In this study, we aimed to evaluate the factors affecting the development of damage and mortality in patients with AAV treated at our tertiary referral center. This retrospective study included data on patients with AAV who fulfilled the Chapel Hill Consensus Conference (CHCC) criteria. Patients were divided into c-ANCA/PR3( +) and p-ANCA/MPO ( +) groups based on ANCA immunofluorescence and/or ELISA results, and relapse, damage, and mortality data were compared across the groups. Data from 254 patients (n = 136, 53.5% female) were included in the analysis. Clinical diagnosis was GPA in 186 (73.2%) and MPA in 68 (26.8%) patients. During the follow-up, 217 of 242 (89.7%) patients developed damage, and the median VDI score of the cohort was 2 (IQR: 2). VDI scores were higher in the first period (1997–2011) than in the second period (2011–2021) in the entire cohort (p = 0.012) and in patients with GPA compared with MPA (p = 0.034). Five-year and overall survival rates were 88.1% and 80.3% in the entire cohort; 87.8% and 81% in c-ANCA/PR3 ( +); 86.2% and 76% in p-ANCA/MPO ( +) (Log-Rank: p = 0.35); 91% and 84.5% in GPA; 81% and 67.2% in MPA patients (Log-Rank: p < 0.001). Development of malignancy, severe infection, and active/persistent disease after the induction phase were associated with higher mortality in patients with AAV. In our AAV cohort, permanent organ damage was detected in the majority of the patients. Although the median VDI score decreased over time, mortality did not change.

Objective: The skin pathergy test (SPT) is an important tool in the diagnosis of Behçet disease (BD), but its decreasing sensitivity over years has resulted in less frequent use in the clinical practice. This study aimed to improve the sensitivity of the SPT without compromising its specificity. Methods: BD patients, patients with other inflammatory diseases, recurrent aphthous stomatitis, and healthy controls comprised the study group. The SPT was conducted using 20G needle and 21G lancet pricks, or with additional application of 23-valent polysaccharide pneumococcal vaccine (PS-23), Alum, or ATP to the prick site. Development of erythema and induration at 24 h/48 h were evaluated by the same observer. Induration (≥2 mm) with erythema at 48 h was accepted as a positive reaction. Proinflammatory cytokine production following stimulation with lipopolysaccharide or PS-23 was investigated by whole-blood assay (WBA) in a subgroup. Results: Stimulation of the forearm skin by PS-23 and a 20G needle prick showed the highest sensitivity and specificity in BD (64.3% and 100%, respectively), especially in patients with active disease (80.3% and 100%, respectively), compared with a sensitivity of 4.8% in all and 6.1% in active patients using a single 20G prick. A positive result was associated with active disease and no use of immunosuppressives. In WBA, increased IL-1β and IL-1Ra production in response to PS-23 was observed in the group with active BD, while the cytokine response to lipopolysaccharide was similar in all groups. Conclusions: The SPT conducted using a 20G needle prick and PS-23 antigens was shown to be a promising tool for the diagnosis of BD owing to its improved sensitivity compared with the standard approach.

Systemic lupus erythematosus (SLE) is an autoimmune disease with a variety of organ/system involvement. Respiratory system involvement is common in these patients and usually manifests itself by disorders of the lung parenchyma, pleura, pulmonary vasculature or diaphragm. In this study, we sought to determine the frequency of interstitial lung disease (ILD) in patients with SLE and associated risk factors. Three hundred randomly chosen patients with SLE were included. Chest x-ray (CXR), lung spirometry and carbon monoxide diffusion test (DLCO) were performed. High-resolution thorax computed tomography (HRCT) was performed for a definite diagnosis of ILD. . Of 300 patients, 16% had ILD. At the start of the study, the prevalence obtained from the patients' records showed that 4% had ILD. The median age, mean duration of disease, and follow-up time were significantly higher and longer in patients with ILD compared to patients without (p < 0.05). Forced expiratory volume (FEV1), forced vital capacity (FVC), DLCO and total lung capacity (TLC) were significantly lower in patients with ILD (p < 0.001). Patients with ILD had a significantly higher frequency of arthritis, serositis, Raynaud's phenomenon, myositis, and anti-Scl70 positivity (p = 0.01, 0.001, 0.02, 0.004, and 0.001, respectively). A significantly higher number of patients had stopped using hydroxychloroquine (HCQ) in the ILD group (p = 0.04).

Background/aim: Rheumatoid pulmonary nodule can be detected in up to 32% of rheumatoid arthritis (RA) patients and approximately one-third of nodules may cavitate. We aimed to evaluate characteristics of patients with RA developing cavitary pulmonary nodular (CPN) lesions under disease-modifying antirheumatic drugs (DMARDs), follow-up of both cavitary and solid nodules, and their outcome with the treatment. Materials and methods: RA patients who presented with CPN lesions during follow-up were recruited retrospectively in this case series analysis. Total numbers and mean diameters of cavitary and solid nodules in each thorax computed tomography (CT) have been determined and followed up by two experienced pulmonary physicians. Moreover, changes in treatment after the development of the CPN lesions and characteristics of cavitary nodules were collected. Results: Eleven patients with CPN lesions were reported. At the time of CPN diagnosis, more patients were taking leflunomide than methotrexate (81% vs 19%). Half of the patients were receiving biologic therapy and only 18% were taking anti-TNF drugs. After a median of 24 (3?65) months of follow-up, the regression of CPN lesions was determined in 45% (5/11) of patients. Four of these 5 (80%) patients were switched to a treatment regimen without leflunomide and three of them to nonanti-TNF biologic treatment or targeted synthetic DMARDs (tocilizumab, tofacitinib, and rituximab). Conclusion: CPN lesions seen in RA patients are often pulmonary manifestations of the underlying disease; however, one must rule out malignancies or infections. If lesions progress under DMARDs, it is advised to discontinue synthetic DMARDs (LEF/MTX) and switch to another biological DMARD with different modes of action