Vasculitis in systemic lupus erythematosus: prevalence and clinical characteristics in 670 patients.

We conducted the current study to determine the prevalence and clinical characteristics of vasculitis in a large series of patients with systemic lupus erythematosus (SLE), focusing on the classification and clinical significance of the different types of vasculitis. We studied 670 consecutive patients who fulfilled 4 or more of the 1997 revised criteria for SLE. Definite vasculitis was diagnosed histologically and/or by arteriography, and probable vasculitis was diagnosed clinically when there were characteristic cutaneous lesions. Vasculitides were categorized according to the definitions adopted by the Chapel Hill Consensus Conference. Seventy-six (11%) patients with SLE had vasculitis (68 female patients and 8 male; mean age, 37.8 yr); only 32 (42%) fulfilled the Chapel Hill definitions. Cutaneous lesions were the main clinical presentation of vasculitis, present in 68 (89%) patients, while the remaining 8 (11%) had isolated visceral vasculitis. Compared with SLE patients without vasculitis, patients with vasculitis had a higher prevalence of livedo reticularis (22% vs. 3%; p = 0.028); a higher mean European Consensus Lupus Activity Measurement (ECLAM) score (5.86 vs. 3.87; p < 0.001); and a higher frequency of anemia (62% vs. 17%; p < 0.001), erythrocyte sedimentation rate (ESR) >50 mm/h (60% vs. 15%; p < 0.001), and anti-La/SS-B antibodies (19% vs. 5%; p = 0.014) in the multivariate analysis. With respect to the size of the vessels involved, 65 (86%) patients had small vessel vasculitis (SVV) and 11 (14%) had medium-sized vessel vasculitis (MVV). SLE patients with MVV had a higher prevalence of mononeuritis multiplex (54% vs. 2%; p < 0.001), visceral vasculitis (100% vs. 5%; p < 0.001), and ulcerated/ischemic cutaneous lesions (36% vs. 11%; p = 0.047) and a higher percentage of surgical interventions (45% vs. 0%; p < 0.001) compared with patients with SVV. In conclusion, we observed a heterogeneous presentation of vasculitides arising in the setting of SLE, with nearly 60% of cases not fulfilling the names and definitions adopted by the Chapel Hill Consensus Conference. SVV was the most frequent vasculitis, overwhelmingly cutaneous and clearly differentiated from MVV, which was less frequent but had predominantly visceral involvement (especially of the peripheral nerves). The presence of vasculitis in our patients with SLE was associated with a higher ECLAM score, livedo reticularis, hematologic parameters (anemia, high ESR), and anti-La/SS-B antibodies.

Atypical autoantibodies in patients with primary Sjögren syndrome: clinical characteristics and follow-up of 82 cases.

OBJECTIVES: To analyze the clinical characteristics, follow-up, and fulfillment of classification criteria for other systemic autoimmune diseases (SAD) in patients with primary Sjögren syndrome (SS) and atypical autoantibodies. METHODS: We studied 402 patients diagnosed with primary SS seen consecutively in our Department since 1994. We considered anti-DNA, anti-Sm, anti-RNP, anti-topoisomerase1/Scl70, anticentromere (ACA), anti-Jo1, anti-neutrophil cytoplasmic antibodies (ANCA), anticardiolipin antibodies (aPL), and lupus anticoagulant as atypical autoantibodies. The patients were prospectively followed after inclusion into the protocol, focusing on the development of features that might lead to the fulfillment of classification criteria for additional SAD. As a control group, we selected an age-sex-matched subset of patients with primary SS without atypical autoantibodies. RESULTS: Eighty-two (20%) patients showed atypical autoantibodies (36 had aPL, 21 anti-DNA, 13 ANCA, 10 anti-RNP, 8 ACA, 6 anti-Sm, 2 anti-Scl70, and 1 anti-Jo-1 antibodies). There were 77 (94%) women and 5 (6%) men, with a mean age of 57 years. Patients with atypical autoantibodies had no statistical differences in the prevalence of the main sicca features, extraglandular manifestations (except for a higher prevalence of Raynaud's phenomenon, 28% versus 7%, P=0.001), immunological markers, and in the fulfillment of the 2002 classification criteria, compared with the control group. After a follow-up of 534 patient-years, 13 (16%) of the 82 patients with atypical autoantibodies developed an additional SAD (systemic lupus erythematosus in 5 cases, antiphospholipid syndrome in 4, limited scleroderma in 3, and microscopic polyangiitis in 1) compared with none in the control group (P<0.001). CONCLUSIONS: This study shows an immunological overlap (defined by the presence of autoantibodies considered typical of other SAD) in 20% of our patients with primary SS. However, the clinical significance of these atypical autoantibodies varies widely depending on the autoantibodies detected, with a broad spectrum of prevalence and clinical patterns of disease expression, and a specific predilection for association with some SAD in preference to others.