Vaskulitisi crijeva kao očitovanja raznih sustavnih autoimunih bolesti liječeni u Kliničkom bolničkom centru Split u desetogodišnjem razdoblju

Autor: Marasović Krstulović, Daniela, Šimac, Petra, Perković, Dijana, Martinović Kaliterna, Dušanka
Jazyk: angličtina
Rok vydání: 2021
Předmět:
Zdroj: Reumatizam
Volume 68
Issue 1
ISSN: 2459-6159
0374-1338
Popis: Introduction. The most common types of vasculitis that involve the gastrointestinal tract (GIV) are immune complex- mediated in systemic lupus erythematosus (SLE ), Sjögren’s syndrome (SS), mixed connective tissue disease (MCTD ), and IgA vasculitis (IgAV). GI manifestations are rarely the leading symptom of systemic vasculitis. Only 1 – 5% of rheumatoid arthritis (RA ) patients develop symptoms of gastrointestinal tract vasculitis (GIV), while up to 40% of them have GI symptoms. GIV is a rare but life-threatening complication in patients with SLE with a prevalence of up to 2.5%. The leading symptoms in patients with GIV include abdominal pain, nausea, vomiting, diarrhoea, small bowel obstruction, and profuse GI bleeding. The objective of this study was to describe the incidence and clinical manifestations of GIV in patients with various systemic autoimmune (AI) diseases who were treated at Split University Hospital Centre over a 10-year period. Materials and methods. A retrospective study was conducted by analysing medical records of patients diagnosed with GIV and treated for SLE , SS, MCTD , vasculitis syndrome, IgAV, and RA between January 2009 and December 2018. Only patients with anamnestic data in relation to abdominal pain or endoscopic and/or radiographic findings of GIV were included in the study. Results. Out of a total number of 12 patients with a confirmed diagnosis of GIV, 9 were male. Eight of them had vasculitis with gastrointestinal involvement (GIV) in IgAV, 2 patients had GIV in SLE , 1 patient had microscopic polyangiitis (MPA ), and one patient had primary SS. In 6 cases, GIV was diagnosed by an MSCT of the abdomen, in one case it was diagnosed by a PET -CT scan, in another case it was diagnosed through histopathological findings, and in 4 cases it was diagnosed through endoscopic findings. The leading symptom in 4 patients was abdominal pain with nausea and vomiting, 2 had profuse GI bleeding, 1 had fatigue without GI symptoms, and the remaining patients’ clinical features included acute abdomen with visible radiographic thickening of the bowel wall with oedema and stratification with ascites. GIV was the cause of death of one patient with SLE . Others had a good or moderate response to treatment with glucocorticoids and immunosuppressants. Conclusion. In conclusion, GIV is a rare manifestation of systemic AI diseases, but the clinical features can be very severe and lead to a fatal outcome, especially if it is not diagnosed at an early stage and treated with aggressive immunosuppressive therapy.
Uvod. Najčešći vaskulitisi gastrointestinalnog trakta (GIV) su oni posredovani imuno-kompleksima u sistemskom eritemskom lupusu, Sjögrenovoj bolesti, miješanoj bolesti vezivnog tkiva, IgA-vaskulitisu (IgAV). Gastrointestinalne (GI) manifestacije rijetko su vodeći simptom sustavnih vaskulitisa. Samo 1–5% bolesnika s reumatoidnim artritisom razvija kliničku sliku vaskulitisa gastrointestinalnoga trakta (GIV), dok ih do 40% ima GI simptome. GIV je rijetka, ali životno ugrožavajuća komplikacija u bolesnika sa sistemskim eritemskim lupusom (SLE ), s prevalencijom do 2,5%. Vodeći simptomi u bolesnika s GIV-om su bol u trbuhu, mučnina, povraćanje, proljev, opstrukcija tankog crijeva i obilno GI krvarenje. Cilj ovog rada bio je ispitati učestalost i klinička očitovanja GIV-a u bolesnika s različitim sustavnim autoimunim (AI) bolestima liječenih u KBC -u Split u desetogodišnjem razdoblju. Materijali i metode. R etrospektivno su analizirani podatci iz medicinske dokumentacije bolesnika koji su se liječili od SLE -a, Sjögrenovog sindroma (SjS), miješane bolesti vezivnog tkiva (MCTD ), sindroma vaskulitisa, IgA-vaskulitisa (IgAV) i i RA , a imali su anamnestičke podatke o boli u trbuhu ili endoskopske ili/i radiografske znakove GIV-a, u razdoblju od 1/2009. do 12/2018. Rezultati. Od ukupno 12 bolesnika s potvrđenom dijagnozom GIV-a, 9 su bili muškarci. Osam ih je imalo GIV u sklopu IgAV-a, dvije bolesnice u sklopu SLE -a, MPA jedna bolesnica, primarnog SS-a jedan bolesnik. U 6 slučajeva GIV je dokazan MSCT-om trbuha, u jednom PET -CT-om, u jednom patohistološki, a u 4 slučaja endoskopski. Vodeći simptom u četvoro bolesnika bila je bol u trbuhu s mučninom i povraćanjem, dva su imala su obilno GI krvarenje, jedna bolesnica je imala umor bez GI simptoma, a preostali kliničku sliku akutnog abdomena s radiološki verificiranim edemom i raslojavanjem stijenke crijeva uz ascites. GIV je bio uzrok smrti jedne bolesnice sa SLE -om. Ostali su imali dobar ili umjeren odgovor na liječenje glukokortikoidima i imunosupresivima. Zaključak. Zaključno, GIV je rijetka manifestacija sustavnih AI bolesti, ali klinička slika može biti vrlo teška i dovesti do fatalnog ishoda te je nužna brza dijagnoza i agresivno imunosupresivno liječenje.
Databáze: OpenAIRE
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