Background/Objectives: Granulomatosis with polyangiitis (GPA) is a necrotising vasculitis characterised by granulomatous inflammation involving small vessels. In addition to specific findings for the affected organ, constitutional symptoms and joint and muscle pain can be observed. The prodromal phase, where symptoms last for months before clinical diagnosis, may suggest infection or malignancy. This may cause a delay in GPA diagnosis. The period from the first symptoms to diagnosis may last from one month to three years. In this study, we aimed to demonstrate that, as the time between the onset of symptoms and diagnosis increases, the disease involvement may become more severe, and the possibility of recurrence may increase, indicating the importance of early diagnosis. Methods: For this cross-sectional retrospective study, data from 40 patients with GPA were evaluated. Demographic, clinical, treatment, and follow-up characteristics of the patients were obtained from their medical records. Results: The mean time between the presentation of the first complaints and GPA diagnosis was 7.40 ± 11.84 (0, 60, 3; mean ± standard deviation [minimum, maximum, and median]) months. The time between the first complaints and diagnosis was longer for patients with a history of recurrence (11.44 ± 16.73 [0, 60, 4.5] vs. 4.71 ± 6.04 [0, 24, 2.5], p value = 0.260). Conclusions: GPA is an inflammatory disease with various clinical presentations. In the management of patients with GPA, awareness of its presentation is important for rheumatologists as well as other clinicians during the initial evaluation, demonstrating the importance of interclinical collaboration.

Familial Mediterranean fever (FMF) is characterized by recurrent episodes of fever and serositis. Blood-based biomarkers determined in FMF patients during attack-free periods could be used to predict the risk of amyloidosis and the severity of the disease. The recently defined pan-immune-inflammation value (PIV) comprises four distinct subsets of blood cells and serves as an easily accessible and cost-effective marker. The objective of this study was to assess the role of PIV in predicting amyloidosis and moderate-to-severe disease. Clinical characteristics and laboratory values during the attack-free period were retrospectively analyzed in 321 patients over 18 years of age diagnosed with familial Mediterranean fever (FMF). In our tertiary adult rheumatology outpatient clinic, disease severity and laboratory markers were evaluated during the first attack-free interval. At baseline, patients with amyloidosis were excluded. Patients were categorized based on the presence of amyloidosis and the severity of the disease. When focusing on amyloidosis in receiver operating characteristic (ROC) analysis, optimal cut-off values for pan-immune-inflammation value (PIV), neutrophil-to-lymphocyte ratio (NLR), and platelet-to-lymphocyte ratio were determined as ≥518.1, ≥2.3, and ≥127.2, respectively. In multivariate analysis, PIV, C-reactive protein (CRP), and the presence of the M694V homozygous mutation emerged as independent risk factors for both amyloidosis and moderate-to-severe disease. Additionally, NLR was identified as an independent risk factor for amyloidosis, while red blood cell distribution width was associated with moderate-to-severe disease. In patients with FMF, especially in the presence of the M694V homozygous mutation, CRP and PIV may be useful in predicting both amyloidosis and moderate-to-severe disease.

Objective: The aim of this study is to assess the efficacy and safety of nintedanib (NTD) therapy in a real world population of patients with connective tissue diseases related interstitial lung disease (CTDs-ILD). Methods: Our multicenter retrospective study included patients with a CTD-ILD diagnosis who started NTD treatment due to the development of PPF during follow-up. The results of the percentage predicted forced vital capacity (%pFVC) and percentage predicted diffusion capacity (%pDLCO) of patients before NTD treatment and 6, 12 and 18 months after the start of NTD treatment were evaluated. In addition, the patients were divided into two groups, SSc-ILD and other CTDs-ILD, and compared in terms of the efficacy and safety of the NTD. Results: In all patients (n = 66), %pFVC and %pDLCO values stabilised after 6, 12 and 18 months compared to baseline values. All patients received at least one immunosuppressive therapy in combination with NTD treatment. The most common side effect after NTD treatment was diarrhoea (n = 20, 30.3%). When we divided the patients into two groups, SSc-ILD (n = 35) and other CTDs-ILDs (n = 31), no significant difference was found between the groups in the change in %pFVC (p values = 0.498, 0.595 and 0.376, respectively) and in the change in %pDLCO (p values = 0.817, 0.185 and 0.399, respectively) at 6, 12 and 18 months follow-up. Again, there was no statistically significant difference between the two groups in terms of adverse events and safety data after NTD treatment (p > 0.05). Conclusion: In summary, the use of NTD in combination with immunosuppressive therapies was effective and safe in SSc-ILD patients as well as in other CTDs-ILD patients. Key Points• This multicenter study provides real-world data on the use of nintedanib in patients with connective tissue disease-interstitial lung disease.• Nintedanib treatment is as effective and safe in patients with other connective tissue disease as in patients with systemic sclerosis.