Clinical and laboratory aspects of Ro/SSA-52 autoantibodies.

Anti-Ro/SSA antibodies, which were described for the first time in systemic lupus erythematosus (SLE) and Sjögren's syndrome (SS), are the most prevalent extractable nuclear antigen (ENA) specificity identified in laboratories. Two types of anti-Ro/SSA antibodies have been described, anti-SSA-52 kDa (aSSA52) and anti-SSA-60 kDa (aSSA60), each specific to different antigens. Anti-Ro/SSA52 autoantibodies are more frequent than other autoantibodies possibly because of the antigen's accessible and ubiquitous nature. The sites involved and the symptoms associated with these autoantibodies depend on the antigen's structural variability. Isolated congenital complete atrioventricular block (CAVB) shows a close association with maternal anti-Ro/SSA and anti-La/SSB antibodies; the highest relative risks of CAVB are seen in offspring of mothers with antibodies against 52-kDa Ro and 48-kDa La proteins. Anti-Ro/SSA52 antibodies have little impact on adult rheumatic autoimmune diseases or adult cardiac arrhythmias, but the course of autoimmune liver diseases is greatly worsened by their presence, and solid tumours tend to relapse. Their diagnostic role in rheumatic diseases is controversial, although a significant association between isolated anti-Ro/SSA52-kDa positivity and myositis and to a lesser extent with systemic sclerosis (SSc) has been described. However, the majority of the specific diagnosis is mostly based on the simultaneous presence of other autoantibodies that seems diagnostically more relevant.

[Frequency and clinical associations of antineutrophil cytoplasmic antibodies. A regional experience]. / Frequenza e associazioni cliniche degli anticorpi anti citoplasma dei neutrofili. Un'esperienza locale.

Serologic and clinical aspects of 50 positives patients for antineutrophil cytoplasmic antibodies (ANCA) have been evaluated (age range 7-94 years, mean age 43 years). 40 (80%) were females. Antineutrophil nuclear antibodies (pANNA), in which the antigenic specificity is unknown, were detected in seventeen patients (34%). About half of these cases (8 patients) had primary sclerosing cholangitis and other 7 patients had severe ulcerative colitis. Two pANNA patients, with increased susceptibility to infections, had undefined diagnosis. Both had thalassemic trait. Anti MPO were detected in 9 patients in which segmental lesions prevail and anti-PR3 were detected in 9 patients with granulomatous component. The patients with higher levels of these autoantibodies (40%) had the typical syndromes described in literature (vasculitis ANCA-related) although patients with lower autoantibodies levels (60%) mostly present variable clinical symptoms with unspecified diagnosis. Fourteen patients were positive for atypical ANCA detectable with commercial kits. They present variable clinical symptoms with unspecified diagnosis but show granulomatous or neoplastic lungs and bowel involvement. Both have mostly contact with environmental microorganisms. All cases are characterized by chronic inflammatory lesions in which the relapses correlate with infectious disorder.