History | Physical
Examination | Radiology Studies
Discussion | Clinical
Case Report # 3: Rapidly Destructive Hip Arthropathy
I. Berkenblit, M.D., Ph.D., Tamon
David S. Hungerford, M.D.
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.
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.
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.
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.
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. Stinchfields 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.
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.
AP pelvis radiograph obtained 5 months prior to the patients
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.
destructive (rapidly progressive) arthropathy
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.
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.
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
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 but was not known in the American
literature until Postel and Kerboulls description in 1970.
Although the pathogenesis is poorly understood, Komiya et al
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.
two largest published clinical series are those of Rosenberg
and Bock, 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.
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.
and MacNab 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.
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.
types of crystal-induced arthropathy can also cause rapid hip
destruction. 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.
of the degree of joint deformity and the patients 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]
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.
one other similar case of bilateral rapidly destructive arthropathy
treated by total hip arthroplasty has been described in the literature,
by Mesko in 1993. In that case, the patients
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.
GW, et al. Rapidly destructive hip disease: clinical
and imaging abnormalities. Radiology 186:461-6 (1993).
HU, MacNab I. Observations on osteoarthritis of the hip joint.
Clin Orthop Rel Res 108:31-40 (1975).
F. Coxite rheumatismales subaigues et chronigues. Thesis, Paris
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).
C-J, Decraemere W, Postel M, Forest M. Chondrocalcinosis and
rapid destruction of the hip. J Rheumatol. 12:130-133
JW. Rapidly progressive femoral head osteolysis. J Arthroplasty.
M, Kerboul M. Total prosthetic replacement in rapidly destructive
arthrosis of the hip joint. Clin Orthop Rel Res 72:138-44
ZA, et al. Rapid destructive osteoarthritis: clinical,
radiographic, and pathologic features. Radiology 182:213-6
PD, Picuti G, Filippo PD. Rapidly progressive osteoarthritis
of the hip. Ital J Orthop Traumatol 13:187 (1987).
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