Hypophysitis in IgG4-Related Disease Associated with p-ANCA Vasculitis Krystallenia I. Alexandraki, MD, PhD, MSc, Maria Kaltsatou, MD, MSc, Eleftherios Chatzellis, MD, Andreas V. Goules, MD, PhD, Georgios Boutzios, MD, PhD, Denise Kolomodi, MD, Gregory A. Kaltsas, MD, PhD, FRCP  The American Journal of Medicine  Volume 129, Issue 6, Pages e25-e27 (June 2016) DOI: 10.1016/j.amjmed.2015.11.021 Copyright © 2016 Elsevier Inc. Terms and Conditions

Figure (A) Pituitary magnetic resonance imaging (MRI) scan before the institution of treatment with corticosteroids. a: Coronal T1 pre-contrast-weighted image. b: Coronal T1 post-contrast-weighted image showing the diffuse but mostly central enlargement of the pituitary gland with in-homogenous contrast uptake. c: Coronal T2-weighted image. d: Sagittal T1-weighted image with intravenous contrast. (B) Repeated post-contrast MRI of the pituitary gland 1 month after treatment with corticosteroids. a: Coronal T1 post-contrast image. b: Sagittal post-contrast T1 image. (C) a, b: T1 pre- and postcontrast axial images at the level of the lateral rectus muscle showing small rather homogenously enhancing mass involving the muscle. c: Histological section of the right retrobulbar mass obtained through transorbital biopsy, haematoxylin and eosin (H&E) stain. d: Immunohistochemical study of the biopsied specimen showing positivity for immunoglobulin (Ig)G deposits. e: IgG4 immunohistochemical stain positive in > 50% of the cells. Overall, this biopsy showed large areas of fibrosis, focally organized in a storiform pattern; concentric fibrosis around vascular channels; patchy lymphocyte and plasma cell aggregates; occasional eosinophils; inconspicuously, neutrophils presence; lymphoplasmacytic cells were noted to infiltrate into the lacrimal glands and, occasionally, germinal centers were present, while the fibrosis was also seen to extend into the lacrimal gland. (D) a: Initial computed tomography of the chest showing triangular-shaped ground-glass opacity at the border of the right upper lung lobe and lateral section of the right middle lobe. b: Repeat chest high resolution after treatment with 2 cycles of 700 mg/month cyclophosphamide and 4 cycles of rituximab∗ 700 mg/week, showing significant improvement with almost complete resolution of the infiltrate. (E) C-reactive protein levels with regards to treatment after the presumptive diagnosis of IgG4-related disease. *One cycle = 4 weekly pulses of 700 mg of rituximab. The American Journal of Medicine 2016 129, e25-e27DOI: (10.1016/j.amjmed.2015.11.021) Copyright © 2016 Elsevier Inc. Terms and Conditions

Figure (A) Pituitary magnetic resonance imaging (MRI) scan before the institution of treatment with corticosteroids. a: Coronal T1 pre-contrast-weighted image. b: Coronal T1 post-contrast-weighted image showing the diffuse but mostly central enlargement of the pituitary gland with in-homogenous contrast uptake. c: Coronal T2-weighted image. d: Sagittal T1-weighted image with intravenous contrast. (B) Repeated post-contrast MRI of the pituitary gland 1 month after treatment with corticosteroids. a: Coronal T1 post-contrast image. b: Sagittal post-contrast T1 image. (C) a, b: T1 pre- and postcontrast axial images at the level of the lateral rectus muscle showing small rather homogenously enhancing mass involving the muscle. c: Histological section of the right retrobulbar mass obtained through transorbital biopsy, haematoxylin and eosin (H&E) stain. d: Immunohistochemical study of the biopsied specimen showing positivity for immunoglobulin (Ig)G deposits. e: IgG4 immunohistochemical stain positive in > 50% of the cells. Overall, this biopsy showed large areas of fibrosis, focally organized in a storiform pattern; concentric fibrosis around vascular channels; patchy lymphocyte and plasma cell aggregates; occasional eosinophils; inconspicuously, neutrophils presence; lymphoplasmacytic cells were noted to infiltrate into the lacrimal glands and, occasionally, germinal centers were present, while the fibrosis was also seen to extend into the lacrimal gland. (D) a: Initial computed tomography of the chest showing triangular-shaped ground-glass opacity at the border of the right upper lung lobe and lateral section of the right middle lobe. b: Repeat chest high resolution after treatment with 2 cycles of 700 mg/month cyclophosphamide and 4 cycles of rituximab∗ 700 mg/week, showing significant improvement with almost complete resolution of the infiltrate. (E) C-reactive protein levels with regards to treatment after the presumptive diagnosis of IgG4-related disease. *One cycle = 4 weekly pulses of 700 mg of rituximab. The American Journal of Medicine 2016 129, e25-e27DOI: (10.1016/j.amjmed.2015.11.021) Copyright © 2016 Elsevier Inc. Terms and Conditions