Boharoon Hessa, Tomlinson James, Limback-Stanic Clara, Gontsorova Anastasia, Martin Niamh, Hatfield Emma, Meeran Karim, Nair Ramesh, Mendoza Nigel, Levy Jeremy, McAdoo Steve, Pusey Charles, Wernig Florian
Department of Endocrinology, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK.
Renal Unit, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK.
J Endocr Soc. 2020 Apr 21;4(6):bvaa048. doi: 10.1210/jendso/bvaa048. eCollection 2020 Jun 1.
The acute presentation of immunoglobulin G4 (IgG4)-related hypophysitis can be indistinguishable from other forms of acute hypophysitis, and histology remains the diagnostic gold standard. The high recurrence rate necessitates long-term immunosuppressive therapy. Rituximab (RTX) has been shown to be effective in systemic IgG4-related disease (IgG4-RD), but experience with isolated pituitary involvement remains limited.
We report 3 female patients with MRI findings suggestive of hypophysitis. All patients underwent transsphenoidal biopsy and fulfilled diagnostic criteria for IgG4-related hypophysitis. Treatment with glucocorticoids (GCs) resulted in good therapeutic response in Patients 1 and 2, but the disease recurred on tapering doses of GCs. GC treatment led to emotional lability in Patient 3, necessitating a dose reduction. All 3 patients received RTX and Patients 2 and 3 received further courses of treatment when symptoms returned and B-cells repopulated. Patient 3 did not receive RTX until 12 months from the onset of symptoms. Patient 1 was not able to have further RTX treatments due to an allergic reaction when receiving the second dose. Rituximab treatment resulted in sustained remission and full recovery of anterior pituitary function in Patients 1 and 2, with complete resolution of pituitary enlargement. By contrast, Patient 3 only showed a symptomatic response following RTX treatment, but pituitary enlargement and hypofunction persisted.
Rituximab treatment for IgG4-related hypophysitis resulted in sustained remission in 2 patients treated early in the disease process but only achieved partial response in a patient with chronic disease, suggesting that early therapeutic intervention may be crucial in order to avoid irreversible changes.
免疫球蛋白G4（IgG4）相关性垂体炎的急性表现可能与其他形式的急性垂体炎难以区分，组织学仍是诊断的金标准。高复发率需要长期免疫抑制治疗。利妥昔单抗（RTX）已被证明对系统性IgG4相关疾病（IgG4-RD）有效，但孤立性垂体受累的经验仍然有限。
我们报告了3例MRI表现提示垂体炎的女性患者。所有患者均接受了经蝶窦活检，并符合IgG4相关性垂体炎的诊断标准。糖皮质激素（GCs）治疗使患者1和患者2获得了良好的治疗反应，但在GCs剂量逐渐减少时疾病复发。GCs治疗导致患者3出现情绪不稳定，需要减少剂量。所有3例患者均接受了RTX治疗，患者2和患者3在症状复发且B细胞重新出现时接受了进一步的疗程治疗。患者3直到症状出现12个月后才接受RTX治疗。患者1在接受第二剂时因过敏反应无法继续接受RTX治疗。利妥昔单抗治疗使患者1和患者2获得了持续缓解，垂体前叶功能完全恢复，垂体肿大完全消退。相比之下，患者3在RTX治疗后仅出现症状性反应，但垂体肿大和功能减退持续存在。
利妥昔单抗治疗IgG4相关性垂体炎，在疾病早期接受治疗的2例患者中导致了持续缓解，但在1例慢性病患者中仅取得了部分反应，这表明早期治疗干预对于避免不可逆变化可能至关重要。
