末梢神経障害の残存する好酸球性多発血管炎性肉芽腫症に対して免疫グロブリン大量療法が奏功した一例

京都府立医科大学附属北部医療センター　総合診療科Department of General internal medicine , North Medical Center , Kyoto Prefectural University of Medicine好酸球性多発血管炎性肉芽腫症(EGPA)は1951年にChurgとStraussによって報告された疾患で、以前はChurg-Strauss症候群やアレルギー性肉芽腫性血管炎と呼ばれていた。EGPAは、好酸球の組織浸潤、小型から中型血管の壊死性血管炎によって特徴付けられる稀な全身性疾患である。EGPAの臨床症状としては、気管支喘息、アレルギー性鼻炎、好酸球の組織浸潤(心臓、消化管、皮膚、腎臓)、末梢神経障害などの血管炎症状である。EGPAの予後を改善させる上で、早期診断と早期治療は極めて重要であり、末梢神経障害による運動麻痺が出現すればQOLは著しく低下する可能性がある。しかし、その一方でEGPAの診断には難渋することが多く、その理由としては慢性好酸球性肺炎、好酸球増多症候群、他の一次性全身性血管炎などと臨床的特徴が類似または重複するためと考えられる。今回、我々は顔面浮腫と関節痛を初発症状とし、末梢血好酸球増多の鑑別をしている際に末梢神経障害を発症し、EGPAの診断に至った症例を経験した。Prednisolone(PSL)による寛解導入後も末梢神経障害は残存していたため、早期に免疫グロブリン大量療法(IVIg)を施行したところ、末梢神経障害の著明な改善を得た。EGPAの早期診断と末梢神経障害に対するIVIgの有効性に関して、文献を踏まえた考察を行い、報告する。(著者抄録

First report of FIP1L1-PDGFR alpha-positive eosinophilic granulomatosis with polyangiitis

First report of FIP1L1-PDGFR\u3b1-positive eosinophilic granulomatosis with polyangiiti

Aspergillosis myocarditis in the immunocompromised host

Invasive cardiac aspergillosis has been rarely described in immunocompromised patients. This disease is difficult to diagnose by conventional laboratory, microbiologic, and imaging techniques, and is often recognized only post-mortem. The authors present the case of a 60-year-old woman admitted with an exacerbation of eosinophilic granulomatosis with polyangitiis (EGPA) who subsequently died from Aspergillus myocarditis, and compare the patient’s case to prior literature. This serves as an up-to-date literature review on the topic of invasive cardiac aspergillosis. Keywords: aspergillus myocarditis, Angioinasive aspergillosis, Eosinophilic granulomatosis with polyangiiti

Successful reconstruction of an ocular defect resulting from granulomatosis with polyangiitis, following treatment with rituximab

Purpose: To report a unique case of orbital inflammatory disease which was ultimately diagnosed as granulomatosis with polyangitis (GPA) and thus successfully treated. Observation: A 47 year-old man presented with a rapidly progressive necrotic soft tissue mass within the medial antero-superior aspect of the right eyelid and orbit. He also had transient retinal vasculitis in the left. Serology, histology and imaging were atypical of, but consistent with, GPA. He was thus successfully treated with intravenous rituximab followed by reconstruction of the medial eyelid. Conclusion and importance: A high index of suspicion of GPA is required in orbital inflammatory disease, especially when typical diagnostic findings are absent. Keywords: Orbital inflammatory disease, Rituximab, Eyelid reconstruction, Granulomatosis with polyangiiti

Health-related quality of life in ANCA-associated vasculitis and item generation for a disease-specific patient-reported outcome measure

Joanna C Robson,1,2 Jill Dawson,3 Peter F Cronholm,4 Nataliya Milman,5 Katherine S Kellom,6 Susan Ashdown,7 Ebony Easley,8 John T Farrar,9 Don Gebhart,10,11 Georgia Lanier,10,11 Carol A McAlear,12 Jacqueline Peck,7 Raashid A Luqmani,7 Judy A Shea,13 Gunnar Tomasson,14 Peter A Merkel10,11 1Department of Rheumatology, Faculty of Health and Applied Sciences, University of the West of England, Bristol, UK; 2School of Clinical Sciences, University of Bristol, Bristol, UK; 3Department of Population Health (HSRU), University of Oxford, Oxford, UK; 4Department of Family Medicine and Community Health, University of Pennsylvania, Philadelphia, PA, USA; 5Division of Rheumatology, Department of Medicine, University of Ottawa, Ottawa Hospital Research Institute, Ottawa, ON, Canada; 6PolicyLab, Children’s Hospital of Philadelphia, Philadelphia, PA, USA; 7Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, UK; 8Department of Family Medicine and Community Health, Mixed Methods Research Laboratory, University of Pennsylvania, Philadelphia, PA, USA; 9Biostatistics and Epidemiology, Hospital of the University of Pennsylvania, Philadelphia, PA, USA; 10Division of Rheumatology, Department of Medicine, University of Pennsylvania, Philadelphia, PA, USA; 11Department of Biostatistics and Clinical Epidemiology, University of Pennsylvania, Philadelphia, PA, USA; 12Vasculitis Research, University of Pennsylvania, Philadelphia, PA, USA; 13School of Medicine, University of Pennsylvania, Philadelphia, PA, USA; 14Department of Public Health Sciences, University of Iceland, Reykjavik, Iceland Objective: The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are multisystem diseases of the small blood vessels. Patients experience irreversible damage and psychological effects from AAV and its treatment. An international collaboration was created to investigate the impact of AAV on health-related quality of life (HRQoL), and develop a disease-specific patient-reported outcome measure to assess outcomes of importance to patients.Methods: Patients with AAV from the UK, USA, and Canada were interviewed to identify salient aspects of HRQoL affected by AAV. The study was overseen by a steering committee including four patient research partners. Purposive sampling of interviewees ensured representation of a range of disease manifestations and demographics. Inductive analysis was used to identify themes of importance to patients; these were further confirmed by a free-listing exercise in the US. Individual themes were recast into candidate items, which were scrutinized by patients, piloted through cognitive interviews and received a linguistic and translatability evaluation.Results: Fifty interviews, conducted to saturation, with patients from the UK, USA, and Canada, identified 55 individual themes of interest within seven broad domains: general health perceptions, impact on function, psychological perceptions, social perceptions, social contact, social role, and symptoms. Individual themes were constructed into >100 candidate questionnaire items, which were then reduced and refined to 35 candidate items.Conclusion: This is the largest international qualitative analysis of HRQoL in AAV to date, and the results have underpinned the development of 35 candidate items for a disease-specific, patient-reported outcome questionnaire. Keywords: ANCA-associated vasculitis, quality of life, patient-reported outcomes, granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiiti