Background This article presents a patient who was initially diagnosed as having granulomatosis with polyangiitis (GPA), and was later diagnosed as having chronic granulomatous disease (CGD) in adulthood. We aimed to raise awareness of CGD, which can be confused with rheumatic diseases. Case Report We present a 33‐year‐old male patient with CGD with recurrent opportunistic bacterial and fungal infections who was diagnosed as having GPA, and had a history of recurrent lung infections and brain abscesses since childhood. The patient, who had cavitary lesions in the lung and mucosal lesions in the nose, was diagnosed as having GPA based on antineutrophil cytoplasmic antibody positivity. CGD was suspected in his last hospitalization after the patient underwent a nitro blue tetrazolium test. Accordingly, neutrophil oxidative function was tested using a dihydrorhodamine assay, which confirmed CGD. Molecular analysis of the patient revealed that the NCF1 gene had a GT deletion at the beginning of exon 2. Our patient was diagnosed as having late‐onset CGD; he is currently well and taking antibiotic prophylaxis. Conclusion As a result of the altered humoral immune response in CGD, there is unregulated inflammation and sustained antigen stimulation. This excessive inflammatory response can be confused with autoimmune diseases and cause delays in diagnosis. This case is important in the differential diagnosis of CGD in adult patients with recurrent opportunistic infections.
To identify the determinants of central sensitization (CS) in patients with axial spondyloarthritis (axSpA). Central Sensitization Inventory (CSI) was used to determine CS frequency. Disease-related variables including Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Ankylosing Spondylitis Disease Activity Score (ASDAS-CRP/-ESR), Maastricht Ankylosing Spondylitis Enthesitis Score (MASES), Bath Ankylosing Spondylitis Functional Index (BASFI), Ankylosing Spondylitis Quality of Life Questionnaire (ASQoL) and Numeric Rating Scale (NRS) GLOBAL were assessed. Biopsychosocial variables were evaluated by the Multidimensional Scale of Perceived Social Support (MSPSS), Brief Illness Perception Questionnaire (B-IPQ), Hospital Anxiety and Depression Scale (HADS) and subscales for Anxiety (HADS-A) and Depression (HADS-D), and Jenkins Sleep Evaluation Scale (JSS). To determine the predictors of the development and severity of CS, multiple linear and logistic regression analyses were performed. The frequency of CS was 57.4% in the study population ( n = 108). CSI score was correlated with the duration of morning stiffness, BASDAI, ASDAS-CRP, ASDAS-ESR, NRS GLOBAL , BASFI, MASES, ASOoL, JSS, HADS, and B-IPQ total scores ( ρ ranged from 0.510 to 0.853). Multiple regression analysis indicated that BASDAI (OR: 10.44, 95% CI: 2.65–41.09), MASES (OR: 2.47, 95% CI: 1.09–5.56) and HADS-A (OR: 1.62, 95% CI: 1.11–2.37) were independent predictors of the development of CS. Additionally, higher NRS GLOBAL , JSS, HADS-D, and HADS-A scores appeared to determine the severity of CS. This study confirms that worse disease activity, more enthesal involvement, and anxiety independently predict the development of CS. Additionally, higher patient-perceived disease activity, sleep impairment and poor mental health significantly contribute to the severity of CS.
Purpose: The aim of this study was to detect infections requiring hospitalization in patients with ANCA-associated vasculitis (AAV). Materials and Methods: This is a single-center, retrospective study conducted in Turkish patients with AAV. Infection episodes requiring hospitalization, reproducing pathogens, laboratory findings, immunosuppressive treatments given for the treatment of vasculitis, and the relationship with the infection were evaluated. Results: Seventy-four patients diagnosed with AAV were included in the study. Hospitalization due to infection was observed in 36 of the patients. The coexistence of diabetes mellitus (DM) was found to be significantly higher in the infected patient group. Cyclophosphamide (CYC) treatment found to increase risk of infection. More than 80% of the infected patient group presented with renal involvement (80.6%). A total of 68 infectious episodes were seen in 36 patients. The most common involvement of infection was the respiratory tract with a rate of 70.6%. Gram-negative bacteria were the most common pathogen, especially Pseudomonas aeruginosa. With the effect of the pandemic, SARS-CoV-2 has come to the fore among viral infections. Aspergillosis was the most frequently detected among fungal infections. Besides, aspergillosis was the cause of 85.7% (6 episodes) of fungal infections. Lymphopenia was observed in 76.5% of the infection episodes. 57.4% of infections developed in the first year of the induction therapy. The most frequently used immunosuppressive therapy for the treatment of vasculitis in infectious episodes was CYC (41.2%). Conclusion: Managing infections during the vasculitis treatment is crucially important. Lymphopenia, kidney involvement, DM and immunosuppressive therapy are factors that increase the risk of infection. Clinicians should take preventive measure especially for respiratory tract infections and gram-negative bacteria as pathogens.
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