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Pituitary Involvement in Granulomatosis with Polyangiitis: A Retrospective Analysis in a Single Chinese Hospital and a Literature Review.

ABSTRACT: Purpose:Granulomatosis with polyangiitis (GPA) is an anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis that can involve virtually many organs, including the pituitary. Pituitary involvement in GPA is rare, with only case reports or small case series published previously. Methods:We used the electronic medical record system in our hospital to identify four patients of pituitary involvement in GPA. We summarized the clinical characteristics, radiographic findings, treatments, and clinical outcomes of the four patients. We further performed a systematic literature review of 66 GPA cases with pituitary involvement that were published on the PubMed database. Results:The four women in our report were between 57 and 73 years of age. All patients had pituitary abnormalities on radiology; three developed diabetes insipidus (DI). All patients had multisystem involvement. After treatment with glucocorticoids and cyclophosphamide (CYC), all patients showed clinical improvement but pituitary function did not resume. Literature review identified 66 additional patients with pituitary involvement in GPA; diabetes insipidus (57/66, 86.4%) and hypogonadism (34/66, 51.5%) were the most frequent pituitary disorders, and the most frequent imaging lesion was an enlarged pituitary (25/64, 39.1%). After treatment with corticosteroids and/or immunosuppressive agents, most patients (45/66, 68.2%) developed remission from systemic disease, 13 patients (13/57, 22.8%) showed remission of DI, and 8 patients (8/46, 17.4%) showed remission of hormone deficiencies. Conclusions:GPA should be carefully considered as a potential cause of pituitary dysfunction (PD), especially when multisystem dysfunction exists. Conventional treatment with corticosteroids and/or immunosuppressive agents improves systemic symptoms, but pituitary disorders persisted in most patients.

SUBMITTER: Liu S

PROVIDER: S-EPMC6874975 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:BackgroundGranulomatosis with polyangiitis (GPA), a necrotizing granulomatous disease, very rarely involves the central nervous system (CNS), particularly the pituitary. Delayed treatment may cause permanent bilateral blindness. We report an isolated case of pituitary GPA that manifested as a progressive bilateral temporal visual field (VF) defect and was diagnosed via pituitary biopsy. Additionally, we review ocular, chiasmal and cranial nerve involvement in pituitary GPA.Case presentationA 20-year-old Chinese man was referred for repeated fever, sudden headache, diplopia with a bilateral best-corrected visual acuity (BCVA) of 10/20, ptosis in both eyes and restricted abduction on the right side. VF tests showed bitemporal hemianopsia. Laboratory tests revealed hypothyroidism and were negative for autoimmune markers. Enhanced magnetic resonance imaging (MRI) showed pituitary enlargement. The diagnosis was lymphocytic pituitaritis. After intravenous (IV) dexamethasone treatment, full recovery occurred within 2 months. Two years later, the patient was readmitted for headache recurrence. With oral prednisone, the visual acuity in his right eye rapidly decreased to hand motion (HM) within one month. Enhanced MRI showed pituitary enlargement and a new, invasive suprasellar CNS lesion. All infection- and autoimmune-related tests were negative. The visual acuity in his right and left eye decreased to no light perception (NLP) after 6 days and 2 weeks, respectively. The biopsy results suggested GPA. After IV methylprednisolone treatment, complete remission of the symptoms occurred and was confirmed by MRI. The 15-month follow-up showed no signs of recurrence.ConclusionGPA typically affects the respiratory tract, lungs and kidneys. To date, 50 cases with pituitary involvement have been reported. Chiasmal and cranial nerve involvement leading to visual acuity impairment are common. We found 2 cases with severe visual loss resembling our case and discuss certain similarities.

Project description:Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare disease associated with vascular inflammation and multisystem organ damage. The literature reporting EGPA incidence or prevalence is limited. We performed a systematic literature review and meta-analysis to describe the incidence, prevalence, and disease burden associated with EGPA. Real-world, observational, English-language studies in MEDLINE, MEDLINE In-Process, and Embase up to 6 June, 2019, were included. A single investigator screened all identified titles/abstracts and extracted data; an additional, independent investigator repeated the screening and validated the extracted data. A random-effects meta-analysis was conducted to generate pooled estimates for EGPA incidence and prevalence. Data from 100 eligible publications were extracted (32 with incidence/prevalence data, 65 with morbidity/healthcare resource data; 3 with both types of data). Significant evidence of between-study heterogeneity for reported incidence (p = 0.0013-0.0016) and prevalence (p = 0.0001-0.0006) estimates was observed. Global and European pooled estimates (95% confidence interval) of EGPA incidence were 1.22 (0.93, 1.60) and 1.07 (0.94, 1.35) cases per million person-years, respectively; global and European pooled estimates (95% confidence interval) for EGPA prevalence were 15.27 (11.89, 19.61) and 12.13 (6.98, 21.06) cases per million individuals, respectively. The proportions of patients experiencing relapses, or who had nasal polyps or severe asthma, varied considerably across studies. EGPA healthcare resource use was high, with inpatient admissions and emergency department visits reported for 17-42% and 25-42% of patients, respectively. Our results indicate that although global and European EGPA incidence and prevalence is low, the associated disease burden is substantial. Key points • We performed a systematic literature review and meta-analysis of real-world, observational studies describing the incidence, prevalence, and disease burden associated with eosinophilic granulomatosis with polyangiitis (EGPA). • Based on meta-analysis data from 35 eligible studies reporting incidence and prevalence, the incidence and prevalence of EGPA were low (globally 1.22 cases per million person-years and 15.27 cases per million individuals, respectively). • Among the 49 studies with morbidity and/or healthcare resource data, most reported a large proportion of patients with EGPA relapses and comorbidities of nasal polyps and severe asthma. • Healthcare resource use was also high among patients with EGPA in these studies, with inpatient admissions and emergency department visits reported for 17-42% and 25-42% of patients, respectively. Taken together, these data indicate the substantial disease burden associated with EGPA.

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Pituitary Involvement in Granulomatosis with Polyangiitis: A Retrospective Analysis in a Single Chinese Hospital and a Literature Review.

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