History | Physical Examination | Radiology Studies | Diagnosis| Discussion | Clinical Course |Case Discussion|References

Case Report # 3: Rapidly Destructive Hip Arthropathy

Scott I. Berkenblit, M.D., Ph.D., Tamon Kabata, M.D.,
David S. Hungerford, M.D.

History

A 66-year-old female presented with bilateral groin and lateral thigh pain of 4 years' duration. Her symptoms, initially worse on the right side, had progressed to the point that she became housebound. She had tried multiple nonsteroidal antiinflammatory drugs and was currently taking meloxicam (Mobic) with only partial relief of her pain. Based on a recent MRI scan, she had been presumptively diagnosed with osteonecrosis involving both femoral heads and was referred for treatment.

Past medical history is notable for hypertension, mitral valve prolapse, and depression. She underwent a total abdominal hysterectomy 25 years ago. She had no risk factors for osteonecrosis. Her medications were Norvasc, hydrochlorothiazide, Mobic, Prilosec, and Paxil.

Review of her MRI scan revealed diffuse marrow signal changes which were thought to be within the range of normal, without the characteristic appearance of osteonecrosis. Laboratory studies, including a CBC with differential, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and HLA-B27, were ordered. In addition, a bone scan was obtained.

At her next visit, approximately 6 weeks later, her pain had progressed to such a degree that she now had difficulty dressing herself. Review of her bone scan revealed only mild degenerative changes in both hips, both knees, and her lumbar spine. Her rheumatologist at this point started her on Medrol, which gave her significant relief. The patient returned for further follow up 3 months later, complaining of worsening pain bilaterally. Repeat radiographs at this point showed a dramatic interval change in both hips.

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Physical Examination

At her initial presentation, she was a very pleasant, non-obese woman in no acute distress. She ambulated with a wide-based gait and a slight Trendelenburg component bilaterally. She had full and symmetric range of motion of both hips, with slight groin discomfort at the limit of internal rotation. Stinchfield’s sign was positive on the right, negative on the left. Mild trochanteric tenderness was present on the left only. She had no spinous tenderness and a negative straight leg raise bilaterally. Neurologic examination revealed 5/5 strength and intact sensation throughout both lower extremities, with 2+ knee and ankle jerk reflexes bilaterally.

By her third visit, the patient was barely able to ambulate even with a walker due to her severe pain. Range of motion of both hips was severely limited secondary to pain. Her neurological examination remained unremarkable.

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Radiology Studies

An AP pelvis radiograph obtained 5 months prior to the patient’s first visit (Fig. 1) shows essentially normal hip joints bilaterally. A repeat film obtained at the time of her initial presentation (Fig. 2) shows only mild degenerative changes bilaterally, with some joint space narrowing (more severe on the right) but minimal osteophyte formation. Both femoral heads have a normal contour. In contrast, repeat films obtained 4 months later (Fig. 3) reveal marked collapse and fragmentation of both femoral heads, strikingly symmetric between the two sides.

Figure 1 Figure 2

Figure 3

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Differential Diagnosis

1. Rapidly destructive (rapidly progressive) arthropathy
2. Septic arthritis
3. Neuropathic arthropathy
4. Osteonecrosis
5. Inflammatory arthritis
6. Analgesic arthropathy
7. Crystal-associated arthropathy

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Clinical Course

Based on the radiographic findings and laboratory studies, a diagnosis of rapidly destructive arthropathy was made. The patient elected to undergo staged bilateral total hip replacements.

The left hip was replaced first, using cementless femoral and acetabular components via the Hardinge direct lateral approach. The postoperative course was complicated by a deep venous thrombosis in the opposite limb which was treated with a course of oral warfarin. At her 6-week follow-up appointment, radiographs (Fig. 4) revealed the prosthesis to be well aligned. However, collapse of the right femoral head had progressed even further, leading to superior subluxation of the joint.

Figure 4

A right total hip replacement was performed 3 months after the left. Postoperatively, she sustained a dislocation of her contralateral hip prosthesis; closed reduction was performed and the patient was successfully treated in an abduction brace. She subsequently dislocated her right hip prosthesis and again was successfully treated in an abduction brace. At her most recent visit, she had excellent pain relief and no further episodes of instability. Radiographs (Fig. 5) showed both of her prostheses to be well aligned.

Figure 5

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Discussion

Rapidly destructive arthropathy, also known as rapidly progressive hip disease (or by a variety of similar names) is a rare and incompletely understood disorder characterized by dramatic destruction of a previously normal-appearing hip joint over a relatively short time period. The disease was first described in the European literature by Forestier in 1957^[3] but was not known in the American literature until Postel and Kerboull’s description in 1970.^[7] Although the pathogenesis is poorly understood, Komiya et al ^[4]have found elevated levels of IL-1bß in the joint fluid of affected patients as well as increased secretion of matrix metalloproteinases by the affected synovium (as compared with conventional osteoarthritis), suggesting that excessive production of these bone resorptive factors may mediate the rapid joint destruction.

The two largest published clinical series are those of Rosenberg^[8] and Bock,^[1] containing 27 and 22 patients, respectively. In both series, women outnumbered men, the average of the patients was in their 70s, and more than 80% of cases were unilateral. In one series, 5 patients had involvement of joints other than the hip, suggesting that the disorder may represent a focal manifestation of a systemic disease process. In all cases, the radiographic progression was from a normal-appearing joint to signifcant collapse, typically over a course of less than 12 months. Patients typically presented with incapacitating pain but maintained good range of motion of the hip. Both investigators describe a characteristic "hatchetlike" deformity of the femoral head that develops as it collapses, with small or absent osteophytes. Histologic analysis of retrieved tissue revealed only degenerative changes in the cartilage, with chronic synovitis. Intraoperative cultures were negative, and no crystals were seen.

Rapidly destructive hip disease is essentially a diagnosis of exclusion. It is particularly important to rule out septic arthritis (on the basis of laboratory studies and possibly joint aspiration) and neuropathic arthropathy (which is often painless and presents in the setting of an underlying neurologic disorder), as both of these diagnoses are associated with worse outcomes following total hip arthroplasty.^[1]

Cameron and MacNab^[2] have described a "capital collapse" variant of osteoarthritis in which collapse of the femoral head occurs through a large region of osteonecrosis within the head. A ring of osteophytes is often present at the articular margin of the head and resists collapse, giving the collapsed head the appearance of an acorn.

In inflammatory arthritis, including both rheumatoid arthritis and seronegative arthritides such as ankylosing spondylitis, joint destruction is accompanied by formation of a large pannus. A rapidly destructive hip condition known as analgesic-related arthropathy has been described in association with NSAID use, particularly indomethacin. The pathogenesis may represent either a toxic effect of the drug on the articular cartilage or a joint overloading phenomenon similar to neuropathic arthropathy due to analgesic suppression of pain impulses from the periarticular tissues.^[8]

Several types of crystal-induced arthropathy can also cause rapid hip destruction.^[5] Calcium pyrophosphate dihydrate (CPPD) deposition is typically characterized by prominent osteophyte formation, sclerosis, and subchondral cysts; patients will often have a history of recurrent pseudogout attacks. Apatite deposition can produce a similar syndrome, most commonly in the shoulder ("Milwaukee shoulder"). Detection of crystals in the joint fluid confirms the diagnosis.

Because of the degree of joint deformity and the patient’s level of disability, the typical treatment of rapidly destructive arthropathy is total hip arthroplasty. Several investigators have reported excellent pain relief with this procedure, with outcomes at 5 years comparable to those of total hip arthroplasty performed for conventional osteoarthritis.^[7,9]

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Case Discussion

This patient presented with an unusual case of bilateral rapidly destructive arthropathy of the hips. Radiographically, she progressed from nearly normal-appearing hips to complete collapse over a period of only 3 months. She has had excellent pain relief as a result of her bilateral total hip replacements. Despite her early postoperative episodes of instability, she is now doing extremely well.

Only one other similar case of bilateral rapidly destructive arthropathy treated by total hip arthroplasty has been described in the literature, by Mesko in 1993.^[6] In that case, the patient’s most rapid period of progression occurred over a 4-month period while she was taking indomethacin; it has been postulated that this may have contributed to her clinical course. She underwent simultaneous bilateral total hip replacements and has had excellent pain relief. In both that case and the present case, the strikingly symmetric progression of both hips suggests that some systemic factor, as yet unidentified, may play a role in this unusual disease.

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References

1. Bock GW, et al. Rapidly destructive hip disease: clinical and imaging abnormalities. Radiology 186:461-6 (1993).

2. Cameron HU, MacNab I. Observations on osteoarthritis of the hip joint. Clin Orthop Rel Res 108:31-40 (1975).

3. Forestier F. Coxite rheumatismales subaigues et chronigues. Thesis, Paris (1957).
   
   

4. Komiya S, Inoue A, Sasaguri Y, Minamitami K, Morimatsu M. Rapidly destructive arthropathy of the hip: studies on bone resorptive factors in joint fluid with a theory of pathogenesis. Clin Orthop Rel Res 284:273-82 (1992).

5. Menkes C-J, Decraemere W, Postel M, Forest M. Chondrocalcinosis and rapid destruction of the hip. J Rheumatol. 12:130-133 (1985).

6. Mesko JW. Rapidly progressive femoral head osteolysis. J Arthroplasty. 8:541-7 (1993).

7. Postel M, Kerboul M. Total prosthetic replacement in rapidly destructive arthrosis of the hip joint. Clin Orthop Rel Res 72:138-44 (1970).

8. Rosenberg ZA, et al. Rapid destructive osteoarthritis: clinical, radiographic, and pathologic features. Radiology 182:213-6 (1992).

9. Torre PD, Picuti G, Filippo PD. Rapidly progressive osteoarthritis of the hip. Ital J Orthop Traumatol 13:187 (1987).

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