Intestinal vasculitis as a manifestation of different systemic autoimmune diseases treated at the Split University Hospital Centre during a 10-year period

Daniela Marasović Krstulović, Petra Šimac, Dijana Perković, Dušanka Martinović Kaliterna

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.