Clinical case of eosinophilic granulomatous polyangiitis debuted by myocardial infarction

The article presents clinical observations demonstrating the difficulties of diagnostic search in establishing the diagnosis of systemic vasculitis associated with antineutrophil cytoplasmic antibodies, which include a rare disease eosinophilic granulomatosis with polyangiitis (ChurgStrauss syndrome). Carefully collected anamnesis, participation of specialists of different profiles, retrospective analysis of laboratory and instrumental data allowed to verify the diagnosis, to prescribe adequate therapy. The aim of the publication is to discuss the need for early diagnosis of eosinophilic granulomatosis with polyangiitis, which can improve the effectiveness of therapy and improve the overall prognosis for this disease, taking into account modern approaches based on the main provisions of international recommendations that were prepared in 2015 with the participation of leading experts from Europe, USA and Canada and were called to become the basis for choosing a personalized patient therapy strategy. In some cases, the diagnosis of eosinophilic granulomatous polyangiitis is complicated by the diversity of the clinical picture, the non-simultaneous appearance of the main symptoms and the violation of the stages of the process. In the differential diagnosis of systemic vasculitis, assessment of initial clinical manifestations, testing for the presence of antibodies to the cytoplasm of neutrophils, multispiral computed tomography of the chest organs and diagnostic biopsy of the affected tissues are crucial. In the histological conclusion, a picture of focal ulcerative-necrotic lesions of the nasal mucosa with signs of vasculitis and a pronounced eosinophilic cell component of inflammation was noted. The distinctive features of this case include the onset of the disease with the development of myocardial infarction and early damage to the hearing organ in the form of cochlear neuritis, complicated by sensorineural hypoacusis. The combination of high doses of glucocorticosteroids and cyclophosphamide is still the gold standard for the treatment of severe cases, but the use of biological agents such as rituximab or mepolizumab seems to be a promising therapeutic alternative (4 figs, bibliography: 3 refs).

A Case of Granulomatosis With Polyangiitis With Pulmonary Nodules and Masses

Background: Granulomatosis with polyangiitis (GPA) is autoimmune-mediated, necrotizing granulomatous vasculitis, primarily involving the upper and lower respiratory tract and kidneys. Pulmonary parenchymal necrosis, vasculitis, and granulomatous inflammation are the main pathological features. Pulmonary imaging visualizes various manifestations, like lung consolidation, ground-glass shadow, speckle shadow, nodules and mass shadows, cavitation, pulmonary interstitial change, pleural effusion, and multiple mediastinal lymph nodes enlargements, and stenosis or occlusion of a lobe or bronchus segment. Case presentation: We report a 53-year-old patient diagnosed with Granulomatous polyangiitis with multiple pulmonary nodules and masses, by ultrasound-guided lung mass puncture biopsy. After treatment with methylprednisolone, Mycophenolate Mofetil, compound sulfamethoxazole and cyclophosphamide, the patient's condition improved and the pulmonary lesions were better than before.Conclusions: In addition to specific infections and tumors, immunological diseases, especially granulomatous polyangiitis, should be considered when treating patients in a clinical setting with multiple pulmonary nodules and weights. Through close multidisciplinary cooperation and communication, the disease will be hopefully received timely diagnosis and early treatment, relieving pain for more patients.

AB0540 CLINICAL ANALYSIS OF AORTA INVOLVEMENT IN PATIENTS OF ANCA-ASSOCIATED VASCULITIS

Background:ANCA-associated vasculitis (AAV) is an autoimmune disease that involves abnormal death of neutrophils and leads to necrotic inflammatory reactions in blood vessels, including microscopic polyangiitis (MPA), Granulomatous polyangiitis (GPA) and Eosinophilic granulomatous polyangiitis (EGPA). AAV is mainly involved in small blood vessels, and intermediate arterial lesions can also occur, but the large arteries and their primary branches are rarely involved.Objectives:To summarize the clinical characteristics of aortic involvement in patients with ANCA-associated vasculitis (AAV).Methods:The clinical manifestations, systemic involvement, laboratory examination, imaging characteristics and treatment of aortic involvement in AAV patients admitted to Peking Union Medical College Hospital from January 2013 to December 2018 were retrospectively analyzed.Results:Nine patients were enrolled in our study. The ratio of male to female was 2:1 and the median age was 47 years old. Of the 9 patients, 4 were GPA (44%), 4 were MPA (44%) and 1 was EGPA (11%). The aorta is involved in an average of 3 locations per case, mainly in 7 locations: 3 ascending aorta and aortic arch, 4 in the head and arm trunk (including carotid and subclavian artery), 2 in the abdominal aorta, and 1 in the abdominal cavity. There were 2 cases of renal artery, 1 case involving brachial radial artery, 2 cases of iliac artery and lower limb artery, and 1 case involving left main coronary artery, anterior descending branch, circumflex branch, and right coronary artery. Aortic lesions: 3 cases had aneurysms, arterial dilatation and / or dissection, 6 cases had arterial stenosis or occlusion and 3 cases had periarteritis.When major arterial involvement was found, the AAV of the patients were mostly active, with an average of 19 points for BVAS vasculitis activity and 1 for FFS score. 6 cases had lung involvement (67%), 6 cases had kidney involvement (67%), 4 cases had ENT involvement (44%), 3 cases had nervous system involvement (33%), and 1 case had gastrointestinal involvement (11%). All patients were treated by steroid and immunosuppressant, while 1 case received the operation of ascending aorta and aortic arch replacement.Conclusion:Mainly involved in small blood vessel inflammation, AAV may also have aorta involvement, which was more common in patients who had active disease and need more positive treatment. The affected aorta areas of these patients were mainly ascending aorta, aortic arch, and head and arm trunk, which can be manifested as aneurysms, dissections, and arterial stenosis Periarteritis, etc. If necessary, surgically treatment of the affected aorta could be considered when the situation of AAV was stable enough.References:[1]Jennette J C, Falk R J, Bacon P A, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides[J]. Arthritis Rheum, 2013,65(1):1-11.DOI:10.1002/art.37715.[2]Eisenberger U, Fakhouri F, Vanhille P, et al. ANCA-negative pauci-immune renal vasculitis: histology and outcome[J]. Nephrol Dial Transplant, 2005,20(7):1392-1399.DOI:10.1093/ndt/gfh830.[3]Guillevin L, Pagnoux C, Seror R, et al. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort[J]. Medicine (Baltimore), 2011,90(1):19-27.DOI:10.1097/MD.0b013e318205a4c6.[4]Chirinos J A, Tamariz L J, Lopes G, et al. Large vessel involvement in ANCA-associated vasculitides: report of a case and review of the literature[J]. Clin Rheumatol, 2004,23(2):152-159.DOI:10.1007/s10067-003-0816-0.[5]Minnee R C, van den Berk G E, Groeneveld J O, et al. Aortic aneurysm and orchitis due to Wegener’s granulomatosis[J]. Ann Vasc Surg, 2009,23(6):715-786.DOI:10.1016/j.avsg.2009.06.011.Disclosure of Interests:None declared