Orthopedics - Oleksa A.P. 2006

Joint Diseases
Arthritis of Unknown Etiology
Juvenile Rheumatoid Arthritis

This form of the rheumatoid process in children has certain distinct features. The disease can present as an articular form (ankylosing polyarthritis) or an articular-visceral form, commonly known as Still's disease (Still-Chauffard disease).

The articular form of rheumatoid Arthritis most frequently begins in early childhood. According to Manheimer R.H., Green R.C., and Kroll F. (1959), who evaluated children in New York, the disease onset occurs before the age of five in 54% of cases.

Typically, the rheumatoid process begins subacutely in any joint, progressing subtly and eventually involving other joints. However, rheumatoid arthritis may remain restricted to a single joint—a scenario reported by Bywaters E. and Ensell (1963) in 50% of cases. The knee joints and the JOINTS OF THE fingers are most commonly affected, and occasionally the spine, leading to subsequent ankylosis.

Despite disease progression accompanied by typical deformities, subluxations, and ankylosis, juvenile rheumatoid arthritis does not present with fever, anemia, or weight loss. Subcutaneous rheumatoid nodules are rare, though the disease is sometimes complicated by iritis, iridocyclitis, and similar conditions.

Blood tests reveal an elevated ESR and mild leukocytosis. Notably, rheumatoid factor is absent from the blood, and the Waaler-Rose test alongside other serological assays remains negative.

Radiographic examination demonstrates classic joint changes depending on the stage of the rheumatoid process, including Osteoporosis, destruction, and fibrous or bony ankylosis. Bardfeld R. and Streda A. (1963) note that pronounced radiological joint changes become visible within 3 to 6 years of disease onset.

The clinical course of the disease in children does not differ from that in adults. With a favorable course and skilled medical care, patients may occasionally achieve full recovery; however, in the vast majority of cases—despite periods of remission—the rheumatoid process progresses and leads to disability resulting from deformities, contractures, and ankylosis.

The articular-visceral form of Rheumatoid Polyarthritis occurs in both adults and children. The polyarthritis is accompanied by visceral involvement, such as cardiovascular or renal manifestations (articular-renal form), as well as pulmonary or neurological complications.

Occasionally, a particularly severe clinical course of articular-visceral polyarthritis is observed, which Nesterov designated as the septic form, though it was originally described by Coste F. and Delbarre F. in 1953 as polyarthritis grave. This form manifests acutely during adolescence, progresses rapidly, and is accompanied by fever, a septic state, visceral involvement, and vasculitis, proving highly refractory to Treatment.

In children, the articular-visceral form of rheumatoid polyarthritis was first described in 1897 by Still G.

In the vast majority of children, Still's disease begins between the ages of 2 and 5, although cases occurring During the first year of life have been documented. The hallmark features of this condition include joint involvement accompanied by polylymphadenopathy, Splenomegaly, fever, and anemia.

As a rule, the disease begins acutely, characterized by a significant spike in body Temperature, painful Swelling primarily of the knee and wrist joints, and severe pain. Later, the joints of the hands and occasionally the spine become involved.

A characteristic feature of the joint pathology is The Development of synovitis followed by proliferative changes, particularly within the periarticular Tissues, yet without significant joint deformation or ankylosis.

Fever is almost invariably accompanied by profuse sweating and a rash (scarlatiniform, petechial, maculopapular, etc.) on the trunk, face, and extremities, which vanishes without a trace once body temperature normalizes. This is followed by enlargement of the Lymph Nodes—most frequently in the upper half of the trunk—and splenomegaly, observed in 75% of children (Copeman W., 1955). Additionally, pericarditis may occur, which, according to Copeman, can be the initial sign of the disease and is often detected radiographically due to its potential asymptomatic course. Iritis and polyneuritis are also observed.

Blood analysis reveals an elevated ESR and neutrophilic leukocytosis, while rheumatoid tests (Waaler-Rose, latex fixation test) yield negative results.

Unlike the adult rheumatoid process, radiographs typically show no significant destructive changes or joint ankylosis. According to Ilyinsky P.I. (1948), Korolyova N.I., and Yevtushenko T.N. (1958), a favorable disease course may allow for complete restoration of joint function. Nevertheless, persistent fever, joint pain, and progressive anemia severely exhaust the child, leading to cachexia, which can occasionally prove fatal due to intercurrent infections or amyloidosis.

The comprehensive management of juvenile rheumatoid arthritis is identical to that of rheumatoid polyarthritis in adults.

Wissler and Fanconi described yet another form of nonspecific joint disease in children that does not fit the criteria of either rheumatoid or rheumatic disorders. They termed this syndrome "allergic pseudosepsis." The condition manifests as mild arthritis accompanied by hectic fever, recurrent erythema, moderate adenopathy and splenomegaly, leukocytosis, and anemia.

Regarding the Classification of this disease as a distinct nosological entity, opinions vary. For instance, Forestier notes that in some cases the disease eventually transitions into rheumatoid arthritis, Grenet considers it an atypical form of rheumatism, and Bernheim views it as infectious rheumatism (Astapenko M.G., Pykhlak E.G., 1966).