Gigantocelularni aortitis dijagnosticiran PET/CT-om – paraneoplastički sindrom? [Giant cell aortitis diagnosed with PET/CT - paraneoplastic syndrome?]

Bakula, Marija and Cerovec, Mislav and Mayer, Miroslav and Huić, Dražen and Anić, Branimir (2016) Gigantocelularni aortitis dijagnosticiran PET/CT-om – paraneoplastički sindrom? [Giant cell aortitis diagnosed with PET/CT - paraneoplastic syndrome?]. Liječnički vjesnik, 128 (5-6). pp. 152-158. ISSN 0024-3477

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Abstract

Vasculitides are heterogenic group of autoimmune connective tissue diseases which often present difficulties in early diagnosing. Giant cell arteritis is vasculitis of large and medium arteries. It predominantly presents with symptoms of affection of the external carotid artery branches. Furthermore, the only symptoms can be constitutional. In clinical practice, vasculitides are sometimes considered as paraneoplastic, but no definite association with malignancies has been established and the mechanisms are still debated. The gold standard for diagnosing giant cell arteritis is a positive temporal artery biopsy, but the results can often be false negative. Additionally, more than half of the patients have aorta and its main branches affected. Considering aforementioned, imaging studies are essential in confirming large-vessel vasculitis, amongst which is highly sensitive PET/CT. We present the case of a 70-year-old female patient with constitutional symptoms and elevated sedimentation rate. After extensive diagnostic tests, she was admitted to our Rheumatology unit. Aortitis of the abdominal aorta has been confirmed by PET/CT and after the introduction of glucocorticoids the disease soon went into clinical and laboratory remission. Shortly after aortitis has been diagnosed, lung carcinoma was revealed of which the patient died. At the time of the comprehensive diagnostics, there was no reasonable doubt for underlying malignoma. To the best of our knowledge, there are no recent publications concerning giant cell arteritis and neoplastic processes in the context of up-to-date non-invasive diagnostic methods (i.e. PET/CT). In the light of previous research results, we underline that the sensitivity of PET/CT is not satisfactory when estimating cancer dissemination in non-enlarged lymph nodes and that its value can at times be overestimated.

Abstract in Croatian

Vaskulitisi se ubrajaju u skupinu sistemskih autoimunosnih bolesti vezivnog tkiva i često se teško pravodobno dijagnosticiraju. Gigantocelularni arteritis zahvaća velike i srednje velike krvne žile. Najčešće se očituje simptomima vezanim uz zahvaćanje temporalne arterije, a može se prezentirati i općim simptomima. U kliničkoj praksi vaskulitisi se u ­nekim slučajevima smatraju paraneoplastičkim sindromom, no dokaz povezanosti ovih entiteta ostaje tema rasprave. Zlatni standard u postavljanju dijagnoze gigantocelularnog arteritisa jest biopsija temporalne arterije. U praksi je pak rezultat pretrage često lažno negativan, a u više od polovice bolesnika zahvaćeni su aorta i njezini glavni ogranci. Važnu ulogu u dijagnosticiranju vaskulitisa velikih krvnih žila imaju neinvazivne slikovne metode, među kojima se PET/CT pokazao ­visoko osjetljivim. Prikazan je slučaj 70-godišnje pacijentice koja je zbog općih simptoma i upalnih parametara, nakon u nekoliko navrata učinjene opsežne obrade, ponovo hospitalizirana. PET/CT-om verificiran je abdominalni aortitis te je uz terapiju glukokortikoidima brzo došlo do kliničke i laboratorijske remisije bolesti. Kratko nakon postavljene dijagnoze aortitisa verificiran je malignom zbog kojeg je bolesnica preminula, a za koji u vrijeme ekstenzivne dijagnostičke obrade nije bilo dokaza. Prema našim saznanjima, nema novijih radova koji su u kontekstu mogućnosti suvremene neinvazivne dijagnostike (tj. PET/CT-a) promatrali povezanost gigantocelularnog arteritisa i neoplazma. Nadalje, skrećemo pozornost na činjenicu da osjetljivost PET/CT-a ne zadovoljava ako je riječ o diseminaciji malignoma u nepovećane limfne čvorove te da se vrijednost ove pretrage u kliničkoj praksi nerijetko precjenjuje.

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