REHABILITATION OF THE CHILD WITH RHEUMATIC DISEASE

Rehabilitation of the child with rheumatic disease requires an interdisciplinary approach that includes the child and family. Although most often the physiatrist is not the treating physician in rheumatologic disease, they can play a key role in the comprehensive management of these conditions, along with other members of the rehabilitation team, to maintain or restore age-appropriate function and development, prevent deformity and contractures, and help manage pain.

JUVENILE IDIOPATHIC ARTHRITIS

Juvenile idiopathic arthritis (JIA), formerly known as juvenile rheumatic arthritis (88), is the most common rheumatic disease of childhood, affecting approximately 16 to 150 in 100,000 (89). In 1995, the International League Against Rheumatism (ILAR), together with the World Health Organization, reclassified chronic childhood arthritis (90); the second revision occurred in 2001 (91). Chronic childhood arthritis is now known as JIA and is divided into the following seven subtypes: systemic arthritis, oligoarthritis, rheumatic factor (RF)-negative polyarthritis, RF-positive arthritis, psoriatic arthritis, enthesitis-related arthritis, and undifferentiated arthritis.

JIA occurs in children before the age of 16 years, persists at least 6 weeks, and has had other known conditions excluded; etiology is unknown, but seems to include genetic and environmental components (89,92). In 2011, the American College of Rheumatology (ACR) published recommendations for the treatment of JIA, organized into five treatment groups; namely, arthritis of four or fewer joints, arthritis of five or more joints, active sacroiliac arthritis, systemic arthritis with active systemic features (and without active arthritis), and systemic arthritis with active arthritis (and without active systemic features) (93). In October 2013, these recommendations were further reviewed and updated, with emphasis on systemic JIA (94). Both recommendations will be discussed further in the treatment section.

Early arthritis may be manifested by swelling, warmth, and joint stiffness, typically worse at the beginning of the day then improving with activity. Symptoms usually fluctuate; uncontrolled inflammation leads to joint damage. Younger children rarely complain of joint pain, but may instead become irritable, stop walking or using an extremity, or regress in their behavior (95). Other symptoms include decreased appetite, malaise, inactivity, morning stiffness, nighttime joint pains, and failure to thrive (95). Enuresis may occur in a recently toilet-trained child (96). Later disease presents with reduced ROM, contractures, overgrowth or undergrowth of affected limbs, and resultant disability.

A characteristic feature of chronic arthritis in children is the effect the disease has on bone and joint development (97,98). Local growth disturbances at inflammation sites can lead to overgrowth (secondary to possible inflammatory-mediated increased vascularization and growth factor release) or undergrowth (secondary to growth center damage or premature fusion of epiphyseal plates). Irregular traction on growing structures secondary to muscle spasms and periarticular fibrosis can also cause aberrant growth (97,98). Micrognathia, leg-length inequalities, and developmental hip anomalies are all possible results from these processes. Steroids can also contribute to severe growth effects, as well as osteoporosis (99).

The differential diagnosis of JIA is large (Table 10.1 provides a full differential diagnosis). The assumption that JIA will universally resolve by adulthood is incorrect (100). Radiological joint damage occurs in children with systemic arthritis and polyarticular arthritis within 2 years, and in oligoarthritis within 5 years (101,102). Despite long-term persistence of disease activity in JIA, much improvement in functional outcomes has been made in the past decade (103,104). Indicators of poor outcome include greater severity or extension of arthritis at onset, symmetrical disease, early wrist or hip involvement, presence of RF, persistent active disease, and early radiographic changes (105). In the 2011 ACR recommendations, features of poor prognosis are further specified by treatment group. Features of poor prognosis for three

TABLE 10.1 DIFFERENTIAL DIAGNOSIS OF JUVENILE IDIOPATHIC ARTHRITIS

DIFFERENTIAL DIAGNOSIS OF JUVENILE IDIOPATHIC ARTHRITIS

Abbreviation: CREST, Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia.

of the five categories, arthritis of four or fewer joints, arthritis in five or more joints, and systemic arthritis with active arthritis (and without systemic features), include arthritis of the hip or cervical spine. Four of the five treatment groups (all except for systemic arthritis with active systemic features [and without active arthritis]) show poor prognosis with radiographic damage (erosions or joint space narrowing by radiograph) (93).

 
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