Primary Sjögren's syndrome in children and adolescents: proposal for diagnostic criteria.

Objective: Primary Sjögren's syndrome (pSS) in childhood is a rare disease. Diagnostic criteria are available for adult patients only. In order to establish diagnostic criteria for juvenile pSS an analysis of 7 girls and one boy suffering from pSS with early onset is reported.

Identification of common epitopes on gliadin, enterocytes, and calreticulin recognised by antigliadin antibodies of patients with coeliac disease.

Background: Sera of patients with coeliac disease, containing IgA and IgG antigliadin antibodies (AGA) and various IgA autoantibodies, react with isolated enterocytes. AGA cross react with enterocyte antigens, one of which has been identified as calreticulin.

Aims: To characterise the antigenic structures of gliadin, enterocytes, and calreticulin recognised by AGA from patients with active coeliac disease.

IL-10 gene promoter polymorphisms in rheumatoid arthritis.

IL-10 is an anti-inflammatory cytokine which may modulate disease expression in RA. Three dimorphic polymorphisms within the IL-10 gene promoter have recently been identified and appear to influence regulation of its expression. The 1082*A allele has been associated with low and the 1082*G allele with high in vitro IL-10 production.

Effect of 1 year cyclosporine A treatment on the activity and renal involvement of systemic lupus erythematosus: a pilot study.

Our objective was to determine the effect of 1 year low-dose cyclosporine A (CSA) treatment on disease activity and renal involvement in systemic lupus erythematosus (SLE). Patients included in the pilot study had an active form of the disease as defined by the SLE Disease Activity Index (SLEDAI). Main organ involvement was represented by lupus nephritis classified in repeated renal biopsies.

Heterogenous nuclear RNP C1 and C2 core proteins are targets for an autoantibody found in the serum of a patient with systemic sclerosis and psoriatic arthritis.

Objective: To determine a target recognized by anti-Bh autoantibody, found in the serum of a patient with the unusual coexistence of systemic sclerosis (SSc) and psoriatic arthritis (PsA).

Methods: Antigens recognized by the anti-Bh serum were characterized by indirect immunofluorescence on HeLa cells, by conventional immunoblotting using nuclear extract or partially purified preparation of heterogenous nuclear RNP (hnRNP) proteins, and by 2-dimensional immunoblotting. For the analysis of cross-reactivity and immunofluorescence patterns, autoantibodies were affinity-purified by blot elution and then retested.

[Practical results of monitoring pregnancy in women with systemic lupus erythematosus].

Background: The authors dealt with the urgent problem under what conditions it is possible to achieve in a woman with systemic lupus erythematosus (SLE) or another collagenosis, or secondary antiphospholipid syndrome (APS) a favourable outcome of pregnancy and the delivery of a healthy infant.

Methods And Results: The investigation comprised 23 women incl. 20 with SLE, two with the mixed form of a diffuse connective tissue disease (MCTD) and one with Sjögren's syndrome of the primary type.

[Rheumatoid pericarditis: correlation with immunologic parameters].

In 35 patients with rheumatoid pericarditis the laboratory findings were compared with those of a group of 65 patients with rheumatoid arthritis without a pericardial exudate. The authors assessed the red cell sedimentation rate and serum levels of immunoglobulins G, M, A, semiquantitative values of the rheumatoid factor, circulating immunocomplexes, C3 and C4 complement components, the positivity of antinuclear antibodies and values of C-reactive protein. The authors found significantly elevated values of immunoglobulins M in patients with rheumatoid arthritis without a pericardial exudate.

Monozygotic rheumatoid arthritis twin pairs express similar levels of conserved immunoglobulin V gene in polyclonal rheumatoid factors irrespective of disease status.

The degree of polyclonal RF heterogeneity was assessed in diseased and non-diseased twins with rheumatoid arthritis (RA). The distribution of variable region determinants encoded by a set of immunoglobulin germline, or minimally mutated germline, genes within IgM RF, IgG RF and IgA RF isotypes was determined by ELISA using specific mouse monoclonal antibodies (MoAb) in fractionated plasma from 12 members of six monozygotic twin pairs with RA. The results reveal that at least 40% (range approximately 18-87%) of IgM RF are encoded by a small set of approximately 10 genes from the VH1, 3 and 4 families.

Heterogeneity of disease phenotype in monozygotic twins concordant for rheumatoid arthritis.

The objective of the study was to investigate the genetic contribution to the clinical expression of rheumatoid arthritis (RA) by comparison of disease features in RA-concordant monozygotic (MZ) twin pairs. Fourteen RA-concordant MZ twin pairs recruited from a nation-wide study were examined to determine the degree of similarity in: (a) age of disease onset; (b) pattern of joint involvement; (c) pattern of extra-articular disease; (d) toxic reactions to drugs; (e) disease course; and (f) serology for rheumatoid factor (RF) and antinuclear antibody. There was considerable within-pair diversity in the variables studied.

Clinical associations of IgG antibodies to the ribonucleoprotein p67 polypeptide in patients with systemic lupus erythematosus.

The ribonucleoprotein (RNP) p67 antigen was purified from rabbit thymus and used in an enzyme linked immunosorbent assay (ELISA) with low interassay variability to detect IgG antibodies to p67 in patients with autoimmune connective tissue diseases. These antibodies were found in eight (80%) patients with a clinical diagnosis of mixed connective tissue disease (MCTD) but also in 27 (40%) patients with systemic lupus erythematosus (SLE). Sixty six per cent of the 12 patients with SLE with high levels of antibodies to p67 (> 50 U) had three or more features of MCTD, including myositis, fibrosing alveolitis, Raynaud's phenomenon, and sclerodactyly.

[Use of pulsed steroid therapy in active rheumatoid arthritis].

The authors implemented a prospective double blind, controlled study where they administered to a group of 34 patients with active rheumatoid arthritis either three pulses a 1000 mg methyl prednisolone in a small infusion or an infusion with placebo. They proved a very favourable effect--after five days a marked diminution of morning stiffness was recorded, as well as a diminution of pain during locomotion and rest and general improvement of the patient's condition. After two weeks the effect declines but even after 8 weeks the difference, as compared with the initial value, was still significant, while this was not the case after placebo.

[Antineutrophil cytoplasmic antibodies--ANCA].

Antibodies against cytoplasm of neutrophil leucocytes (ANCA) with a high sensitivity are found in idiopathic necrotizing vasculitis and idiopathic glomerulonephritis with formation of sickles (IGS). C-ANCA are directed against the myeloid lysosomal enzyme--proteinase 3 and are found above all in Wegener's granulomatosis and microscopic polyarteritis. P-ANCA are directed mainly against myeloperoxidase, less against leucocytic elastase or lactoferrin and are found above all in microscopic polyarteritis, IGS, polyarteritis nodosa or Churg-Strauss syndrome.

[Rheumatoid factor in rheumatoid arthritis in old age].

Rheumatoid factors (RF) detected by agglutination methods (latex fixation test, haemagglutination test) were found in a group of patients with rheumatoid arthritis which started in advanced age (RAVV) less frequently than in a group with RA in middle age (RAMV). The author describes the ELISA method for assessment of RF which makes it possible to estimate immunoglobulin classes RF (IgM-RF, IgA-RF, IgG-RF). By this method RF were detected in class IgM in 15% of patients and in some immunoglobulin classes in 20 per cent where are RF negative when assessed by agglutination methods.

[New findings on the pathogenesis of rheumatoid arthritis].

In the submitted review the authors present some new findings and approaches pertaining to the pathogenesis of rheumatoid arthritis (RA). First they describe cells participating in the development of the articular inflammation and mediators which transmit signals between these cells and which may lead to their activation. The authors mention the immune complex role of rheumatoid factors and some new theories on their development.

Acute cricopharyngeal obstruction in dermatomyositis.

We report a case of a 44-year-old woman with dermatomyositis complicated by acute total cricopharyngeal obstruction resistant to corticosteroid therapy. This condition was successfully resolved by cricopharyngeal myotomy.