Research Article
Hypophyseal Involvement in Immunoglobulin G4-Related Disease: A Retrospective Study from a Single Tertiary Center
Table 1
Clinical characteristics of ten patients with IgG4-related hypophysitis.
| ID | Age/sex | Onset age of IgG4-RH (years) | Onset age of IgG4-RD (years) | First symptoms | Serum IgG4 (mg/dl) | Other systems involved | MRI | Biopsy | Histopathology | Pituitary function | Prompt response to GC | Thickened stalk | Pituitary mass | AH | CDI |
| 1 | 64/M | 62.4 | 62.4 | DI | 199 | − | + | + | N/D | N/D | + | + | + | 2 | 61/M | 60.4 | 60.4 | Fatigue, anorexia | 4680 | Lymph nodes; paranasal sinus | + | + | Paranasal sinus mass | IgG4+/IgG+ > 40%, IgG4+ > 50/HPF | + | + | + | 3 | 58/M | 50.1 | 48.6 | Swollen eyelids | 5410 | Submandibular gland; lacrimal gland; parotid gland; retroperitoneum | + | − | N/D | N/D | + | + | + | 4 | 51/M | 48.3 | 48.3 | DI | 1980 | Submandibular gland; parotid gland; retroperitoneum | + | − | Submandibular mass | IgG4+ 20/HPF | − | + | + | 5 | 46/M | 43.3 | 43.3 | DI | 327 | Lymph nodes | + | − | Lymph node | IgG4+/IgG+ > 40%, IgG4+ > 100/HPF | + | + | + | 6 | 44/M | 44.2 | 26.4 | Swollen eyelids | 1910 | Pancreas; lacrimal glands; thyroid gland | + | − | Pancreas | AIP | − | + | + | 7 | 36/M | 36.4 | 20.6 | Submandibular mass | 2470 | Submandibular gland; lacrimal gland; parotid gland; pericardium | + | − | Lacrimal mass | IgG4+/IgG+ > 40%, IgG4+ > 50/HPF | − | − | + | 8 | 57/F | 51.5 | 49.4 | Cough | 2250 | Lacrimal gland; lung; kidney | + | − | Lacrimal mass | IgG4+ > 20/HPF | − | + | + | 9 | 16/F | 15.8 | 15.7 | Submandibular mass | 43.7 | Submandibular gland; parotid gland | + | − | Submandibular mass | IgG4+/IgG+ < 40%, IgG4+ 8/HPF | + | + | n/a | 10 | 50/M | 49.0 | 42.0 | Proptosis | 426 | Submandibular gland; parotid gland; lung; kidney; large artery; lymph nodes; thyroid gland; paranasal sinus; extraocular muscles | − | + | Pituitary mass | Inflammatory pseudotumor, lymphocyte infiltration, IgG(+), IgG4(−) | + | − | n/a |
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IgG4-RH/-RD: IgG4-related hypophysitis/disease; DI: diabetes insipidus; N/D: not determined; n/a: not available; IgG+/IgG4+: IgG-/IgG4-positive plasma cell; AH: anterior hypopituitarism; CDI: central DI; GC: glucocorticoid.
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