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Background and Aims

Giant Cell Arteritis (GCA) is a systemic vasculitis of medium and large arteries. Inflammation of carotid artery branches gives rise to the classic GCA symptoms. However, GCA can involve the aorta and its major branches, leading to a different atypical clinical picture, challenging to diagnose. This study aims to analyze the clinical presentation of large-vessel GCA compared to classical cranial disease.

Methods

100 consecutive GCA-patients were enrolled in this retrospective study. Based on vascular involvement, patients were classified in only cranial arteritis (C-GCA), only extracranial large-vessel vasculitis (LV-GCA) or both cranial and large-vessel vasculitis (LV-C-GCA).

Results

61 patients had C-GCA, 16 LV-GCA and 23 LV-C-GCA. Compared to C-GCA, patients with large vessel involvement (LV-GCA and LV-C-GCA) were younger and more frequently women, with a further significant difference in patients with isolated LV-GCA. Patients with isolated LV-GCA had also the longer duration of symptoms at GCA diagnosis. Systemic symptoms, as fever and fatigue, were associated with large-vessel involvement. Polymyalgia rheumatica was equally reported in all three groups and no significant differences were found in inflammatory markers levels. In patients with large-vessels vasculitis, thoracic aorta and subclavian arteries were the most frequently involved arteries.

Conclusions

the different clinical manifestations of large-vessel GCA lead to a longer time to diagnosis. Female gender, younger age and systemic symptoms are associated with large-vessel vasculitis, regardless the presence or absence of cranial symptoms. In patients with these characteristics, a large-vessel involvement should be considered in order to reduce the time to diagnosis.

Background and Aims

Patients with ANCA-associated vasculitis (AAV) exhibit higher rates of malignancy than general population, but data on eosinophilic granulomatosis with polyangiitis (EGPA) are still lacking. The aim of the study was to investigate the incidence of malignancies in EGPA patients and to examine the effect of immunosuppressive therapy on malignancy risk in these patients.

Methods

The study population included 72 EGPA patients (61% female, 48% MPO-ANCA positive, 55 [42-62] years old at diagnosis), diagnosed between 1993 and 2018. Demographic, clinical and laboratory data, and immunosuppressive drugs were assessed. Incidence rates from the Italian Cancer Registry were used to compare malignancy incidence in the AAV cohort and to obtain age- and sex-standardized incidence ratios (SIR).

Results

During the 285 person-years observation period, we found 13 cancers in 8 of the 72 patients, during a median follow-up of 47.5 [16-108] months. The SIR (95% CI) malignancy risk was 2.37 (1.02-4.66) for cancers at all sites, and 2.90 (1.25-5.71) for all cancers excluding non-melanoma skin cancers. Median latency from EGPA onset and first cancer diagnosis was 2.5 (1-6.5) years, with 62.5% of patients developing cancer within 1 and 5 years. Comparing patients who developed malignancies with those who did not, no significant difference was noted regarding sex, ANCA status, age at diagnosis, clinical manifestations, BVAS, FFS, environmental exposure, smoking habit and cancer familiarity. Type of treatment and cumulative doses of cyclophosphamide were not associated with higher incidence of cancers.

Conclusions

EGPA patients have a persistent increased risk for overall malignancy compared to general population, irrespective of type of treatment.

Background and Aims

The study evaluates 6 described phenotypes of Behcet's disease (BD) - cutaneous-mucous, articular, ophthalmic, vascular, neurological and intestinal in different ethnic groups.

To assess the occurrence of BD phenotypes in different ethnic groups.

Methods

The study included 202 patients with BD from the 5 most common ethnic groups. The male-female ratio was 2.4:1. Patients’ mean age was 31 years [24;37], mean age at the disease onset was 21 years [15;28]; and mean disease duration was 7 years [3;14]. The severity of BD (mild, moderate and severe) was assessed based on the I. Krause’s Clinical Severity Scoring for BD.

Results

Severe BD was more often diagnosed in Azerbaijanis and indigenous residents of Dagestan compared to Russians (75 and 70.4% vs. 36.2%), in Armenians - 50% and Chechens - 54.5% out of all BD cases. Russians were significantly more likely to have a neurological phenotype (15.5% vs. 0-9.4% in all other ethnic groups) and intestinal phenotype (36.2% vs. 13.8-22.7 in all other ethnic groups). Azerbaijanis demonstrated higher prevalence of ocular involvement (68.7% versus 36.2% in Russians, 50% - in Chechens and Armenians, and 57% - in Dagestanis). Dagestanis were more likely to have a vascular phenotype (40.7% versus 15.6% in Azerbaijanis and 18.9% in Russians). The male/female ratio among Russian patients was 1:1, among Dagestanis 4.4:1, Azerbaijanis 3.5:1, Chechens and Armenians 2.6: 1.

Conclusions

BD phenotypes vary and demonstrate significant association with the patient’s ethnic affiliation therefore, ethnicity should be viewed as the prognostic marker of specific organ-system involvement in case of a disease.