Review article

https://doi.org/10.33004/reumatizam-66-1-3

Case report of a patient with cANCA vasculitis without airway involvement

Željka Kardum ; Division of Rheumatology, Clinical Immunology, and Allergology, Department of Internal Medicine, UHC Osijek, Osijek, Croatia
Jasminka Milas Ahić ; Division of Rheumatology, Clinical Immunology, and Allergology, Department of Internal Medicine, UHC Osijek, Osijek, Croatia;Faculty of Medicine, Josip Juraj Strossmayer University of Osijek, Osijek, Croatia
Ivana Kovačević ; Division of Rheumatology, Clinical Immunology, and Allergology, Department of Internal Medicine, UHC Osijek, Osijek, Croatia
Ana Marija Lukinac ; Division of Rheumatology, Clinical Immunology, and Allergology, Department of Internal Medicine, UHC Osijek, Osijek, Croatia
Ana Kovač ; Division of Rheumatology, Clinical Immunology, and Allergology, Department of Internal Medicine, UHC Osijek, Osijek, Croatia
Kristina Kovačević Stranski ; Division of Rheumatology, Clinical Immunology, and Allergology, Department of Internal Medicine, UHC Osijek, Osijek, Croatia
Višnja Prus ; Division of Rheumatology, Clinical Immunology, and Allergology, Department of Internal Medicine, UHC Osijek, Osijek, Croatia; Faculty of Medicine, Josip Juraj Strossmayer University of Osijek, Osijek, Croatia

Abstract

Antineutrophil cytoplasmic autoantibody (ANCA )-associated vasculitis (AAV ) is a small-vessel vasculitis, characterized by necrotizing inflammation of small vessels and positive ANCA s. cANCA s 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.

Keywords

Anti-neutrophil cytoplasmic antibody-associated vasculitis – diagnosis, pathology; Antibodies, antineutrophil cytoplasmic – analysis; Granulomatosis with polyangiitis – diagnosis, drug therapy, pathology; Purpura – etiology; Glomerulonephritis – etiology; Arthritis – etiology

Hrčak ID:

226241

URI

https://hrcak.srce.hr/226241

Publication date:

16.10.2019.

Article data in other languages: croatian

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