Gaafar Ragab

Systemic lupus erythematosus (SLE) is an autoimmune disorder characterized by multisystem involvement, including the gastrointestinal (GI) tract. There is a significant variation in the clinical presentation and severity of GI disorders. When GI symptoms present as the initial manifestation of SLE, there is likely to be a delay in the diagnosis. The cause of these GI manifestations in SLE may be the disease, or the side effects of medications, or infections. In this study we investigated the GI manifestations in a group of SLE patients. Our study was conducted on 40 SLE patients and 30 healthy controls to assess the prevalence of GI symptoms in SLE patients. The prevalence of gastrointestinal manifestations in our study was 42.5%. GI manifestations in our SLE patients were: acute abdominal pain (due to pleurisy and peritonitis), 6%; diffuse abdominal pain, 23.5%; epigastric pain, 29%; epigastric pain with vomiting, 23.5%; epigastric pain with chronic constipation, 6%; chronic constipation, 6%; and diffuse abdominal pain with bleeding per rectum, 6%. In our study, we found a higher incidence ofGiardiainfestation in SLE patients than in healthy controls, and 10% of these patients were asymptomatic. There was moreGiardiainfestation in patients with GI symptoms as compared with patients with no GI symptoms, with aPvalue of 0.009. In our study SLE patients with GI symptoms had a peak systolic velocity (cm/s) with a mean of 108.4 ± 32.1 standard deviation (SD) in the celiac Doppler study. Patients without GI symptoms had a peak systolic velocity with a mean of 111.9 ± 37.7 SD, meaning that our patients mostly had no evidence of celiac trunk stenosis, but there was significant difference between SLE patients without GI symptoms and controls, as the mean was higher in SLE patients than in the controls. Also, the celiac end diastolic velocity was higher in both groups of SLE patients with GI symptoms and those without GI symptoms, compared to controls.

Background Serum cryoglobulins (SC) may be found in many diseases (1), and the presence of serum cryoglobulins is a known risk factor for lymphoma evolution in some non malignant diseases.Objectives The aim of this study was to distiguish the role of cryoglobulinemic vasculitis (CV), classified according to the recent validated criteria (1,2), and primary Sjögren's syndrome (pSS) as risk factors of lymphoma in patients positive serum cryoglobulins. Importantly, SC, CV and pSS may occur together.Methods 950 charts from consecutive patients with positive SC were evaluated. Patients carrying both pSS and HCV infection, as well as incomplete charts, were excluded.Results 657 patients with SC were selected, 374 with CV and 283 without CV, according to the published criteria (2,3). PSS, classified according to the American-European Group Criteria was present in 96 patients (44 with CV, 52 without). Lymphoma was reported in 61/657 (9.8%) patients with SC. Among them, CV was present in 44/61 (72,1%; 14 also with pSS), and pSS in 17/61 (27,9%; and 14/17 had CV). Patients with SC with CV showed an higher prevalence of lymphoma than patients with SC without CV (44/374, 11.5% vs.17/283, 6.3%; p=0.025, OR=1.93 [95%IC: 1.08-3.39]. Patients with pSS, SC and CV also showed a higher prevalence of lymphoma than patients with pSS, SC but without CV (14/44, 31.8% vs. 3/52, 7.4%; p=0.001, OR=7.62 [95%CI 2.02-28.74]. CV and pSS were confirmed as independent risk factor for lymphoma by multivariate analysis (OR 2,18 95%CI 1,18-3,83, p=0,012; OR 2,65 95%CI 1,04-6,76, p=0,042, respectively). Infection by the hepatitis C virus (HCV) was detected in 467/561 (83,2%) patients with SC without pSS, and did not statistically predispose to lymphoma when associated with CV in this subset (p=1,0).Conclusions Cryoglobulinemic vasculitis and pSS are independent risk factors for lymphoma in patients with evidence of SC. Patients with both the conditions (CV and pSS) have the highest risk. In the follow-up of SC positive patients, a very high attention should be deserved to pSS, in particular when CV is present.ReferencesDe Vita S, et al. Ann Rheum Dis. 2011; 2) Quartuccio L, et al. Rheumatology (Oxford). 2014Disclosure of Interest None declared

OBJECTIVE: The aim of this study was to validate the classification criteria for cryoglobulinaemic vasculitis (CV).

METHODS: Twenty-three centres were involved. New patients with CV (group A) and controls, i.e. subjects with serum cryoglobulins but lacking CV based on the gold standard of clinical judgment (group B) and subjects without cryoglobulins but with clinical features that can be observed in the course of CV (group C), were studied. Positivity of serum cryoglobulins was necessary for CV classification. Sensitivity and specificity of the criteria were calculated by comparing group A vs group B. The group A vs group C comparison was done to demonstrate the possible diagnostic utility of the criteria.

RESULTS: The study included 268 patients in group A, 182 controls in group B and 193 controls in group C (small vessel vasculitis, 51.8%). The questionnaire (at least 2/3 positive answers) showed 89.0% sensitivity and 93.4% specificity; the clinical item (at least 3/4 clinical involvement) showed 75.7% sensitivity and 89.0% specificity and the laboratory item (at least 2/3 laboratory data) showed 80.2% sensitivity and 62.4% specificity. The sensitivity and specificity of the classification criteria (at least 2/3 positive items) were 89.9% and 93.5%, respectively. The comparison of group A with group C demonstrated the clinical utility of the criteria in differentiating CV from CV mimickers.

CONCLUSION: Classification criteria for CV were validated in a second, large, international study confirming good sensitivity and specificity in a complex systemic disease.