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Table 2 Clinical characteristic of relapsing of Immunoglobulin G4 (IgG4)-related hypophysitis during steroid therapy

From: Clinical course of IgG4-related hypophysitis presenting with focal seizure and relapsing lymphocytic hypophysitis

Author, year

Sex, age, race

Manifestations before steroid treatment

Steroid type and dosage

Symptoms and signs after steroid treatment

Treatment after relapse and result

Taniguchi, 2006 [11]

M 75 y, Japanese

Autoimmune pancreatitis, uveitis, organizing pneumonia, panhypopituitarism with mass (Lymphocytic hypophysitis)b

Pred 50 mg/day for 1 week then taper to 10 mg/day within 7 months

Recurrent pituitary mass and organizing pneumonia

Pred 50 mg/day then 20 mg/day, contracted pituitary mass

Haragushi, 2010 [18]

M 68 y, Japanese

Diabetes insipidus and gradual loss of anterior pituitary function with mass (Lymphocitic hypophysitis)b, retroperitoneal fibrosis

Hydrocortisone replacement for 4 years

Headache, pituitary swelling

Pred 30 mg/day for 2 weeks then 10 mg/day, decrease pituitary swelling

Leporati, 2011 [2]

M 75 y, Caucasian

Panhypopituitarism with mass, sphenoid mass

Pred 40 mg/day taper to 10 mg/day over 4 weeks

Recurrent headache

Pred 15 mg/day, then taper/reescalation and suspend until 1.3 years, improved headache but hypopituitarism

Caputo, 2014 [27]

M 40 y, Vietnamese

Lacrimal gland mass, diabetes insipidus, panhypopituitarism with mass (Lymphocytic hypophysitis)a, enlarged infraorbital nerve

Pred 30 mg/day for 3 months

Enlarging pituitary mass with new optic nerve compression

Azathioprine 75 mg twice daily whilst weaning Pred for 10 months, recovery from adrenal insufficiency and growth hormone deficiency, ongoing bilaterally enlarged infraorbital nerves but normal pituitary size

Ohkubo, 2013 [6]

M 70 y, Japanese

Hashimoto’s thyroiditis, pancreatic and retroperitoneal mass, salivary gland enlargement, pituitary mass, bitemporal hemianopsia (Lymphocytic hypophysitis)a

Pred 40 mg/day for 2 weeks then taper to 5 mg/day

Reduction in pituitary lesion, improved vision but new DI and panhypopituitarism

Pred 30 mg/day then taper to 5 mg/day for 2 months, no further pituitary mass reduction and did not restore the pituitary function

Hydrocortisone 100 mg on day of surgery + Pred 5 mg/day for 1 month

Ngaosuwan, 2015 (the presented case)

M 43 y, Thai

Frontal lobe seizure, multiple pituitary hormone deficiency with pituitary mass (Lymphocytic hypophysitis)a

Pred 15 mg/day for 6 weeks, 10 mg/day for 3 months, and 7.5 mg/day for 3 months

Bitemporal hemianopsia, Headache, Inflammation of optic chiasm

Pred 60 mg/day for 2 weeks, gradually decrease to 30 mg/day for 4 weeks, 20 mg/day for 4 weeks, and 10 mg/day for maintenance, complete recovery of vision, contracted pituitary mass, decreased optic chiasmatic swelling, but did not restore pituitary function

  1. Pred Prednisolone, mg milligrams
  2. aInitial diagnosis based on initial histopathology
  3. bInitial diagnosis based on clinical manifestation and imaging