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Objective: Introduction: Musculoskeletal symptoms are not characteristic for granulomatosis with polyangiitis (GPA) patients. However, they could be present in two-thirds of patients at the onset of the disease and may provoke diagnostic difficulties. The aim: To assess retrospectively musculoskeletal manifestations in a group of GPA patients., Patients and Methods: Material and methods: The analyzed group consisted of 44 patients with GPA (27M, 17F) aged 46.7 years (16-75) and treated at the Department of Rheumatology and Connective Tissue Diseases in Lublin between 2010-2016. All patients were c-ANCA positive, and p-ANCA were present only in one patient (2.3%). The delay in GPA diagnosis averaged 15.9 months (0-166)., Results: Results: Joint manifestations were present in 43.2 % of patients (non-destructive arthritis in 22.7%, arthralgia in 20.5%), in 73.7% of cases anticipating GPA diagnosis by on average of 20.1 months (4-98). In 57.5% of patients joint manifestations were present from the beginning of the disease, most commonly as polyarthritis (68.4%). Myalgia was reported by 11.4% of patients. Among patients with arthritis 5 were RF-positive, none ACPA-positive, and 1 had ANA present., Conclusion: Conclusions: Musculoskeletal manifestations appear in a high proportion of GPA patients, in most cases preceding the diagnosis. Such a situation may cause diagnostic difficulties and the diagnosis delay.

Unlabelled: Wegener's granulomatosis there is a small--and middle--vessels vasculitis. The pathomorphological diagnostic criteria is known as Wegener's triad: 1) necrotizing granlomatous inflammation of upper and/or lower respiratory tract, 2) systemic or focal necrotizing vasculitis involving arteries and vein, and 3) focal segmental necrotizing crescentic gromerulonephritis. According to the current theory of pathogenesis of Wegener's granulomatosis, Staphylococcus aureus is involved. The rise in ANCA level during vascular inflammation is very important for monitoring disease. The main localization of Wagener's granulomatosis is in lung, nasal sinusites, nose, kidneys and nasal pharynx., Material and Methods: The history of 60-years old female patient who was being treated in Department of Otolaryngology for chronic bilateral otitis media for many years is presented. During hospitalization the sinusites, rhinitis and renal failure additionally were found. The CT sinuses and head, histopatological and ANCA exam in patient were performed., Results: Based on clinical, physical, additional exam Wegener's granulomatosis was diagnosed., Conclusions: 1) Surgical procedure is not indicated method of treatment of Wegener's granulomatosis, because it can increase the pathologic process. 2) The prognosis of Wegener's granulomatosis depends mainly on the ability to diagnose the early disease and the aplication of adequate treatment.

Systemic autoimmune diseases form a diverse group which includes: systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD), scleroderma, dermato-polymyositis, Wegener's granulomatosis, Sjögren syndrome. Although multisystem involvement is the hallmark of these diseases, the heart seems to be less affected than other organ systems. The aim of the study was to study possible cardiac abnormalities in patients with documented systemic autoimmune diseases and to assess whether there was any relation between antiphospholipid, anti-dsDNA antibodies and myocardial dysfunction findings. 76 patients (53 with SLE, 9 with MCTD, 8 with scleroderma, 6 with Wegener's granulomatosis) were subjected to our study, 69% of these patients manifested cardiac involvement, based on two-dimentional echocardiografic examination (36%--post-inflammatory valvular thickening, 20%--pericardial effusions, 15%--valvular regurgitation, 7%--left atrial enlargement, 5%--left ventricular hypertrophy, 4%--left ventricular dysfunction). None of the patients showed characteristic, acute Libman-Sacks endocarditis, which probably can be explained by chronic corticosteroid-treatment. Clinical evidence of cardiac abnormalities has been observed, in as many as 58% of cases with positive echocardiographic findings. The frequency and extend of cardiac pathology positively correlated with the detection of antiphospholipid antibodies. No such relationship was observed in patients with the presence of very high titers of antinuclear antibodies (anti-dsDNA). In conclusion, our results indicate that echocardiography is a useful method for assessment and monitoring cardiac involvement in the systemic autoimmune diseases.

Antineutrophil cytoplasmic antibodies (ANCA) have specificity for constituents of neutrophil granules. There are two different types of ANCA identifiable by indirect immunofluorescence method. One type produces the cytoplasmic staining pattern (C-ANCA) and the second-perinuclear (P-ANCA). The aim of the study was to evaluate the frequency of ANCA in patients with connective tissue diseases (CTD). Serum samples were obtained from 394 patients suffering from CTD. The patients group consisted of 86 patients with lupus erythematosus systemic (LES) (including 30 with LES accompanied with glomerulonephritis), 136 cases with rheumatoid arthritis (RA) (including 18 patients with RA and vasculitis), 42 patients with systemic sclerosis (SSc), 76 cases of Sjögren's syndrome (SS), 30 with Wegener's granulomatosis (WG), and 24 patients with polyarteritis nodosa (PAN). All patients fulfilled ARA criteria for the classification of CTD. The control group consisted of 42 healthy individuals. ANCA were detected by immunofluorescence method according to Wiik, and by an antigen-specific--enzyme-linked immunosorbent assay (ELISA). Proteinase 3 (PR-3), myeloperoxidase (MPO), elastase (ELA), lactoferrin (LC) and lysozyme (LZ), as well as cathepsin G were used as antigens in ELISA method. ANCA were detected in sera of 86 (21.8%) patients with CTD. C-ANCA pattern was observed in 28 (7.1%) cases, and p-ANCA in 58 (14.7%). C-ANCA were detected in sera of 28 (93%) patients with WG. P-ANCA were showed in 12 (13.9%) patients with LES, in 12 (50%) cases with PAN, in 20 (14.7%) with RA, in sera of 4 (9.5%) patients with SSc and in 10 (13.1%) with SS. No ANCAs were detected in healthy individuals. Ani-PR-3 antibodies were showed in sera of 26 patients, anti MPO in 30 cases, anti-ELA in sera of 12 patients, and anti-LC in 14 cases, but anti-LZ in 4 patients with CTD. The presence of ANCA in CTD patients may indicate the vascular inflammatory process during the course of the disease. It is a very important factor for the clinical course, and prognosis in the CTD patients.

A two cases Wegener's syndrome was described. The current knowledge concerning the role of neutrophils in pathogenesis of Wegener's syndrome is reviewed.

A case of Wegener's granulomatosis (WG) with involvement of the upper and lower respiratory tract and kidneys is presented. Despite specific symptoms the diagnosis was changed several times. Final diagnosis was made with immunofluorescence test for antineutrophil-cytoplasm antibodies (IF-ANCA). In Poland, this is the first report emphasizing the role of ANCA in diagnosis of WG. The significance of this test in diagnosing of WG is presented.