Background Among all arthritis in children the systemic juvenile arthritis (SoJA) is one of the most severe by its clinical implications, the most complicated in terms of differentiated diagnostics, selection of therapy and unpredictable. It differs by various options of course and outcomes, from extremely favorable to extremely aggravated up to death, and in this respect such disease is of unclear nature.
Objectives Establish the course and outcomes in adult patients suffering from SoJA for a long time.
Methods The study included 19 adult patients with SoJA diseased in childhood. There were 7 males, 12 females. The average age of pts was 28.8±9.1 years, and varied from 20 to 60 years. Onset age was 6.7±4.4 (1-16 years). The duration of the disease was from 11 to 53 years (av. 24.7±11.4 years). Onset systemic implications included fever (18), rash (8), lymphadenopathy (7), gepatosplenomegaly (8), pericarditis (1). 9 of them (47.4%) had onset oligoarthritis, 7 (36.8%) - polyarthritis, and 3 patients had arthralgia only.
Results In the course of the disease the frequency of systemic implications was reduced, and at the moment of the research they occurred only in 3 pts, primarily as fever episodes and lymphadenopathy. 2 pts had uveitis in the past medical history. During the evolution 84.2% of the pts developed polyarthritis. At the moment of examination almost half of the pts (47.4%) had no active arthritis, and half of them (21.1%) had no changes in joints at all. Half of the patients had active arthritis (52.6%), generally oligoarthritis. Despite the fact that upon examination the general inflammatory disease activity was maintained in the majority of patients (88.9%), its intensity was low or moderate (1-2 degree). Average HAQ was 1.2 points, HAQ=0-1.0 in 66.6% pts, HAQ>2 - in 6.7%. Over a half had growth retardation or disproportionate body structure. Average height of patients was 167.1±14.8 (140-193 cm). 28.6% of them had no radiological changes, erosions were revealed in 35.7%, ankylosis – in 21.4% of pts, 4 stage sacroiliitis – in 2. 42.9% of pts had aseptic necrosis (hip and upper arm bones). Amyloidosis was diagnosed in 2 pts.60% of patients continued to take NSAID, 46.7% - systemic corticosteroids, 13.3% - periodical intra-articular injections, 46.7% - DMARDs, primarily methotrexate. At the moment of examination the stabilization (almost inactive disease) of the process occurred in 5 (26.3%), complete remission – in 1 (5.3%), nobody had drug remission, recurrence occurred in 13 pts (68.4%). In 11 (57.9%) the remission were registered during disease (generally without treatment) with duration from 5 to 23 years.
Conclusions Continuous duration of systemic JA tends to outcome in polyarthritis. Despite the gravity of the disease in onset, maintained inflammatory activity and recrudescence of arthritis in majority of patients, with the course of time the intensity of systemic and joint implications is reduced, most patients are functionally safe. The disease has long-term remissions.However one third of patients form erosion process, and almost a half have aseptic necrosis that is the ground for active therapy continuation.
Disclosure of Interest None Declared
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