with salicylates, indomethacin, corticosteroids, gold salts, and physical therapy. Continuous skin traction, with periods off for an active exercise program, was.
1570
BRIEF REPORT
REMODELING OF THE HIP JOINT IN JUVENILE RHEUMATOID ARTHRITIS OSVALDO GARCIA-MORTEO, J U A N CARLOS BABINI, JOSE A. MALDONADO-COCCO, SUSANA GAGLIARDI, and JORGE YABKOWSKI
Although joint destruction has generally been considered to be irreversible, recent investigators have shown that some repair of articular surfaces by fibrocartilage can occur in different situations (1,2). In 1977, Bernstein et a1 reported 6 patients with longstanding juvenile rheumatoid arthritis (JRA) with radiographic evidence of severe hip joint involvement, who afterward developed significant space widening and remodeling of hip joint articular surfaces (3). The purpose of this report is to describe definite radiographic evidence of partial hip restoration in 3 children with rheumatoid arthritis. The finding of joint modeling was also investigated in another 47 JRA patients with clinical and radiologic involvement of the hip joint. Materials and Methods. All of the patients included in this study met the American Rheumatism Association criteria for the diagnosis of JRA (4,5). The 3 patients with hip joint remodeling had several radiographs performed at various intervals as clinically .~
From the Section of Rheumatology, Department of Medicine, Instituto Nacional de Rehabilitacibn. Universidad de Buenos Aires, Buenos Aires, Argentina. Presented in preliminary form at the I1 Congreso Intemacional de Reumatologia del Cono Sur, Santiago, Chile, September 1980.
Osvaldo Garcia-Morteo, MD: Associate Professor of Medicine, Universidad de Buenos Aires and Head, Rheumatology Section; Juan C. Babini, MD: Former Fellow in Rheumatology: Jose A. Maldonado-Cocco, MD: Staff Physician; Susana Gagliardi, MD: Staff Physician; Jorge Yabkowski, MD: Fellow in Rheumatology, Section of Rheumatology, Instituto Nacional de Rehabilitaci611, Buenos Aires. Address reprint requests to Dr. Osvaldo Garcia-Morteo, Secci6n Reumatologia, Instituto Nacional de Rehabilitacih, Echeverria 955, 1428 Buenos Aires, Argentina. Submitted for publication January 23, 1981; accepted in revised form April 9, 1981. Arthritis and Rheumatism, Vol. 24, No. 12 (December 1981)
indicated. From our population of patients with JRA with hip involvement (pain and/or limitation of motion and radiologic alterations of the hip joint), 47 were selected because at least two radiographs of their hips had been taken, with intervals no shorter than 24 months. The criteria definining radiologic hip joint remodeling included the combined findings of widened joint space, filled in subchondral cysts, and articular margins that had become smoother. In all cases, radiographs taken were comparable in technique and projection. Case Reports Patient 1 . This white male (FB) had a polyarticular onset JRA at 7 years of age. He was first seen at that age, and since then his disease has been persistently polyarticular. During followup, he occasionally had hectic fever, splenomegal y , and adenopathies. Rheumatoid factors have been persistently negative. At 10 years of age, he developed severe bilateral hip pain with limitation of motion and progressive impairment of ambulation. Both hips were severely limited in motion, and flexion contraction measured 30". Hip radiographs showed marked narrowing of joint space, erosions, and subchondral sclerosis of both hips. Lateral subluxation of the left femoral head was also present (Figures 1A and B). He was treated with salicylates, indomethacin, corticosteroids, gold salts, and physical therapy. Continuous skin traction, with periods off for an active exercise program, was. initiated. Later he could walk with crutches and then with a cane. At the age of 1 5 , 5 years after the onset of hip involvement and coincidentally with clinical improvement of his arthritis, progressive relief of hip pain and improvement of range of motion was
BRIEF REPORT
1571
A
B
C
Figure 1. Patient I: A, Age 12. Hips show narrowing of the joint space and subcortical erosions bilaterally. Note lateral subluxation of the left femoral head. B. Age 14. Hips show subchondral sclerosis and partial filling in of erosions. C. Age 15. Both hips show better defined joint spaces and articular surfaces that had become smoother.
observed. Radiographs taken at that time revealed definite bilateral joint space widening, smoother articular surfaces, and healing of erosions (Figure IC). Patient 2. This white male (OG) had an acute onset of hectic fever and polyarthritis at 4 years of age. His disease was polyarticular with episodic systemic manifestations (skin rash, spienomegaly, adenopathies). Rheumatoid factors were persistently negative. At 10 years of age, the patient developed severe pain and motion limitation in both hips and impaired ambulation. Roentgenograms showed marked narrowing of the joint space and erosive changes on the right hip. The left hip had moderate space narrowing. He was treated with aspirin, indomethacin, low doses of prednisone, and physical therapy. Bed rest and continued skin traction interrupted for active exercises and hydrotherapy were initiated, followed by a progressive
program of aided ambulation. The patient experienced a transient improvement followed by further deterioration, and followup x-ray films showed progressive radiologic damage in both hips (Figure 2A). Five years after the onset of hip involvement, at the age of 15 years, he experienced improvement with amelioration of the pain and increased motion in both hips. There was also radiologic improvement, with smoother articular surfaces, widening of the joint space, and filling in of erosions (Figure 2B and C). Patient 3 . This white male (FP) had a polyarticular JRA which began at 3 years of age and followed a chronic polyarticular course. Tests for rheumatoid factors were positive on several occasions. At 8 years of age, he developed progressive hip involvement and severe restriction of motion. X-ray films, taken 2 years after the onset of the hip involvement when the patient
BRIEF REPORT
I572
A
B
C
Figure 2. Patient 2: A , Age 13. Hips show narrowing of the joint space and subcortical erosions bilaterally. B. Age IS. Widening of the joint space and smoother articular surfaces. Note healing of erosions, mainly at the left femoral head. C, Age 17. Both hips show smoother articular margins and widening of the joint space. Subcortical erosions have healed.
was 10 years old, showed major erosive changes and flattening of the femoral head (Figure 3A). He received aspirin, indomethacin, gold salts, and physical therapy on a regular basis. Other treatment included bed rest, continued skin traction, active exercises, and hydrotherapy. Later on, gradual ambulation was prescribed when his condition had improved moderately. When FP was 12 years old, his disease went into remission with simultaneous clinical and radiologic improvement of both hips. X-ray films at that age showed the articular surfaces were smoother and erosions were healing (Figure 3B). Two years later, there was evident remodeling of both hips (Figure 3C). Discussion. Patterns of cartilage destruction and their causes have been described in adult rheumatoid arthritis. There is, as yet, no information available on
the mechanisms of cartilage destruction in JRA (6). Although joint destruction has been generally considered an irreversible phenomenon, repair of articular surface by fibrocartilage has been seen on pathologic specimens (1,7). Our patients had courses analogous to some children with chondrolysis, either idiopathic (8) or complicating slipped capital femoral epiphysis (9). Recovery of the joint space observed in roentgenograms has also been described in this entity (10,ll). Chondrolysis of the hip in adolescence is characterized by pain and limping, with progressive loss of articular cartilage space and stiffness of the hip. Although occasionally some cartilage space is reconstituted (10,l I), the usual course is marked limitation of motion or progressive degenerative changes of the involved hip (12).
BRIEF REPORT
1573
A
B
C
Figure 3. Patient 3: Comparison of progressive improvement in both hips. A. Ape 10. Hips show flattening and erosions of thc femoral head. B. Age 12. Widening of the joint space is more evident on the right side. C. Age 14. There is a definite improvement in joint space. healing of erosions, and smoother surfaces. mainly on the right side.
The cause of chondrolysis remains obscure. Its mechanism may not be specific for necrosis of articular cartilage after slipping of the epiphysis, and it may be found in other joint conditions (9). We obtained no pathologic documentation of the "regeneration" of joint space in our JRA patients. Remodeling oljoints of the hands and feet in JRA has been already mentioned by Ladksonen (13). During the last year we have seen a 6-year-old girl with similar restoration pattern in the shoulder joint. In 1977, Bernstein et al first reported the Occurrence in JRA of restoration of major weight-bearing joints such as the hips (3). However, in at least one of their reported cases, this presumptive improvement was not adequately demonstrated, since joint space widening
was associated with increased destruction of the femoral head. Our 3 patients met our radiologic criteria for joint remodeling, which include the combined findings of widened joint space, filled in subchondral cysts, and smoother articular margins. In our study, patients with simultaneous occurrence of increased destruction of the femoral head were excluded. The 3 patients whom we studied were boys with persistent polyarthritis; in 2, it was associated with systemic manifestations such as hectic fever, skin rash, splenomegaly, and adenopathies. Symptoms and signs of persistent hip joint involvement occurred 3 to 6 years after JRA onset. All of them had bilateral pain and limitation of motion with severe impairment in walking. Drug treatment and
1574
physical therapy programs did not differ in these children from those prescribed for 47 other JRA patients with hip involvement who did not develop joint restoration. In these 47 patients, the interval between the first and the last hip x-ray examination was no shorter than 24 months, with a maximum followup of 204 months and a median of 81 months. W e prescribed active exercises, muscle strengthening, hydrotherapy, aided ambulation, and sometimes continuous skin traction with three daily periods off for exercise in all our JRA patients with severe hip joint involvement. Instead of increasing ambulation as mentioned by Bernstein (3), our patients were instructed to avoid weight bearing and encouraged to perform the above-mentioned program. The use of wheelchair is avoided, because this will aggravate flexion contractures. Radiographic findings of hip remodeling, together with marked improvement in hip function, were observed 4 to 5 years after the onset of hip involvement. Hip restoration was associated with clinical improvement of the rheumatoid disease. One of the patients has experienced remission, while the other 2 have had marked improvement of the polyarthritis. Radiologic evidence of hip remodeling was observed in all six hip joints affected. The sequence of radiographic improvement in patient 1 was less dramatic than in patients 2 and 3 . However, this clinical and radiologic improvement was not sustained; on further followup, new deterioration occurred in 2 patients. In patient 1, after 15 months of sustained hip improvement, new damage was seen in the left hip. In patient 3, hip improvement lasted for 4 years; coincidentally with a flare of his polyarthritis, he again developed severe bilateral hip pain and restriction of motion. Xray films at this time showed the narrowing of the joint space and erosions. One year later, when he was 19 years old, this patient underwent bilateral total hip joint replacement. Patient 2, at present 19 years old, has had painless, functional hips for the last 4 years, with moderately active polyarthritis. Remodeling of the hip joint in JKA is an interesting phenomenon. Though we d o not yet understand its mechanism, its occurrence seems to correlate with
BRIEF REPORT
improvement of the underlying disease. Hip remodeling may not be sustained, and further deterioration can occur.
REFERENCES 1. Harrison MH, Menon MP: Legg-Calve-Perthes disease:
the value of roentgenographic measurement in clinical practice with special reference to the broomstick plaster method. J Bone Joint Surg 48A:1301-1318, 1966 2. Robins RHC, Piggot J: McMurray osteotomy: with a note on the “regeneration” of articular cartilage. J Bone Joint Surg 42B:480-488, 1960 Bernstein B, Forrester D, Singsen B, King KK, Kornreich H, Hanson V: Hip joint restoration in juvenile rheumatoid arthritis. Arthritis Rheum 20: 1099-1 104, 1977 Brewer EJ, Bass JC, Cassidy JT, Duran BS, Fink CW, Jacobs JC, Markowitz M, Reynolds WE, Schaller J, Stillman JS, Wallace SL: Criteria for the classification of juvenile rheumatoid arthritis. Bull Rheum Dis 23:712719, 1972-1973 Brewer EJ, Bass J, Baum J, Cassidy JT, Fink C, Jacobs J, Hanson V , Levinson JE, Schaller J , Stillman JS: Current proposed revision of JRA criteria. Arthritis Rheum (Suppl) 20:195-199, 1977 6. Cruess RI,; Mitchell NS: Mechanisms of cartilage destruction. Arthritis Rheum (suppl) 20558-561, 1977 7. Arden GP, Ansell BM: Surgical Management of Juvenile Chronic Polyarthritis. London, Academic Press, 1978, pp 102-10s 8. Duncan JW, Nasca R, Schrantz J : Idiopathic chondrolysis of the hip. J Bone Joint Surg 61A:1024-1028, 1979 9. Maurer KC, Larsen IJ: Acute necrosis of cartilage in slipped capital femoral epiphysis. J Bone Joint Surg 52A:30-50, 1970 10. Lowe HG: Necrosis of articular cartilage after slipping of the capital femoral epiphysis: report of six cases with recovery. J Bone Joint Surg 52B: 108-1 18, 1970 11. Hartman JT, Gates DJ: Recovery from articular cartilage necrosis following slipped capital femoral epiphysis. Orthop Rev 1:33-37, 1972 12. Wilson PD, Jacobs B, Schecter L: Slipped capital femoral epiphysis: an end-result study. J Bone Joint Surg 47A:1128-1145, 1965 13. Laaksonen A: A prognostic study of juvenile rheumatoid arthritis. Acta Paediatr Scand (suppl) 166:98, 1966
