RATIONALE:Patients with severe antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)-related diffuse alveolar hemorrhage (DAH) face high mortality. In the PEXIVAS trial, plasma exchange (PLEX) did not reduce death or end-stage kidney disease, but only 9% had severe DAH. OBJECTIVES:This study aimed to assess whether PLEX lowers mortality in patients with severe DAH. METHODS:We emulated a target trial using retrospective data from a national multicenter cohort of patients with severe AAV-related DAH. The primary endpoint was 30-day mortality after intensive care unit (ICU) admission, analyzed using a Cox model adjusted for prespecified confounders. MEASUREMENTS AND MAIN RESULTS:We included 184 patients (median age 66 [53-75] years; 51% female; 51% with granulomatosis with polyangiitis; 53% MPO (Myeloperoxydase)-ANCA positive). Of these, 144 (78.3%) received PLEX, and 40 (21.7%) did not. Baseline characteristics were similar, except for more severe renal impairment (creatinine 357 vs 171 µmol/L, P = .01) and more frequent cyclophosphamide use (77% vs 55%, P = .01) in the PLEX group. Severity at ICU admission (median Simplified Acute Physiology Score II score: 42) and mechanical ventilation needs (54%) were comparable between groups. At 30 days, overall survival was 85%. No significant difference in mortality was observed between the PLEX and no-PLEX groups: 30-day survival was 85% (95% CI, 81-90) with PLEX vs 88% (95% CI, 77-96) without (hazard ratio, 1.23; 95% CI, 0.57-3.89). Secondary outcomes were also similar. CONCLUSIONS:In this emulated target trial, PLEX did not reduce 30-day mortality in patients with severe AAV-related DAH.

Introduction Stroke is an uncommon but pivotal prognostic factor in giant cell arteritis (GCA) and Takayasu arteritis (TAK). While both conditions are large vessel vasculitis stroke characteristics may differ between them, with potential implications for diagnosis and acute management. This study aimed to compare the epidemiological, clinical, and prognostic features of stroke in GCA and TAK. Methods We conducted a multicenter retrospective cohort study including patients who met the ACR/EULAR 2022 classification criteria for GCA or TAK and experienced at least one imaging-confirmed stroke. Patients with transient ischemic attacks, strokes occurring after the age of fifty in TAK, or strokes secondary to atrial fibrillation were excluded. Results A total of 108 patients were analyzed (68 GCA, 40 TAK). The female-to-male ratio was 0.78 in GCA and 5.2 in TAK (p<0.001). Stroke occurred at a mean age of 75±12 years in GCA and 35±11 years in TAK (p<0.001). Hypertension (64.7% vs. 32.4%, p=0.003) and dyslipidemia (36.8% vs. 8.8%, p=0.002) were more frequent in GCA. Cerebellar syndrome (29.4% vs. 0%, p=0.001), cranial nerve involvement (19.7% vs. 0%, p=0.017), and sensory deficits (55.9% vs. 18.4%, p<0.001) were more frequent in GCA. Stroke involved the vertebrobasilar territory in 75% of GCA vs. 20.5% of TAK (p<0.001) and the carotid territory in 35.3% of GCA vs. 79.5% of TAK (p<0.001). Vascular intervention was required in 41% of TAK vs. 6% of GCA (p<0.001). Conclusion Stroke presentation differs between GCA and TAK, with predominant vertebrobasilar involvement in GCA. Carotid involvement is more frequent in TAK and often requires vascular procedures. These differences are crucial for appropriate management.

OBJECTIVES:Polyarteritis nodosa (PAN) is a rare necrotizing vasculitis occurring alone or associated with other conditions, including VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome and chronic myelomonocytic leukaemia (CMML). We aimed to compare the presentation and outcomes of VEXAS- or CMML-associated PAN (VEXAS-PAN and CMML-PAN) with those of primary PAN. METHODS:We conducted a retrospective, multicentre study of patients diagnosed with primary PAN, VEXAS-PAN or CMML-PAN. Each VEXAS-PAN or CMML-PAN case was matched to three primary PAN cases by age and gender. We analysed baseline and therapeutic characteristics, along with survival and relapse rates. RESULTS:Twenty-three patients were included (12 with VEXAS-PAN, 11 with CMML-PAN). Secondary PAN occurred at older ages: 73 years for VEXAS-PAN, 70 years for CMML-PAN and 54 years for primary PAN (P < 0.01). VEXAS-PAN was associated with a higher frequency of skin manifestations (100%), orchitis (67%) and ocular manifestations (58%) than primary PAN. By contrast, the clinical features of CMML-PAN closely resembled those of primary PAN. VEXAS-PAN was less likely than primary PAN to achieve remission [adjusted odds ratio (aOR) 0.15; 95% CI 0.02-1.00] and more likely to experience relapses [adjusted hazard ratio (aHR) 3.24; P = 0.043]. Similar trends were observed for CMML-PAN regarding remission (aOR 0.12; 95% CI 0.02-0.73) and relapses (aHR 2.23; P = 0.145). Mortality was higher in VEXAS-PAN (50%) than in CMML-PAN (15%) or primary PAN (17%). CONCLUSION:VEXAS- and CMML-PAN are distinct clinical entities with poorer prognosis and unique therapeutic challenges. Recognizing these conditions early and providing individualized management are crucial to improving outcomes in these patients.

IgA vasculitis (IgAV) primarily affects small vessels, but rare cases with necrotizing arteritis (NA) raise questions about overlap with polyarteritis nodosa (PAN). To characterize IgAV with necrotizing arteritis (IgAV-NA) and compare its phenotype with classical IgAV and PAN. We performed a multicenter retrospective study combined with a systematic literature review (1990-2025). Patients fulfilled EULAR/PRINTO/PRES IgAV criteria, had pathological or imaging evidence of NA in small or medium arteries, and were ANCA-negative. Thirty patients were included (7 from databases, 23 from the literature). NA was confirmed by biopsy (n = 16) or vascular imaging (n = 14). Clinical features, treatments, remission, and mortality were compared with 257 adult IgAV and 196 PAN patients. Median age was 54.5 years. IgAV-NA was characterized by severe manifestations, including gastrointestinal bleeding, perforation, surgical abdomen, neuropathy, pancreatitis, and livedo. Compared with classical IgAV, IgAV-NA showed significantly higher rates of multi-organ involvement and mortality. Compared with PAN, IgAV-NA shared vascular complications but had less fever and neuropathy. Despite arterial involvement, patients did not fulfil PAN criteria. IgAV-NA represents a rare, severe IgAV phenotype with life-threatening complications rather than an IgAV-PAN overlap. Severe or atypical IgAV presentations should prompt vascular imaging and intensified immunosuppression.

Patients with severe antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)-related diffuse alveolar hemorrhage (DAH) face high mortality. In the PEXIVAS trial, plasma exchange (PLEX) did not reduce death or end-stage kidney disease, but only 9% had severe DAH. This study aimed to assess whether PLEX lowers mortality in patients with severe DAH. We emulated a target trial using retrospective data from a national multicenter cohort of patients with severe AAV-related DAH. The primary endpoint was 30-day mortality after ICU admission, analyzed using a Cox model adjusted for prespecified confounders. We included 184 patients (median age 66 [53–75] years; 51% female; 51% with granulomatosis with polyangiitis; 53% MPO-ANCA positive). Of these, 144 (78.3%) received PLEX and 40 (21.7%) did not. Baseline characteristics were similar, except for more severe renal impairment (creatinine 357 vs. 171 µmol/L, P = 0.01) and more frequent cyclophosphamide use (77% vs. 55%, P = 0.01) in the PLEX group. Severity at ICU admission (median SAPS II score: 42) and mechanical ventilation needs (54%) were comparable between groups. At 30 days, overall survival was 85%. No significant difference in mortality was observed between the PLEX and no-PLEX groups: 30-day survival was 85% (95% CI 81–90) with PLEX vs. 88% (95% CI 77–96) without (HR 1.23; 95% CI 0.57–3.89). Secondary outcomes were also similar. In this emulated target trial, PLEX did not reduce 30-day mortality in patients with severe AAV-related DAH.

Objective. Large vessel-vasculitis (LVV) accounts for up to 70% of patients with giant cell arteritis (GCA). Stenotic involvement in GCA-LVV remains largely unknown. The purpose of this study was to assess the long-term outcome and prognosis of GCA with stenotic LVV. Methods. This was a retrospective multicenter study of 3,149 patients with GCA, including 198 (6.3%) with stenotic LVV. Hierarchical clustering on principal components was performed on baseline arterial localizations and logistic regression assessed factors associated with vascular complications. Results. Stenotic LVV affected mostly the subclavian artery (63%), the carotid artery (58%), vertebral artery (37%), and axillary artery (33%), followed by the femoral artery (30%) and mesenteric arteries (13%). Stroke (31%) was the main complication, followed by limb ischemia (21%), myocardial infarction (4%), and mesenteric ischemia (2%). Cumulative incidence of vascular complications was 13.1% (95% confidence interval [CI] 8.9-18.3), 17.3% (95% CI 12.3-22.9), and 19.5% (95% CI 14.3-25.4), at 1, 5, and 10 years, respectively. Hierarchical clustering analysis identified three clusters among which cluster 1 (n = 123; 62%) included older patients with more arteritic anterior ischemic optic neuropathy (P = 0.04), vertebral artery stenosis, and a higher mortality rate (P < 0.044). In multivariate analysis, age at diagnosis (hazard ratio [HR] 1.06, 95% CI 1.03-1.10; P = 0.0004) and vertebral involvement (HR 1.87, 95% CI 1.03-3.40; P = 0.039) were significantly associated with higher risk of vascular complication. Conclusion. Stenotic LVV accounts for less than 10% of GCA and is associated with poor vascular prognosis.

OBJECTIVE:VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is a recently identified adult-onset autoinflammatory disorder caused by somatic mutations in UBA1. It is characterized by heterogeneous inflammatory and haematological manifestations, making diagnosis challenging. 18F-FDG-PET/CT imaging, which identifies metabolic activity associated with inflammation and malignancy, is often performed during the diagnostic work-up. However, its utility in VEXAS syndrome remains unclear. METHODS:We conducted a multicentre retrospective observational study of patients with genetically-confirmed VEXAS syndrome who underwent 18F-FDG-PET/CT imaging. Clinical, laboratory and imaging data were collected, and 18F-FDG-PET/CT findings were analysed according to disease activity, myelodysplastic syndrome status and mortality. Qualitative 18F-FDG-PET/CT interpretations were based on local nuclear medicine reports. RESULTS:A total of 125 18F-FDG-PET/CT scans were analysed in 66 patients. Bone marrow (83%) and lymph nodes (53%) were the most frequent sites of abnormal FDG uptake, with combinations of bone marrow, lungs, lymph nodes and spleen being the most common patterns. Pulmonary (38%) and vascular involvement (11%) were also observed. The number of organs with abnormal FDG uptake was significantly higher in patients with active disease compared with remission (median 2 vs 1 abnormal sites, P < 0.001). However, 90% of scans performed during presumed clinical remission showed persistent abnormalities, especially in the bone marrow (57%). CONCLUSION:18F-FDG-PET/CT imaging reveals frequent but non-specific abnormalities in VEXAS syndrome, with persistent bone marrow hypermetabolism suggesting subclinical disease activity. While 18F-FDG-PET/CT may have limited diagnostic utility, it holds potential for disease monitoring.

OBJECTIVE:Eosinophilic granulomatosis with polyangiitis (EGPA) is a small vessel vasculitis characterized by eosinophilia, asthma, and ear, nose, and throat (ENT) involvement. Although glucocorticoids (GCs) are effective in controlling symptoms, relapses and GC dependence are common. The aim of this study was to develop predictive models for vasculitis relapse and GC-dependent asthma and/or ENT symptoms. METHODS:This multicenter European retrospective cohort study included patients with EGPA fulfilling the 2022 American College of Rheumatology/EULAR criteria. Using Fine-Gray and logistic regression, we developed two multivariable prediction models, one for vasculitis relapse and another for GC-dependent asthma and/or ENT symptoms at 2 Internal validation was performed using bootstrapping. RESULTS:A total of 809 patients were observed for a median of 72 months (interquartile range 37-115). Vasculitis relapse occurred in 228 patients with a 12-year cumulative incidence of 41.2% (95% confidence interval 36.3-46.8). GC-dependent asthma and/or ENT symptoms were observed in 66.4% at two years. Predictors of vasculitis relapse included age (nonlinear), GC-dependent asthma before EGPA diagnosis (hazard ratio [HR] 1.57), arthralgia (HR 1.27), myocarditis (HR 1.74), peripheral neuropathy (HR 1.39), myeloperoxidase-antineutrophil cytoplasmic antibody (HR 1.56), and baseline eosinophil count (nonlinear). Predictors of GC-dependent asthma and/or ENT symptoms included older age (odds ratio [OR] 0.98 per year), GC-dependent asthma at diagnosis (OR 1.50), chronic sinusitis (OR 1.78), and baseline eosinophil count (OR 0.70 per 109/L). CONCLUSION:Using a large cohort with EGPA, we developed predictive models for vasculitis relapse and GC-dependent asthma and/or ENT symptoms. These tools may help guide treatment decisions. Prospective external validation in the current therapeutic era is warranted.

OBJECTIVE:Systemic sclerosis (SSc) is an autoimmune disease characterized by autoantibody production, fibrosis, and vasculopathy. The coexistence of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) in SSc is rare and poorly characterized, with limited data on the impact of treatments, particularly high-dose glucocorticoids (GCs), on both conditions. This study aimed to describe the clinical phenotype, management, and outcomes of patients with overlapping SSc and AAV. METHODS:We conducted a multicenter retrospective study in 18 French centers, including patients who met the 2013 American College of Rheumatology (ACR)/EULAR criteria for SSc and the 2022 ACR/EULAR criteria for AAV. Clinical, biologic, and radiologic data were collected. RESULTS:We included 30 patients (median age 51.5 years, 83% female). SSc preceded AAV in all cases; 27% had diffuse cutaneous SSc, whereas 73% had limited cutaneous SSc. Anti-Scl70 antibodies were detected in 50%, and interstitial lung disease (ILD) was present in 80%, predominantly with a fibrosing nonspecific interstitial pneumonia pattern (54%). AAV was microscopic polyangiitis in 90%, with myeloperoxidase (MPO)-ANCA positivity in 93%. Renal involvement was common (76%), with a median serum creatinine level of 170 μmol/L (interquartile range [IQR] 120-361 μmol/L) and proteinuria (urine protein to creatinine ratio of 2 g/g creatinine [IQR 0.9-2.3 g/g creatinine]). All patients received GCs in combination with cyclophosphamide (50%) or rituximab (47%). No cases of scleroderma renal crisis were observed. SSc manifestations, including ILD and skin involvement, remained stable during follow-up. CONCLUSION:AAV, predominantly microscopic polyangiitis with MPO-ANCA, can occur in SSc, particularly in patients with fibrosing ILD and anti-Scl70. Standard vasculitis treatments appear to be effective and do not worsen outcomes in SSc.

BACKGROUND:Eosinophilic granulomatosis with polyangiitis (EGPA) is an eosinophilic antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Rituximab has emerged as the standard of care in other types of ANCA-associated vasculitis, but controlled studies on its use in EGPA are yet lacking. OBJECTIVE:To compare rituximab with conventional strategy for the induction of remission in patients with EGPA. DESIGN:Phase 3, multicenter, randomized, controlled, double-blind, superiority trial. (ClinicalTrials.gov: NCT02807103). SETTING:France. PARTICIPANTS:Patients with a diagnosis of EGPA, newly diagnosed or relapsing disease at the time of screening, with active disease defined as a Birmingham Vasculitis Activity Score (BVAS) of 3 or greater. INTERVENTION:Glucocorticoids plus rituximab (1 g 2 weeks apart) compared with the conventional strategy (glucocorticoids alone or in combination with cyclophosphamide in severe forms) for induction of remission. MEASUREMENTS:The primary end point was remission defined as a BVAS, version 3, of 0 and a prednisone dose of 7.5 mg/d or less at day 180. Secondary end points included duration of remission during the study, average daily glucocorticoid dose, and safety. RESULTS:A total of 105 participants were randomly assigned. Thirty-three (63.5%) patients in the rituximab group achieved the primary end point compared with 32 (60.4%) in the control group (relative risk, 1.05 [95% CI, 0.78 to 1.42]; P = 0.75). Results were similar at day 360. The mean duration of remission was 48.5 ± 6.51 weeks in the rituximab group and 49.1 ± 7.42 weeks in the conventional strategy group (P = 0.41). All relapse and major relapse rates were similar between the 2 groups. There was no statistically significant difference in the average daily glucocorticoid dose and no statistically significant differences in the rates of adverse events between the treatment groups. LIMITATION:Design not appropriate to answer the question of equivalence between rituximab and cyclophosphamide in patients with severe EGPA. CONCLUSIONS:Rituximab was not superior to a conventional remission induction strategy in EGPA. PRIMARY FUNDING SOURCE:French Ministry of Health.

Introduction:The identification of prognostic factors for renal failure in antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) remains a challenge. The benefit of plasma exchange (PLEX) has been questioned, and the target population remains to be defined. We investigated the outcome of patients requiring renal replacement therapy (RRT) at baseline and factors associated with their prognosis at 1 year. Methods:This retrospective multicenter study evaluated the 1-year composite end point of death or end-stage kidney disease (ESKD) in patients with biopsy-proven renal AAV involvement. Results:Of the 394 patients included, 105 (26.6%) were on dialysis at baseline. Of these, 60 (57.1%) reached the composite end point compared with 29 patients (10.0%) who were not on RRT at baseline (P < 0.001). On multivariate analysis, age and sex were not associated with the composite outcome (P = 0.945 and P = 0.154, respectively); however, myeloperoxidase (MPO)-ANCA was (odds ratio [OR]: 3.60; 95% confidence interval [CI]: 1.79-7.60), as was a high baseline histologic renal risk score (OR: 1.29; 95% CI 1.17-1.44). The most strongly associated factor remained the need for dialysis at baseline (OR: 10.91; 95% CI: 5.52-22.70). Of the 91 patients surviving after requiring dialysis at baseline, 45 were weaned from RRT (49.5%) at 1 year, and PLEX was independently associated with a reduced risk of the composite outcome (OR: 0.23, 95% CI: 0.05-0.80). Conclusion:MPO-ANCA, need for dialysis, and high histological renal risk score at baseline were associated with the 1-year composite end point of death or ESKD. Almost half of the patients on dialysis at baseline were off dialysis at 1 year, with a better prognosis in those who had received PLEX.

OBJECTIVES:Current recommendations suggest treating eosinophilic granulomatosis with polyangiitis (EGPA) without severe manifestations with glucocorticoids (GCs) and EGPA with severe manifestations with GCs plus cyclophosphamide (CYC) regardless of poor-prognostic factors. However, GCs plus CYC and GCs alone have never been compared in EGPA without poor-prognosis factors assessed by the 1996 Five Factor Score (FFS). We aimed to compare the efficacy of GCs plus CYC vs GCs alone for the treatment of EGPA without poor-prognosis, including among patients with severe manifestations. METHODS:We emulated a target trial using observational data from a European multicentre retrospective study. We included patients with (i) newly diagnosed EGPA, (ii) a FFS = 0 at diagnosis and (iii) treated with GCs or GCs plus CYC. Primary outcome was overall relapse at 12 months. Inverse probability of treatment weighting-based analysis was used to adjust for potential confounding factors. In a subgroup analysis, we focused on patients with severe manifestations not included in the FFS. RESULTS:A total of 250 patients were included: 177 treated with GCs alone and 73 with GCs plus CYC. After adjustment, no reduction in the risk of overall relapse was observed between the two treatment groups. Similar results were observed in the subgroup of patients with severe manifestations. CONCLUSION:This study shows that the adjunction of CYC to GCs does not reduce the risk of relapse in patients with EGPA and no poor-prognosis factors. It supports current guidelines in patients without severe manifestations but challenges the need of CYC adjunction in patients with severe manifestations but no poor-prognosis factors.

Systemic sclerosis (SSc) is an autoimmune disease characterized by autoantibody production, fibrosis, and vasculopathy. The coexistence of ANCA‐associated vasculitides (AAV) in SSc is rare and poorly characterized, with limited data on the impact of treatments, particularly high‐dose glucocorticoids (GCs), on both conditions. This study aimed to describe the clinical phenotype, management, and outcomes of patients with overlapping SSc and AAV. We conducted a multicenter retrospective study in 18 French centers, including patients who met the 2013 ACR/EULAR criteria for SSc and the 2022 ACR/EULAR criteria for AAV. Clinical, biologic, and radiologic data were collected. We included 30 patients (median age 51.5 years, 83% female). SSc preceded AAV in all cases; 27% had diffuse cutaneous SSc, while 73% had limited cutaneous SSc. Anti‐Scl70 antibodies were detected in 50%, and interstitial lung disease (ILD) was present in 80%, predominantly with a fibrosing non‐specific interstitial pneumonia pattern (54%). AAV was microscopic polyangiitis in 90%, with MPO‐ANCA positivity in 93%. Renal involvement was common (76%), with a median serum creatinine of 170 μmol/l (IQR 120‐361) and proteinuria of 2 g/g (IQR 0.9‐2.3). All patients received GCs in combination with cyclophosphamide (50%) or rituximab (47%). No cases of scleroderma renal crisis were observed. SSc manifestations, including ILD and skin involvement, remained stable during follow‐up. AAV, predominantly microscopic polyangiitis with MPO‐ANCA, can occur in SSc, particularly in patients with fibrosing ILD and anti‐Scl70. Standard vasculitis treatments appear to be effective and do not worsen outcomes in SSc.

Objectives IgA vasculitis is a rare disease in adults, with a relapsing or refractory course in some cases. Non-severe cutaneous relapses have been less documented than those with renal involvement, and data on the therapeutic management of such situations are lacking. We aimed to evaluate the cutaneous response rate with different treatment regimens. Methods This French retrospective multicenter study included patients with IgA vasculitis who experienced cutaneous relapses or refractory disease after first-line therapy. The primary endpoint was the rate of cutaneous response at month 3. Results Fifty-two patients were included, 64% of whom were male. First-line therapy was colchicine in 54% and low-dose glucocorticoids (GCs) in 38%. After failure of the first-line therapy, patients received a median of 1 [1-2.3] additional line of treatment. Cutaneous response at 3 months was achieved in 82% with dapsone alone, 73% with GCs alone or in combination with colchicine or dapsone, 71% with cDMARDs and 57% with colchicine. The relapse rate was 46% with dapsone alone, 23% with GCs alone or in combination with colchicine or dapsone, 29% with cDMARDs and 7% with colchicine. Conclusion In adult relapsing or refractory cutaneous IgA vasculitis, dapsone, GCs and cDMARDs provided the highest rates of cutaneous response. Considering the risk-benefit ratio and potential adverse events, dapsone appears to be an interesting option in adult relapsing or refractory cutaneous IgA vasculitis.

OBJECTIVES:To describe the characteristics and outcome of patients with the association of large vessel vasculitis (LVV, Takayasu arteritis [TA] or GCA) and IBD. METHODS:An observational, multicentre, retrospective case-control study. Cases were LVV-IBD patients from European countries, whereas controls had isolated LVV (iLVV). RESULTS:A total of 39 TA-IBD and 12 GCA-IBD cases were enrolled, compared with 52 isolated GCA (iGCA) and 93 isolated TA (iTA) controls. LVV occurred after IBD in 56% in TA-IBD and 75% in GCA-IBD, with a median interval of 1 year (interquartile range [IQR] 1-7) in TA-IBD and 8.6 years (IQR 1-17.7) in GCA-IBD. Crohn's disease was more common in TA-IBD (67%), whereas ulcerative colitis was more common in GCA-IBD (58%). Compared with iTA, TA-IBD were significantly younger at diagnosis of TA (median age 27 vs 37 years, P < 0.001) and had more upper limb claudication (36% vs 12%, P = 0.006). GCA-IBD patients had more frequent arterial thickening or stenosis than controls (75% vs 30%, respectively, P = 0.044) and tended to more frequently involve gastrointestinal arteries (20% vs 0%, respectively, P = 0.06). LVV occurred in IBD patients despite treatment with glucocorticoids (36%), azathioprine (25%) or TNF-alpha blockers (29%). The presence of the IBD was not associated with a higher LVV relapse rate in multivariate analysis (adjusted hazard ratio [aHR] 0.62 [0.13-2.83] for GCA and aHR 0.92 [0.44-1.89] for TA). CONCLUSION:This study identifies specific clinical and imaging characteristics of LVV-IBD patients, in particular a more severe vascular presentation of GCA-IBD patients compared with iGCA patients.

BACKGROUND AND AIMS:IgG4-related disease (IgG4-RD) is a rare disease considered an acquired systemic autoimmune condition. Myotonic dystrophy type 2 (DM2) is a rare dominantly inherited multisystem disorder, with a high prevalence of associated autoimmune diseases, but IgG4-RD has not been described in this context. METHODS:A case series of three patients with concurrent IgG4-RD and DM2. RESULTS:All three patients, from a cohort of 47 patients with DM2 (prevalence = 6%), were male, aged 61-80 years and exhibited at least pancreatic involvement. Elevated IgG4 levels were observed in blood, and two patients had lymphoplasmacytic infiltrates rich in IgG4+ plasma cells and CD4+ T cells, with fibrosis present in biopsies. In two cases, DM2 was diagnosed after IgG4-RD. All patients presented with a myopathic phenotype in the lower limbs, with myotonic discharges at myography. INTERPRETATION:The prevalence of IgG4-RD in the cohort of DM2 herein is more than 1000 times higher than expected. As both diseases display common organ involvement, especially the pancreas, IgG4-RD screening should be considered in DM2 patients with diabetes or/and atypical associated phenotypes. Additionally, genetic testing for DM2 should be considered in IgG4-RD patients with elevated creatine kinase levels, myopathic phenotype, cardiac disorders and/or cataracts. The present report also suggests that IgG4-RD may have a genetic predisposition, potentially elucidating an aspect of the disease's pathophysiology.

Background: The treatment and prognosis of cryoglobulinemia vasculitis (CryoVas) depend on etiology. Rituximab (RTX) and corticosteroids (CS) are the first line treatment for mixed essential (ME) CryoVas and connective tissue disease (CTD)-related CryoVas. The prognosis and long term outcomes of these forms of CryoVas are as yet unknown. Objectives: The aim of this study was therefore to describe the risk of relapse and treatment-related morbidities in patients with ME and CTD-related CryoVas. Methods: A retrospective study was conducted of 63 patients in remission of ME or CDT-related CryoVas after RTX-CS therapy, with a median follow-up time of 58 months (interquartile range, 33–88 months). Results: Thirty-nine out of 63 patients (62%) had a relapse a median of 42 (23–65) months after the initial flare. The relapse incidence was 38% at 2 years and 46% at 3 years. The factors associated with relapse were purpura at the time of the qualifying flare (HR, 2.2; 95% confidence interval (CI), 1.1–4.4; p = 0.002) and prior history of CryoVas flares (HR, 1.9; 95% CI, 1.0–3.7; p = 0.04). Maintenance therapy was associated with a lower risk of relapse 6–24 months after the initial flare (HR, 0.27; 95% CI, 0.09–0.78; p = 0.02), but not thereafter (HR, 2.0; 95% CI, 0.7–5.7; p = 0.21). The most common form of maintenance therapy was 500 mg RTX every 6 months. The most frequent complication was infection, and maintenance RTX therapy was associated with a higher risk of severe infection (HR, 2,2; 95% CI, 0.9–5,6; p = 0.08). Conclusion: In this group of patients in RTX-CS remission of ME and CTD-related CryoVas, relapses were common and the risk of relapse was significantly associated with purpura during the qualifying flare and a prior history of relapse. 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Efficacy of low-dose rituximab for the treatment of mixed cryoglobulinemia vasculitis: Phase II clinical trial and systematic review. Autoimmun Rev. 2015 Oct;14(10):889–96. [9] Colantuono S, Mitrevski M, Yang B, Tola J, Carlesimo M, De Sanctis GM, et al. Efficacy and safety of long-term treatment with low-dose rituximab for relapsing mixed cryoglobulinemia vasculitis. Clin Rheumatol. 2017 Mar 1;36(3):617–23. Acknowledgements: NIL. Disclosure of Interests: Claire POGGI: None declared, Eric Hachulla BOEHRINGER INGELHEIM, GSK, SANOFI,, JANSSEN-CILAG, Bayer HealthCare SAS, GSK, BRISTOL MYERS SQUIBB, IQVIA Operations France, United Therapeutics Corporation, NOVARTIS PHARMA SAS, CHUGAI PHARMA FRANCE, ASTRAZENECA, MSD, JANSSEN-CILAG, Bayer HealthCare SAS, GSK, BRISTOL MYERS SQUIBB,, GENZYME, SANOFI, RE-IMAGINE Health Agency, Otsuka Pharmaceutical France SAS, AXONAL, PFIZER SAS, LIVE! 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