Takeshi Imai, Souichirou Shibata, Kensuke Shinohara, Kenzo Sakurai, Masahiro Horiuchi, Kensuke Sakai, Shiko Asai, Yasuhiro Hasegawa
• Journal: Medicine (Medicine (Baltimore)) Vol. 99 Issue 40 Pg. e22484 (Oct 02 2020) ISSN: 1536-5964 [Electronic] United States
• PMID: 33019443 (Publication Type: Case Reports, Journal Article)

Chemical References

• Adrenal Cortex Hormones

Topics

• Abducens Nerve Diseases (etiology)
• Adrenal Cortex Hormones (therapeutic use)
• Aged
• Humans
• Hypophysitis (complications, drug therapy)
• Immunoglobulin G4-Related Disease (complications, drug therapy)
• Male