Search Results (187)

Background/Objectives: The relationship between large-scale natural disasters and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) remains uncertain. This study compared crude case numbers, diagnostic patterns, and clinical characteristics of newly diagnosed AAV before and after the 6 February 2023 Kahramanmaraş earthquakes in Türkiye. Methods: This multicenter retrospective study included patients newly diagnosed with AAV between 6 February 2020 and 5 February 2026 at five tertiary referral centers in four provinces heavily affected by the earthquakes. Equal 36-month pre- and post-earthquake periods were compared. Demographics, AAV subtype, ANCA serology, biopsy-confirmed diagnosis, organ involvement, Birmingham Vasculitis Activity Score (BVAS), laboratory findings, treatments, and recorded outcomes were evaluated. Results: A total of 127 patients were included: 58 (45.7%) before and 69 (54.3%) after the earthquakes. The crude number of cases was numerically higher after the earthquakes, but the difference was not statistically significant (p = 0.375). AAV subtype, ANCA serology, organ involvement, BVAS, inflammatory markers, renal function, and most laboratory findings were comparable. Biopsy-confirmed diagnoses were significantly less frequent in the post-earthquake period than in the pre-earthquake period (46.4% vs. 70.7%, p = 0.010). Treatment patterns were comparable between the two periods. Although the crude proportions of recorded outcomes did not differ significantly, these comparisons were limited by the substantially shorter follow-up duration in the post-earthquake group and should not be interpreted as evidence of comparable prognosis. Conclusions: In major tertiary referral centers serving earthquake-affected provinces, crude AAV case numbers and baseline clinical phenotypes were largely comparable before and after the earthquakes. These crude case numbers should not be interpreted as incidence estimates, because reliable population denominators were unavailable after the earthquakes. The significant reduction in biopsy-confirmed diagnoses may reflect post-disaster changes in diagnostic healthcare pathways. These real-world data provide an important regional overview of newly diagnosed AAV after a major natural disaster. Full article

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare vasculitis. A key bio-logical hallmark is peripheral blood eosinophilia while antineutrophil cytoplasmic an-tibody (ANCA) positivity is not always present. Therefore, an early phase suspicion of EGPA leading to a correct diagnosis can be challenging. We performed an observa-tional cross-sectional study limited to baseline data on 38 patients between December 2019 and December 2025 at the Allergy and Clinical Immunology Unit, Messina, Italy. Data collection included eosinophil, neutrophil, and lymphocyte counts; ANCAs and antinuclear antibodies (ANAs); erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) determination; and the Birmingham vasculitis activity score (BVAS) questionnaire: Median eosinophil counts were 685 cells/µL, the neutro-phil-to-lymphocyte ratio (NLR) was 2.29, ESR median values were 24 mm/h, and CRPs were 1.99 mg/L. ANCAs were positive in five patients, and ANAs in 20. The trend in ANA+ patients was of lower median eosinophil counts and higher ESR and CRP me-dian values and median BVASs. General, nervous system, and ENT BVAS domains had higher scores.: Disease activity in EGPA at diagnosis can be difficult to recognize. We defined it as chameleon-like and could not be captured by specific immunological sig-natures. Therefore, an early diagnosis needs multidimensional assessment, integrating laboratory parameters with clinical scores. In our EGPA cohort, ANAs were detected in just over half the patients, suggesting that rather than acting as bystanders, these could help clinicians, together with eosinophil counts, NLRs, and BVASs, to suspect EGPA at an early stage for referral to specialized medical centers. Full article

Background/Objectives: Nontuberculous mycobacterial pulmonary disease (NTM-PD) has been associated with myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA)-positive ANCA-associated vasculitis (AAV). However, clinical factors associated with MPO-ANCA positivity in patients with NTM-PD remain unclear. This study determined the frequency of MPO-ANCA positivity among tested patients with NTM-PD and explored the clinical factors associated with MPO-ANCA positivity. Methods: This study retrospectively analyzed patients aged ≥15 years old with NTM-PD who were treated at Tokyo Shinagawa Hospital and underwent MPO-ANCA testing between April 2020 and March 2024. Clinical data, including nontuberculous mycobacterial species, serum Mycobacterium avium complex (MAC) antibody titers, radiographic findings, and nodule, infiltration/consolidation, cavity, and ectasis scores, were collected from their electronic medical records. Patients were classified into MPO-ANCA-positive and negative groups, and clinical factors were compared. Results: Among 109 patients with NTM-PD, 49 underwent testing for MPO-ANCA and proteinase 3 (PR3)-ANCA. MPO-ANCA was positive in five patients (10.2%), whereas PR3-ANCA was positive in one (2.0%). Among the five MPO-ANCA-positive patients, three had MAC antibody titers above the upper limit of measurement (>10 U/mL), and one developed AAV with biopsy-proven pauci-immune crescentic glomerulonephritis and mononeuritis multiplex. The MAC antibody titer was significantly higher in the MPO-ANCA-positive group than in the MPO-ANCA-negative group [10.0 (3.89–10.0) vs. 1.92 (0.19–6.19) U/mL, p = 0.038]. Conclusions: MPO-ANCA positivity was observed in a subset of tested patients with NTM-PD and was associated with higher MAC antibody titers. These findings suggest that chronic MAC-related antigen exposure or local pulmonary disease activity in NTM-PD may be associated with MPO-ANCA positivity. Full article

Granulomatosis with polyangiitis (GPA) is a systemic vasculitis frequently associated with serum anti-neutrophil cytoplasmic antibodies (ANCAs), particularly anti-proteinase 3 (PR3). Multisystem involvement is typical, although disease onset with simultaneous manifestations affecting both the ocular and otorhinolaryngologic systems is rare and poorly reported in the literature. We describe a 65-year-old woman with renal impairment with microscopic hematuria and proteinuria and PR3-positive who developed bilateral otitis leading to sensorineural hearing loss and severe anterior scleritis, with a high suspicion of ANCA-associated vasculitis. Despite immunosuppression therapy, persistently elevated serum anti-PR3 levels in the absence of overt clinical activity prompted further diagnostic evaluation, which revealed previously unrecognized subglottic stenosis (SGS), confirmed by MRI, and fully resolved after anti-CD20 therapy. Concurrent involvement of the upper airways and ocular district as the initial presentation of GPA is unusual and may precede renal involvement by several months. Persistent elevation of PR3 levels in an apparent state of clinical remission may indicate ongoing active disease in other anatomical sites. The integration of laboratory tests, imaging studies, and multidisciplinary assessment is essential for early diagnosis and effective therapeutic management. Full article

Relapsing polychondritis (RP) is a rare autoimmune disease of cartilaginous structures, often diagnosed late due to nonspecific presentations. Both RP and granulomatosis with polyangiitis (GPA) can cause diffuse tracheobronchial wall thickening on computed tomography (CT) and may be seronegative for anti-neutrophil cytoplasmic antibody (ANCA), creating a diagnostic impasse. We report a 46-year-old man with two months of fever, productive cough, and sternal pain. A saddle nose deformity was the only cartilaginous sign; serum ANCA was repeatedly negative. Neck CT showed diffuse tracheal and bilateral main bronchial wall thickening; the report listed amyloidosis and GPA as differential diagnoses, omitting RP. Despite laboratory, microbiological, and imaging workup, the fever fulfilled criteria for fever of unknown origin (FUO), prompting ¹⁸F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT. PET/CT demonstrated intense FDG uptake in the cartilaginous wall of the tracheobronchial tree, forming the classic inverted-Y sign, with bilateral costal cartilage hypermetabolism (a site not involved in GPA) and no uptake in the kidneys, sinuses, or orbits, collectively establishing a diagnosis of RP. Corticosteroid therapy elicited prompt clinical and biochemical response. This case demonstrates that ¹⁸F-FDG PET/CT can differentiate RP from GPA when CT and serology are uninformative. Full article

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) represents a group of rare systemic autoimmune disorders marked by inflammation and damage to small- and medium-sized blood vessels. The clinical presentation of AAV is highly variable, ranging from isolated organ involvement to severe, life-threatening multisystem disease, posing significant challenges in diagnosis, treatment, and prognosis. Objective: To demonstrate the clinical heterogeneity and different outcomes in three pediatric cases of ANCA-positive disease and emphasize the importance of integrating clinical findings with laboratory and imaging investigations for accurate diagnosis. Methods: We present three pediatric patients (ages 12–15 years) with ANCA-positive results but distinct clinical presentations, evaluated at the Children’s Emergency Hospital “Louis Turcanu”, Timisoara, between 2020 and 2024. All cases were investigated according to EULAR/PRINTO/PReS criteria for pediatric vasculitis. Results: Case 1 (PR3-ANCA positive) developed severe multi-organ involvement, including granulomatosis with polyangiitis (GPA) with pulmonary hemorrhage, pericarditis, thrombotic events, and renal impairment, requiring intensive immunosuppression with cyclophosphamide, rituximab, and mycophenolate mofetil, ultimately developing chronic kidney disease stage 3a. Case 2 (BPI-ANCA positive) presented with purpuric lesions and painless joint swelling, responding favorably to corticosteroid therapy with subsequent remission. Case 3 (MPO-ANCA) manifested as polyarticular arthritis without other organ involvement and was ultimately diagnosed as seronegative juvenile idiopathic arthritis (JIA), achieving complete remission with adalimumab therapy. Conclusions: This case series highlights the diverse clinical and biological features of ANCA-positive conditions in children, emphasizing that ANCA positivity requires careful clinical correlation as it may indicate true vasculitis requiring aggressive treatment or alternative diagnoses such as JIA with incidental ANCA positivity. Tailored therapeutic strategies based on clinical presentation and continued research are essential to improve patient outcomes. Full article

Circulating immune complexes (CICs) are frequently detected in the sera of patients with myeloperoxidase (MPO)–antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (MPO-AAGN) and their role in activating the classical complement pathway has been suggested. However, the precise composition and functional characteristics of CICs in AAGN remain poorly understood. We analyzed serum samples from four patients with MPO-AAGN and confirmed CICs. An immunoadsorbent column was prepared using a monoclonal rheumatoid factor antibody that binds to CICs, enabling extraction via immunoprecipitation. CICs were analyzed by Western blotting to detect MPO and IgG. Their capacity to activate complement was assessed in vitro by measuring complement activation products using the enzyme-linked immunosorbent assay following incubation with normal human serum. Western blotting revealed distinct bands for both MPO and IgG in all samples. Incubation of extracted CICs with normal human serum resulted in elevated levels of complement activation products, including C5a and C5b-9. This increase was completely inhibited by the addition of EDTA or EGTA. In conclusion, CICs in the serum of patients with MPO-AAGN contain MPO and IgG (presumably MPO-ANCA). These CICs can activate the complement system, likely through the classical pathway. Our findings support the hypothesis that CICs composed of MPO and MPO-ANCA contribute to complement activation via the classical pathway and play a significant role in AAGN pathogenesis. Full article

Background: Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated necrotizing vasculitis primarily affecting small and medium-sized vessels. The typical presentation commonly includes upper and/or lower respiratory tract and renal involvement. GPA has a particularly strong association with proteinase-3 (PR3) ANCA. Though well defined, GPA may be clinically difficult to recognize, particularly in early disease. Initial presentations may include nonspecific symptoms, including but not limited to fatigue, fever, and sinus congestion or sinusitis, which may be mistaken for infection. Though initial ANCA testing is useful, it is not definitive as early stages of disease may be negative, thus delaying diagnosis; Clinical Significance: This case highlights the importance of including GPA in the differential diagnosis of patients with unremitting upper or lower respiratory and constitutional symptoms despite negative ANCA testing. Though atypical, GPA cases may lack renal involvement and even have negative ANCA serologies, leading to a delay in diagnosis and increased morbidity. ANCA positivity can be as low as 60% in limited GPA cases, and less than 20% of individuals have renal involvement at presentation. If GPA suspicion is high, repeat testing and biopsy are warranted; Case Presentation: A woman in her 50s initially presented to the emergency department with recurrent/persistent fever with nonspecific sinus symptoms that remained unresolved despite multiple outpatient treatments and tests. Infectious work-up was negative. She was found to have multiple pulmonary nodules on various scans. Initial testing on admission was unremarkable or nondiagnostic, including anti-neutrophil cytoplasmic antibody (ANCA) serologies. The patient’s hospital course was complicated by acute hypoxic respiratory failure with distributive shock during bronchoscopy. Repeat serological testing was positive for PR3-ANCA, and lung biopsy demonstrated necrotizing granulomatous vasculitis consistent with a diagnosis of granulomatosis with polyangiitis (GPA). The patient demonstrated clinical improvement with avacopan, glucocorticoids, and rituximab; Conclusions: The diagnosis of GPA should be suspected in all patients with nonspecific constitutional symptoms along with clinical evidence of upper/lower respiratory tract involvement, regardless of renal function. Physicians with a strong suspicion of an autoimmune disease, such as GPA, should utilize a thorough clinical history, physical exam, and other labs in the setting of a negative autoimmune marker and/or negative imaging. Clinical judgment is required to not rule out GPA despite a negative workup when other more serious causes have been excluded, as the diagnosis may be life-threatening. Full article

Background: Rapidly progressive glomerulonephritis (RPGN) is a severe nephrological emergency, frequently secondary to anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. In older adults, the coexistence of comorbidities and monoclonal gammopathy of undetermined significance (MGUS) makes it difficult to distinguish between ANCA vasculitis and monoclonal gammopathy of renal significance (MGRS), which differ in prognosis and treatment. The coexistence of PR3-ANCA-associated vasculitis and MGUS is uncommon and sparsely documented. Case Presentation: A 72-year-old woman with hypertension and type 2 diabetes presented with acute deterioration and rapidly progressive renal failure, requiring hemodialysis. She had subnephrotic proteinuria, hematuria, and an active urinary sediment. The autoimmune workup showed ANCA negativity using immunofluorescence, but PR3-ANCA positivity using ELISA. Hematologic characterization documented an IgG kappa monoclonal spike; no bone lesions, amyloidosis, or criteria for multiple myeloma were found; and the patient was classified as MGUS. Renal biopsy revealed necrotizing extracapillary pauci-immune glomerulonephritis with cellular and fibrocellular crescents and no monoclonal deposits, consistent with PR3-ANCA vasculitis. Induction therapy with methylprednisolone pulses and oral prednisone was initiated; cyclophosphamide was not administered because of catheter-associated Staphylococcus aureus bacteremia and upper gastrointestinal bleeding complicated by disseminated intravascular coagulation. The patient died on day 25 due to infectious and hemorrhagic complications. Conclusions: This case provides additional documentation of an uncommon overlap between PR3-ANCA-associated vasculitis and MGUS in a Latin American patient and highlights the role of renal biopsy in distinguishing MGRS from pauci-immune vasculitis in the presence of paraproteinemia. It also underscores the need to tailor immunosuppression in frail older adults, balancing disease control against the risk of severe infection. Full article

Background: The diagnostic interpretation of antineutrophil cytoplasmic antibody (ANCA) testing remains challenging due to variable concordance between indirect immunofluorescence (IIF) patterns and antigen-specific assays targeting proteinase 3 (PR3) and myeloperoxidase (MPO). The additional value of formalin fixation in distinguishing true perinuclear ANCA (P-ANCA) patterns from redistribution artifacts in routine laboratory practice remains incompletely defined. Methods: We conducted a retrospective real-world analysis of 7276 patients who underwent concurrent ANCA IIF (ethanol- and formalin-fixed substrates), anti-PR3, and anti-MPO testing between 2022 and 2024. Concordance between IIF and antigen-specific assays was assessed using cross-tabulation and Cohen’s kappa statistics. Associations were evaluated using chi-square tests and odds ratios (ORs) with 95% confidence intervals (CIs). The impact of formalin reactivity within P-ANCA patterns and the relationship between ANCA titer strength and antigen positivity were analyzed using the chi-square test for trend. Results: Concordance between ANCA IIF and PR3 antibodies was poor (κ = 0.109; p < 0.001), with 59% of PR3-positive patients being IIF-negative. Agreement between ANCA IIF and MPO antibodies was stronger but remained modest (κ = 0.221; p < 0.001); 77% of MPO-positive patients were IIF-positive. Formalin-resistant P-ANCA patterns showed a strong association with MPO positivity (OR = 67.3; 95% CI 33.3–136.6; p < 0.001), whereas MPO positivity was uncommon in formalin-sensitive patterns. Increasing ANCA titers were associated with progressively higher MPO positivity (chi-square test for trend, p < 0.001), rising from 0.4% in ANCA-negative cases to 33.3% in the highest observed titer category. Dual PR3/MPO positivity was rare (0.16%). Conclusions: In this large cohort, ANCA IIF demonstrated differential diagnostic performance depending on antibody specificity. IIF showed limited sensitivity for PR3-associated autoimmunity, whereas MPO positivity correlated more strongly with IIF patterns and formalin resistance. Integration of antigen-specific assays with formalin-based pattern interpretation may improve the interpretative accuracy of ANCA testing and should be considered in routine laboratory algorithms. Full article

Background/Objectives: Personalized treatment approaches are increasingly recognized as essential in the management of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), given the substantial heterogeneity in disease severity and patient characteristics. Kidney biopsy has the potential to serve as an effective tool for personalized treatment decision-making in patients with AAV. This study aimed to investigate the association of kidney biopsy with intensive immunosuppressive therapy and clinical outcomes in patients with AAV and kidney impairment. Methods: In this retrospective study, propensity score overlap weighting was applied to compare intensive immunosuppressive therapy and clinical outcomes (ESKD, death, combined ESKD and death, and infectious complications) between patients with AAV who underwent kidney biopsy and those who did not. Results: Out of 74 patients with AAV, 38 underwent kidney biopsy. Overlap weight analysis revealed that kidney biopsy was significantly associated with intensive immunosuppressive therapy (risk difference [RD], 28.9%; 95% confidence interval [CI], 0.017 to 0.562). Kidney biopsy was not associated with combined ESKD and death (RD, −0.2%; 95% CI, −0.302 to 0.298), death (RD, −3.8%; 95% CI, −0.264 to 0.189), ESKD (RD, −7.3%; 95% CI, −0.353 to 0.207), and infectious complications (RD, −25.9%; 95% CI, −0.537 to 0.020). Conclusions: In this observational cohort, kidney biopsy was associated with intensification of immunosuppressive therapy. However, after adjustment using overlap weighting, no statistically significant difference in clinical outcomes was detected, and the reduced effective sample size limited statistical power. These findings should be interpreted cautiously, as causal inference regarding the prognostic impact of kidney biopsy remains limited. Full article

Background: Perianal Crohn’s disease (PD) remains a major therapeutic challenge, with heterogeneous responses to anti-TNF therapy and limited real-world data on predictors of long-term outcomes. This study aimed to evaluate clinical and radiological response to anti-TNF therapy initiated exclusively for PD and to identify factors associated with treatment response. Methods: A retrospective study was conducted in a cohort of 65 patients with PD treated with anti-TNF. The primary endpoint was clinical response assessed at weeks 24, 52, and 60 months. It was defined as a ≥50% reduction in drainage, and remission as complete absence of drainage. Radiological response was assessed by magnetic resonance imaging at the same time points whenever feasible. Multivariate logistic regression analyses were performed to identify independent predictors of response. Results: At week 24, 84.6% of patients achieved a clinical response, while radiological response was observed in 30.8%. At week 52, clinical and radiological response rates were 80.0% and 52.3%, respectively. At 60 months, 61.5% maintained clinical response and 46.1% radiological response. Among patients who responded at week 24, 90.7% maintained response at week 52, with a secondary loss of response rate of 9.3%. Multivariate analysis identified absence of antineutrophil cytoplasmic antibodies (ANCA) as an independent predictor of clinical response at week 52 (OR 0.06, 95% CI 0.006–0.59; p = 0.01). No significant associations were observed between anti-TNF serum levels and clinical or radiological outcomes. Conclusions: In this real-world cohort of patients initiating anti-TNF exclusively for PD, early response (week 24) emerged as a potential marker of long-term outcomes, highlighting the importance of early reassessment and individualized therapeutic strategies. Full article

Vasculitides are a heterogeneous group of disorders characterized by inflammation of blood vessel walls, leading to tissue ischemia and organ injury. Traditional inflammatory markers such as the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are widely used but lack diagnostic specificity. This has driven the search for more informative biomarkers across vasculitis subtypes. This review summarizes current evidence for validated and emerging biomarkers in large-, medium-, small-, and variable-vessel vasculitis, as well as single-organ vasculitis. Key analytes reflect systemic inflammation, such as serum amyloid A (SAA) and interleukin-6 (IL-6), as well as endothelial activation, complement pathways, neutrophil and macrophage activation, and organ-specific damage. Promising candidates include pentraxin-3 (PTX3) and matrix metalloproteinase-9 (MMP-9) in large-vessel vasculitis; N-terminal pro-B-type natriuretic peptide (NT-proBNP) and S100 proteins in Kawasaki disease; galactose-deficient immunoglobulin A1 (Gd-IgA1) and urinary angiotensinogen (AGT) in IgA vasculitis; and tissue inhibitor of metalloproteinases-1 (TIMP-1), S100 proteins, complement C3, and PTX3 in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Although these biomarkers provide mechanistic insight, most lack disease-specificity, external validation, or standardized assays. Future progress will require multicenter studies, harmonized testing, and integrated biomarker panels combined with imaging modalities to improve diagnosis, activity assessment, and monitoring. Full article

Background/Objective: In recent years, artificial intelligence (AI) has gained increasing prominence in the fields of diagnostic decision-making in medicine. The aim of this study was to compare multidisciplinary team (MDT: rheumatology, pulmonology, thoracic radiology) decisions with single-session plans generated by ChatGPT-4o. Methods: In this cross-sectional concordance study, adults (≥18 years) with confirmed systemic autoimmune rheumatic disease (SARD) and having MDT decisions within the last 6 months were included. The study documented diagnostic, treatment, and monitoring decisions in cases of SARDs by recording answers to six essential questions: (1) What is the most likely clinical diagnosis? (2) What is the most likely radiological diagnosis? (3) Is there a need for anti-inflammatory treatment? (4) Is there a need for antifibrotic treatment? (5) Is drug-free follow-up appropriate? and (6) Are additional investigations required? Consequently, all evaluations were performed with ChatGPT-4o in a single-session format using a standardized single-prompt template, with the system blinded to MDT decisions. All data analyses in this study were conducted using the R programming language (version 4.3.2). An agreement between AI-generated and MDT decisions was assessed using Cohen’s Kappa (κ) statistic where κ (kappa) values represent the level of agreement: <0.20 = slight, 0.21–0.40 = fair, 0.41–0.60 = moderate, 0.61–0.80 = substantial, >0.80 = almost perfect agreement. These analyses were performed using the irr and psych packages in R. Statistical significance of the models was evaluated through p-values, while overall model fit was assessed using the Likelihood Ratio Test. Results: A total of 47 patients were involved in this study, with a predominance of female patients (61.70%, n = 29). The mean age was 61.74 ± 10.40 years. The most frequently observed diagnosis was rheumatoid arthritis (RA), accounting for 31.91% of cases (n = 15). This was followed by cases of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, interstitial pneumonia with autoimmune features (IPAF), and sarcoidosis. The analyses indicate a statistically significant level of agreement across all decision types. For clinical diagnosis decisions, agreement was moderate (κ = 0.52), suggesting that the AI system can reach partially consistent conclusions in diagnostic processes. The need for an immunosuppressive treatment and follow-up without medication decisions demonstrated a higher level of concordance, reaching the moderate-to-high range (κ = 0.64 and κ = 0.67, respectively). For antifibrotic treatment decisions, agreement was moderate (κ = 0.49), while radiological diagnosis decisions also fell within the moderate range (κ = 0.55). The lowest agreement—though still moderate—was observed in further investigation required decisions (κ = 0.45). Conclusions: In patients with SARDs with pulmonary involvement, particularly in complex cases, concordance was observed between MDT decisions and AI-generated recommendations regarding prioritization of clinical and radiologic diagnoses, treatment selection, suitability for drug-free follow-up, and the need for further diagnostic investigations. Full article

Background: Antineutrophil cytoplasmic antibody-associated systemic vasculitis (AAV) most often involves the kidneys, upper airways and lungs, and peripheral and central nervous systems (PNS, CNS). However, in contrast to PNS, the involvement of the CNS is rarely taken into account in the recognition and assessment of systemic vasculitis, probably because of nonspecific symptoms such as headaches and dizziness, aphasia, memory disorders, or mood changes. In addition, it is not clear whether treatment of systemic vasculitides reduces cerebral vascular alterations. In this study, we aimed to evaluate the effects of AAV treatment on vascular and vasogenic alterations in the brain in patients with acute vasculitis onset with renal involvement. Methods: Twenty-nine patients (17F, 12M, age 60.4 ± 9.8) with AAV relapse with renal involvement were included in the study. The initial baseline assessment and the second evaluation, performed 12.6 ± 2.5 months after the beginning of immunosuppressive treatment, included clinical, neurological, and renal function assessments, along with a brain MRI. Results: Compared with baseline, improvement in clinical, neurological, and renal function was observed during the second clinical evaluation. A significant reduction in the occurrence of vascular dilatation and narrowing in secondary (37.9% vs. 17.2%; p = 0.031) and tertiary (37.9% vs. 10.3%; p = 0.008) cerebral vascular branches was observed. However, the number of vasogenic cerebral white matter lesions detected on the FLAIR sequence increased significantly (36.0 vs. 48.0%; p < 0.001). Conclusions: Intensive immunosuppressive treatment of acute-onset systemic AAV with renal involvement decreases disease activity, improves kidney function, and decreases central nervous system vascular but not vasogenic alterations. Full article