Evaluation of disease activity by laboratory tests in juvenile rheumatoid arthritis.

We studied 29 juvenile rheumatoid arthritis patients with polyarticular onset in order to detect the presence of disease activity by laboratory tests. Laboratory studies included hemoglobin, hematocrit, erythrocyte sedimentation rate (ESR), white blood cell (WBC) and platelet counts. We also determined the levels of IgG, IgA, IgM, C3, C4 and C reactive protein, as well as the presence of rheumatoid factor and antinuclear antibodies. There were 13 patients in the group with polyarticular disease activity, and 16 in the group with asymptomatic juvenile rheumatoid arthritis. Low hematocrit and hemoglobin values, abnormal ESR and elevated WBC and platelet counts occurred in polyarticular juvenile rheumatoid arthritis patients with active disease. Increased levels of IgA, C3 and C4 were also found in the exacerbation stage, as compared to the asymptomatic control group. No differences were found in rheumatoid factor and antinuclear antibodies between the active and inactive juvenile arthritis patients. Our data agreed with previous studies in that a single laboratory test cannot necessarily confirm juvenile arthritis activity, and they suggest that two or more abnormal parameters would be useful in assessing the flare-up of the disease.

Pulmonary function abnormalities in patients with primary Sjögren's syndrome.

Thirty-two patients with primary Sjögren's syndrome (SS) were studied in order to determine the frequency of pulmonary disease using respiratory function tests. Eleven of the 32 patients revealed positive symptoms compatible with lung disease, including dyspnea, dry or productive cough and pleuritic pain. Seven of these 11 patients also showed a positive physical examination, and 5 showed abnormal chest roentgenograms. Restrictive lung disease was detected in 28.1% of the patients, obstructive disease in 21.8%, and 25% showed a combination of these features (mixed disease). Only 8 patients had completely normal function. There was no significant relationship between respiratory changes and age, smoking habits or glandular and extraglandular manifestations. Positive antinuclear antibodies (p < 0.03) detected by the indirect immunofluorescence test, and anti-SS-A(Ro) and anti-SS-B(La) antibodies (p < 0.009) detected by Ouchterlony occurred more frequently in those patients with primary SS and restrictive pulmonary disease. The patients with anti-SS-A(Ro) and anti-SS-B(La) antibodies also showed significantly lower values for total lung capacity (TLC) (p < 0.0001) and forced vital capacity (FVC) (p < 0.0005) than patients without these serum abnormalities. The study of primary SS may help to elucidate the prevalence of lung abnormalities and their relationship with serum autoantibodies.

Anti-dsDNA and Sm autoantibodies in systemic lupus erythematosus.

During the present study the coincidence of anti-dsDNA and Sm antibodies was detected in 16 percent of 51 consecutive SLE patients. These antibodies were detected by the standard indirect immunofluorescence and Ouchterlony tests. All patients with anti-dsDNA and Sm antibodies showed disease activity, including renal, CNS and pulmonary disease. We excluded a cross reactivity of these antibodies by ELISA, using competitive experiments with dsDNA and Sm antigens. The results support the presence of multiple autoantibody production during SLE activity, and suggest that different mechanisms may underlie the induction and regulation of both autoantibodies.

[Generalized sclerosis (scleroderma) in children]. / Esclerosis generalizada (esclerodermia) en pacientes pediátricos.

Five patients with childhood scleroderma, were studied from a total group of 50 cases with the disease, 39 of them with diffuse systemic sclerosis and 11 with the CREST syndrome. The average age for these five patients when the disease onset was 13 (the age ranged from 5.5 to 16 years) with an average follow-up of 3.6 years (ranging from 1 to 6.5 years). Of the five, four girls were classified as having diffuse systemic sclerosis and the remaining boy, as suffering from the CREST syndrome. We found no family history or personal and occupational antecendents related with the appearance of the illness. Also excluded were conditions associated with changes similar to scleroderma as are seen in cases of diabetes mellitus, phenylketonuria, toxic oil syndrome, or graft-host rejection reactions. The clinical manifestations seen at the start of the disease included the Raynaud phenomenon, subcutaneous edema and muscular-skeletal abnormalities as arthralgia and myalgia with objective data of inflammatory myopathy. Proximal scleroderma was seen in all five patients; three of them, in addition, developed rapidly progressive cutaneous changes, causing the loss of elasticity and cutaneous hardening of the face during the first year of the disease. In all of the cases, the skin biopsy showed histopathological changes compatible with the diagnosis already given. The most important changes seen in the organs of these children were oesophageal dysfunction and fibrosis of the lung. The X-rays of three of the patients showed them to suffer from intestinal malfunction. We found no kidney, liver or nervous system disorders.(ABSTRACT TRUNCATED AT 250 WORDS)

[Autoantibodies in juvenile rheumatoid arthritis. Clinical and serologic study]. / Autoanticuerpos en artritis reumatoide juvenil. Estudio clínico y serológico.

Twenty five patients with juvenile rheumatoid arthritis were studied in order to define the frequency and kind of circulating autoantibodies in this entity, as well as, their relationship with the different disease subgroups and complications. Also the association of these autoantibodies with the activity stage of the illness was determined. There were 14 pauciarticular, 8 polyarticular and 3 systemic juvenile rheumatoid arthritis patients. Twelve have a flare of the disease and 13 were completely asymptomatic. Rheumatoid factor measured by the latex agglutination tests was positive in 6 children. These included 2 patients with pauciarticular disease, showing titers below 1:40 and 4 cases with polyarticular disease, showing titers above 1:320. One of these last patients developed and adult type rheumatoid arthritis during her evolution and was treated with D-penicillamine. The polyarticular but not the pauciarticular patients showed positive tests for rheumatoid factor by nephelometry. No definite association between these laboratory results and the activity of the disease was noted. Positive antinuclear antibodies by the indirect immunofluorescence test were found in 3 pauciarticular and one polyarticular patients. A predominant homogeneous staining was found with the mouse kidney substrate, whereas homogeneous and speckled patterns were noted with the homologous HEp-2 cells. One patient with persistent positive antinuclear antibodies revealed uveitis. Three of the 4 sera with positive antibodies by the indirect immunofluorescent test have also anti-nucleoprotein antibodies by the hemagglutination test with titers above 1:32.(ABSTRACT TRUNCATED AT 250 WORDS)

Circulating autoantibodies in patients with pigeon breeder's disease.

Sera from 19 patients with hypersensitivity pneumonitis induced by avian antigens were studied in order to determine the presence of circulating autoantibodies. IgM and IgG rheumatoid factors were positive in 68% and 100% of the cases respectively. IgM-rheumatoid factor was detected with at least two methods, showing titers between 1:20 and 1:1280 by the latex agglutination test and between 140 and 579 IU/ml by nephelometry test. The IgG rheumatoid factor was studied by the indirect immunofluorescence technique, showing positive determinations in all of our hypersensitivity pneumonitis patients. Titers of these autoantibodies ranged from 1:80 to 1:640. In addition, we studied the presence of antinuclear, anti-nDNA, anti-mitochondrial, and anti-smooth muscle antibodies by the immunofluorescence test using HEp-2 cells, mouse kidney, and Crithidia luciliae targets. Sera from all of our hypersensitivity pneumonitis patients have negative results of autoantibodies to these antigens. Negative results of autoantibodies to the nRNP, Sm, SS-A(Ro) and SS-B(La) nuclear antigens by counterimmuno-electrophoresis and double immunodiffusion techniques were also obtained. As controls we studied 14 healthy individuals and 8 subjects exposed to avian antigens but without hypersensitivity pneumonitis symptoms and no positive determinations for rheumatoid factor, antinuclear antibodies, as well as to anti-mitochondrial and anti-smooth muscle antibodies, were found. These findings support that different immune abnormalities are present in patients with hypersensitivity pneumonitis induced by avian antigens. One of these immune alterations or a combination of them may promote or facilitate the acute interstitial lung injury and/or perpetuate a chronic inflammatory process.

[Systemic lupus erythematosus in children with renal and hematologic manifestations. Relation to anti-nDNA antibodies]. / Lupus eritematoso generalizado en niños con daño renal y hematológico. Relación con anticuerpos anti-DNAn.

We studied prospectively 22 children with systemic lupus erythematosus. Serologic tests included: immunoglobulins, C3 and C4 determinations. Antinuclear antibodies, by the indirect immunofluorescence test, anti-nDNA, anti-nRNP, anti-Sm and anti-SSB (La) studies were also obtained. From the 22 patients there were 16 girls and 6 boys. The mean age at the onset of the disease was 12.4 years (range 8-15 years). Four patients had the disease before being 10 years old. We mainly observed two clinical subgroups, those with kidney injury, which included 11 patients (50%), and a second group of 8 patients (36%) with prominent hematologic manifestations, 4 with hemolytic anemia and 4 with thrombocytopenic purpura. The patients with kidney disease included 7 with diffuse proliferative glomerulonephritis, one with focal and one with mesangial glomerulonephritis. No biopsies were obtained in two patients. From the group with hematological disturbances, only one patient with hemolytic anemia developed renal involvement during her evolution. Three of the 22 patients presented lymphoproliferative disorders in coexistence with systemic lupus erythematosus. The presence of anti-nDNA antibodies and the decrease of C3, were statistically associated with kidney disease, but not with the hematologic manifestations. Nevertheless 50% of patients with hemolytic anemia and thrombocytopenic purpura have positive anti-nDNA and low C3 determinations. If immunochemical differences between the anti-nDNA antibodies in these groups of patients with systemic lupus erythematosus can establish a distinction in their clinical evolution with or without nephropathy is an interesting question that remains to be determined.