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• W2230606987 abstract "Hypophysitis is an inflammatory process of the pituitary gland that belongs to the group of nonhormonal secreting sellar masses.1Howlett T.A. Levy M.J. Robertson I.J. How reliably can autoimmune hypophysitis be diagnosed without pituitary biopsy.Clin Endocrinol (Oxf). 2010; 73: 18-21PubMed Google Scholar We report herein a young woman with infertility secondary to hypophysitis as part of a multisystemic disease involving both immunoglobulin G4 (known as IgG4)- and vasculitis-related disorders. A 38-year-old woman presented with a 2-month history of amenorrhea, bilateral galactorrhea, polydipsia, polyuria, temporal headache, and clinically bilateral mild exophthalmos. Endocrine testing revealed high prolactin levels (83 ng/mL; normal: 1.9-25); pituitary magnetic resonance imaging (MRI) showed diffuse pituitary enlargement and the absence of the posterior lobe bright spot (Figure A). Laboratory investigations revealed mild anemia, an underlying autoimmune process (antinuclear antibodies positive: 1/1250; antithyroid peroxidase antibodies: 85 IU/mL, normal: < 35), hypogonadotropic hypogonadism and high-plasma osmolality (303 mOsm/kg, normal: 270-290) suggestive of diabetes insipidus. The patient was started on 40 mg prednisolone/day; 1 month later her menstrual cycle resumed, polyuria and polydipsia improved; pituitary MRI showed partial empty sella and persistent absence of the bright spot (Figure B). Following prednisolone tapering to 10 mg/day, the patient conceived and had an uneventful delivery. Postnatally, she developed blurred vision and worsening of exophthalmos associated with raised C-reactive protein (CRP: 167 mg/L, normal: < 6) levels; MRI of the orbits showed bilateral pseudotumors (Figure C). Further investigations revealed increased serum IgG4 levels (3.770 g/L, normal: 0.03-2), IgG4-to-total IgG ratio > 50, and a subsequent orbital biopsy confirmed the presence of IgG4-related disease.2Leporati P. Landek-Salgado M.A. Lupi I. Chiovato L. Caturegli P. IgG4-related hypophysitis: a new addition to the hypophysitis spectrum.J Clin Endocrinol Metab. 2011; 96: 1971-1980Crossref PubMed Scopus (196) Google Scholar, 3Stone J.H. Zen Y. Deshpande V. IgG4-related disease.N Engl J Med. 2012; 366: 539-551Crossref PubMed Scopus (1873) Google Scholar The prednisolone dose was increased (35 mg/d) with subsequent reduction of CRP levels (11.2 mg/L), but the patient developed a persistent cough. Chest computed tomography revealed focal ground-glass infiltrates of the right middle lobe and multiple pulmonary nodules (Figure D). Combination treatment with prednisolone (25 mg/day) and azathioprine 150 mg/day was initiated in the context of either IgG4-related disease affecting the lungs or another immune-mediated process. However, due to worsening of her cough, an increase in CRP levels (60 mg/L) and the appearance of bilateral inferior lobe fibrosis along with a 3-cm infiltrate of the right medial lobe, treatment was changed to rituximab (4 pulses/week) with methylprednisolone (125 mg), with subsequent tapering of the prednisolone dose. After the second pulse the patient developed high fever and a purpuric rash involving the trunk and the upper/lower extremities. Infection screen was negative; she was found to have raised p-anti-neutrophil cytoplasmic antibody (ANCA) titers (antimyeloperoxidase: 24.4 U/mL, normal: < 10). A skin biopsy was consistent with leukocytoclastic vasculitis. After increasing prednisolone dose (25 mg/day) there was resolution of the purpuric lesions, and 2 further pulses of rituximab were administered, leading to improvement of the lung lesions, suggesting a p-ANCA-associated vasculitic process (Figure D, E). Pituitary involvement is not uncommon in IgG4-related disease, but can also be found in granulomatosis with polyangiitis that mostly affects the posterior rather than the anterior pituitary lobe and can also involve the orbits, albeit mostly unilaterally. However, in our patient, pituitary and orbital involvement are more likely attributed to IgG4-related disease, because retrobulbar biopsy did not favor the diagnosis of granulomatosis with polyangiitis.4Holle J.U. Gross W.L. Neurological involvement in Wegener's granulomatosis.Curr Opin Rheumatol. 2011; 23: 7-11Crossref PubMed Scopus (93) Google Scholar Lung involvement with nodular infiltrates responding to rituximab is commonly seen in granulomatosis with polyangiitis (GPA) that may be associated with raised p-ANCA titers in approximately 10%, as opposed to 70%, of patients with microscopic polyangiitis (MPA).5Seo P. Stone J.H. The antineutrophil cytoplasmic antibody-associated vasculitides.Am J Med. 2004; 117: 39-50Abstract Full Text Full Text PDF PubMed Scopus (328) Google Scholar Purpuric lesions and leukocytoclastic vasculitis could also be attributed to granulomatosis with polyangiitis, rather than a serum sickness-like reaction to rituximab, because its subsequent administration did not reproduce the clinical picture. In conclusion, this is a case of IgG4-related disease manifested by hypophysitis and retrobulbar masses along with a p-ANCA vasculitis expressed with lung involvement, purpura, and acute phase reactants resembling granulomatosis with polyangiitis, highlighting that patients with hypophysitis require increased awareness and prolonged follow-up because histological confirmation is usually not available, and the gland may be involved in the context of an underlying multisystem disease.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.View Large Image Figure ViewerDownload Hi-res image Download (PPT)" @default.
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• W2230606987 title "Hypophysitis in IgG4-Related Disease Associated with p-ANCA Vasculitis" @default.
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