Læknablaðið : fylgirit - 01.05.1978, Qupperneq 14

Læknablaðið : fylgirit - 01.05.1978, Qupperneq 14
of vertebral bodies and apophyseal narrowing, chiefly at C-2 and C-3. Asymptomatic sacroiliitis is another early X- ray finding that appears to correlate with hip involvement (shown here), rheumatoid factor, and with onset of disease at 10 years of age or older. Axial articulations other than the cervical and sacroiliac are rarely involved. On the other hand, hips are frequently involved and when progressive • may rapidly produce ankylosis. The joint manifestations are the most prominent component of a polyarticular onset. Nevertheless, rash, lymphadenopathy, splenomegaly, hepatomegaly, pericarditis, and pneumonitis do occur, but are less frequent than in acute febrile onset. Fever, on the other hand, is frequent (38 out of 48 patients) but is invariably low grade, with one or two daily peaks less than 39°C (102°F). Subcutaneous nodules, present in 8 of our chfldren, occurred only in this mode of onset. In Oligoarticular onset, the arthritis is usuaUy insidious with swelling and stiffness and little or no pain. The initial joint most often involved is the knee. The knee proved to be the initial site in 14 of our 32 patients. Next in order of frequency were the hip (5 patients), ankle (4), and elbow (3). In 2 children, a single metatarsophalangeal joint was affected, and in one chUd, a proximal interphalangeal joint of the hand. Painful tendinitis or bursitis of the heel was the initial manifestation in 3 chUdren. When progressive, heel involvement may cause premature formation and closure of the apophysis (noted here on the involved left side). Note also soft tissue radiodensities from achUlobursitis and achillotendinitis as well as erosions and loss of the fine trabecular pattern of the calcaneus. Comparatively few systemic manifestations occur in this form of disease. Low-grade quotidian fever was present in only half of the group. Hash, lymphadenopathy, splenomegaly, and hepatomegaly were only occasionaUy observec. whUe cardiac and pulmonary involvement were notably absent. Clearly, the most important and potentially serious systemic manifestation is chronic iridocyclitis. It occurred in 7 of 32 patients, or 227o, a frequency comparable to that reported by others. Does the laboratory help in early diagnosis? There is no single consistent laboratory abnormality. Elevation of the erythrocyte sedimentation rate and low-grade anemia are frequent, somewhat less so in oligoarticular onset. While nonspecific, each of these abnor- malities are especiaUy helpful in following the • course of individual patients. One of the most consistent abnormaUties in acute febrile onset is a striking neutrophiUc leukocytosis, usually between 20,000 and 30,000 — and sometime higher; it was up to 80,000 in one patient. Leukocytosis is less frequent in polyarticular onset, and counts are generaUy between 12 and 20,000. The white cell count is usually normal in oligoarticular onset. Leukopenia rarely occurs, and its presence should lead one to suspect leukemia or systemic lupus erythematosus. Prolonged and elevated ASO titers, from 400 to 2,500 Todd units, occurred in 29Jo of our patients, and were rather even distributed among each mode of onset. Intramuscular monthly injections of benzathine penicillin over a period of 12 months failed to lower titers in 6 of our patients, seemingly confirming the nonspecific nature of these titers. Nineteen patients had latex titers of 1:160 or greater, which was most often found in poly- articular onset (297o). Rheumatoid factor occurs primarily in children with disease beginning after 12 years of age, and appears to correlate with subcutaneous nodules and a poorer functional outcome. The titers of ANA are lower in JRA than those found in SLE. Their incidence is higher in girls as well as in patients with chronic iridocyclitis, as recently disclosed by Schaller, in 51 of 58 patients, or 887o. ANA titers evolved concomi- tantly with the onset of iridocyclitis in 8 of these patients whose titers were normal previously. ANA may thus prove useful in identifying patients at risk for iridocyclitis. Unlike ANA, LE cells occur only rarely in JRA. The significance of serum immunoglobulins has already been cited. Their determination also serves to detect patients with underlying agammaglobulinemia or selective IgA deficiency. The results of synovial fluid analysis vary considerably, and do not necessarily correlate with the intensity of the underlying arthritis. The average white cell count is 10,000 cells, but the range is wide, from only 150 to 50,000 cells. Complement values are usually but not always depressed. Early X-ray findings include juxta-articular demineralization, radiodensities from soft tissue swelling and effusion, periosteal proliferation, and early closure of epiphyses. Occasionally, accelerated epiphyseal maturation and metaphyseal overgrowth occur. Erosions are only late findings; their early presence should lead one to suspect leukemia or some other form of malignancy. Let us now examine the COURSE OF DISEASE 12
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