Prikaz bolesnice s c-ANCA vaskulitisom bez zahvaćenosti dišnih putova

Željka Kardum, Jasminka Milas Ahić1, Ivana Kovačević, Ana Marija Lukinac, Ana Kovač, Kristina Kovačević Stranski, Višnja Prus,

Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is a small-vessel vasculitis, characterized by necrotizing inflammation of small vessels and positive ANCAs. cANCAs are directed against proteinase-3 (PR-3) and are specific for granulomatosis with polyangiitis (GPA, formerly known as Wegener’s disease), although their role in the immunopathology of the disease is still unclear. This is why the European Medicines Agency suggested that cANCA positivity, in addition to the clinical picture, can be enough for the diagnosis of GPA. Granulomatosis with
polyangiitis is characterized by granulomatous inflammation that usually involves the upper and lower respiratory tract, necrotizing vasculitis that affects small and medium-sized vessels, and often rapidly progressive glomerulonephritis. We present a patient with an unusual presentation of cANCA-associated vasculitis, who presented with arthritis, palpable purpura on legs, and pauci-immune necrotizing glomerulonephritis with highly positive cANCA antibodies, without any signs or symptoms of airway involvement. Although renal-limited ANCA vasculitis is recognized as a
separate entity, our patient also had signs of skin and joint involvement and prominent constitutional symptoms that contributed to the diagnosis of systemic disease. In this paper, we present a patient with an atypical course of GPA, which can also be called a limited form of the disease. An atypical course or limited form belongs to the group of sine syndromes in inflammatory rheumatic diseases, which are not very common, but should be considered in the differential diagnosis. To our knowledge, there are only a few described case reports with a similar presentation.

Vaskulitis povezan s antineutrofilnim citoplazmatskim protutijelima (engl. Anti-neutrophil Cytoplasmatic Antibody – ANCA) ili skraćeno ANCA vaskulitis (engl. ANCA-associated vasculitis – AAV) jest vaskulitis malih krvnih žila karakteriziran nekrotizirajućom upalom malih krvnih žila i pozitivnim antineutrofilnim citoplazmatskim ma. Protutijela c-ANCA usmjerena su na proteinazu 3 (PR-3) i karakteristična za granulomatozu s poliangiitisom (GPA), prije poznatu kao Wegenerova bolest, premda njihova uloga u imunopatologiji bolesti još nije razjašnjena. Upravo zbog visoke specifičnosti protutijela Europska agencija za lijekove predložila je da pozitivna protutijela c-ANCA uz popratnu kliničku sliku budu dovoljna za dijagnozu GPA. Granulomatozu s poliangiitisom karakteriziraju granulomatozna upala koja uobičajeno zahvaća gornje i donje dišne putove, nekrotizirajući vaskulitis što zahvaća male i srednje velike krvne žile i često prisutan brzoprogresivni glomerulonefritis. U ovom radu prikazana je bolesnica s neuobičajenom prezentacijom c-ANCA vaskulitisa uz prisutne artritis, palpabilnu purpuru na nogama, pauciimuni nekrotizirajući glomerulonefritis i visokopozitivna protutijela c-ANCA, međutim, bez znakova i simptoma zahvaćanja dišnih putova. Premda je ANCA vaskulitis ograničen na bubreg otprije poznat kao poseban entitet, naša je bolesnica također imala prisutno zahvaćanje kože i zglobova te izražene konstitucijske simptome, što upućuje na sustavni oblik bolesti. Prikazana je bolesnica s atipičnim oblikom GPA, koji se može nazvati i ograničenim oblikom bolesti. Atipični ili ograničeni oblik bolesti pripada skupini sindroma sine u upalnim reumatskim bolestima, koji nisu česti, ali ih je potrebno razmotriti pri diferencijalnoj dijagnozi. Prema našim saznanjima, samo je nekoliko opisanih prikaza bolesnika sa sličnom prezentacijom bolesti.