| Abstract | Imunoglobulin A vaskulitis (IgAV) je sistemski vaskulitis kojeg obilježava taloženje imunoglobulina A (IgA) imunokompleksa u stijenkama malih krvnih žila. Najčešće obolijevaju djeca, dok je u odraslih incidencija znatno niža. Kliničkom slikom dominira palpabilna, netrombocitopenična purpura uz artralgije ili artritis, abdominalnu bol i bubrežnu bolest. Cilj ovog rada bio je istražiti kliničke karakteristike, tijek bolesti i liječenje bolesnika s IgAV u odrasloj dobi. U retrospektivno, kohortno istraživanje uključeno je 18 bolesnika s dijagnozom IgAV prema EULAR/PRINTO/PRES kriterijima koji su liječeni u Kliničkoj bolnici Dubrava na Zavodu za kliničku imunologiju, alergologiju i reumatologiju. U istraživanju je bilo 7 muškaraca i 11 žena, prosječne dobi 56 ± 20 godina, najveće pojavnosti IgAV od 45 do 75 godina. Purpura je bila lokalizirana na donjim ekstremitetima u svih bolesnika, u 7 bolesnika na gornjim ekstremitetima (39%), u 4 bolesnika po trupu (22%), bule je imalo 3 (17%), artralgije 12 (67%), bubrežnu bolest 14 (78%), a bol u trbuhu 6 bolesnika (33%). Recidiva je bilo u 2 bolesnika (11%). Glukokortikoidima (GK) su se liječili svi bolesnici (100%), nesterodinim antireumaticima (NSAR) 10 (56%), topičkim GK 6 (33%), ciklofosfamidom 3 (17%), azatioprinom 1 (6%), dapsonom 1 (6%), inhibitorima angiotenzin-konvertirajućeg enzima (ACEi) 13 (67%), blokatorima angiotenzin II receptora (ARB) 3 bolesnika (17%). Arterijsku hipertenziju je imalo 12 bolesnika (66%), a šećernu bolest 6 (33%). U 11 bolesnika učinjena je biopsija bubrega (61%). Pogoršanja bubrežne funkcije i smrtnih ishoda nije bilo. Rezultati ovog istraživanja su u skladu su s rezultatima dosadašnjih istraživanja prema kojima je IgAV u odraslih rijetka bolest, teže kliničke slike, s najvećom pojavnošću u dobi od 45 do 75 godina, sa češćim zahvaćanjem bubrega, a rjeđim zahvaćanjem zglobova i trbuha u usporedbi s djecom. Bolest je prema dosadašnjim rezultatima češća u muškaraca, dok je u našem istraživanju učestalija u žena. GK su temelj u liječenju IgAV uz rjeđu primjenu imunosupresiva. Često se u terapiji koriste ACEi i ARB zbog IgA nefritisa i arterijske hipertenzije kao najčešćeg komorbiditeta. U istraživanje nismo uključili odrasle oboljele od IgAV s blažom kliničkom slikom koji su provodili liječenje u kućnim uvjetima, što je uz mali broj bolesnika glavni nedostatak ovog istraživanja. U skrbi odraslih oboljelih od IgAV nedostaju podaci kliničkih istraživanja o karakteristikama bolesti kao i preporuke i jednoglasni stavovi krovnih društava vezano za obradu i liječenje. |
| Abstract (english) | Immunoglobulin A vasculitis (IgAV) is a systemic vasculitis characterised by immunoglobulin A (IgA) immunocomplex deposits found in the walls of small blood vessels. The incidence of IgAV is significantly higher in children compared to adults. Patients mainly present with palpable non-thrombocytopenic purpura that can be followed by arthralgia or arthritis, abdominal pain and kidney disease. The aim of this paper was to investigate the clinical features, disease progression and treatment of adult IgAV patients. This retrospective cohort study included a total number of 18 patients diagnosed according to the EULAR/PRINTO/PRES criteria and treated in Clinical Hospital Dubrava at the Division of Clinical Immunology, Allergology and Rheumatology. The study included 7 men and 11 women. The age average was 56 ± 20 years. The highest incidence was in the group between 45 and 75 years. All patients presented with purpura of the lower extremities, whereas 7 had purpura of the upper extremities (39%) and 4 had purpura of the corpus (22%). Bullae were reported in 3 patients (17%), arthralgia in 12 patients (67%), kidney disease in 14 patients (78%) and abdominal pain in 6 patients (33%). A relapse occured in 2 patients (11%). All patients (100%) were treated with glucocorticoids, 10 patients with nonsteroidal anti-inflammatory drugs (56%), 6 patients with topical glucocorticoids (33%), 3 patients with cyclophosphamide (17%), 1 patient with azathioprine (6%) and 1 with dapsone (6%), 13 patients (67%) with angiotensin-converting enzyme inhibitors (ACEi) and 3 patients (17%) with angiotensin II receptor blockers (ARB). Arterial hypertension was reported as a comorbidity in 12 patients (66%) and diabetes in 6 patients (33%). Kidney biopsy was performed in 11 patients (61%). There were no deaths or kidney disease exacerbations. The results of this paper are in line with other recently conducted studies, according to which IgAV in the adult population is a rare disease with a relatively worse clinical presentation than in children, with the greatest risk at the age between 45 and 75y. Furthermore, kidney disease is more common, whereas abdominal or joint involvement is less common in adults than in children. According to other literature available, IgAV is more common in men, however, in this research it is more common in women. Glucocorticoids are the basis of IgAV patient treatment with a comparatively less frequent use of immunosuppressive drugs. The study did not include adult IgAV patients with milder clinical presentations treated in home conditions, which is, alongside a small number of patients, the main limitation of this research. There is a lack of clinical research regarding the clinical characteristics of adult IgAV, as well as recommendations of an optimal diagnostic and treatment plan. |
