Rapidly destructive coxarthrosis (RDC) is a rare syndrome that involves aggressive hip joint destruction within 6–12 months of symptom onset with no single diagnostic laboratory, pathological, or radiographic finding. We report an original case of RDC as an initial presentation of seronegative rheumatoid arthritis (RA) in a 57-year-old Caucasian woman presenting with 6 months of progressive right groin pain and no preceding trauma or chronic steroid use. Over 5 months, she was unable to ambulate and plain films showed complete resorption of the right femoral head and erosion of the acetabulum. There were inflammatory features seen on computed tomography (CT) and magnetic resonance imaging (MRI). She required a right total hip arthroplasty, but arthritis in other joints showed improvement with triple disease modifying antirheumatic drugs (DMARD) therapy and almost complete remission with the addition of adalimumab. We contrast our case of RDC as an initial presentation of RA to 8 RDC case reports of patients with established RA. Furthermore, this case highlights the importance of obtaining serial imaging to evaluate a patient with persistent hip symptoms and rapid functional deterioration.
Rapidly destructive coxarthrosis (RDC) of the hip joint was initially coined in 1970 [
A 57-year-old Caucasian woman was referred to our rheumatology outpatient center from the orthopedics service for assessment of a possible inflammatory etiology for her rapidly destructive arthritis. Table
Major clinical, lab, and imaging findings.
(1) Rapid progressive right hip destructive arthritis | |
(2) Morning stiffness greater than 1 hour | |
(3) Maximum active joint count of 11 | |
(4) Chronic metatarsal phalangeal joints arthritis with erosions, periarticular osteopenia on imaging of hands | |
(5) Right knee arthritis with no imaging evidence of crystal arthropathy | |
(6) Erythrocyte sedimentation rate (ESR) of 56 mm/h and C-reactive protein (CRP) 106.4 mg/L, antinuclear antibody (ANA) was weakly positive at 1:80, with a negative rheumatoid factor (RF), anticitrullinated peptide (anti-CCP), antidouble stranded DNA (anti-dsDNA), and extractable nuclear antigen (ENA) screen | |
(7) Two incidental pulmonary nodules | |
(8) A soft tissue calcified mass in the right sacroiliac (SI) fossa and right gluteal muscles | |
(9) Presence of extracellular calcium pyrophosphate dehydrate (CPPD) crystals in right hip joint, negative synovial biopsy for crystals | |
(10) Biopsy showed chronic inflammation, fibrosis, multinucleated giant cell reaction with dystrophic calcification, and reactive synovial proliferation |
Initial diagnostic work-up was significant for elevated inflammatory markers, weakly positive antinuclear antibody (ANA) and otherwise negative autoimmune markers including both rheumatoid factor (RF) and anticitrullinated peptide (anti-CCP) (Table
Initial plain films of her right hip and pelvis showed femoral head lucencies compatible with subchondral cysts (but no definite fracture) and moderate diffuse articular joint space loss with flattening of the femoral head (Figure
(a) AP radiograph of the right hip. A focal area of subchondral lucency is present involving the superolateral aspect of the right femoral head (arrow). (b) The follow-up radiograph taken 5 months later reveals near complete destruction of the femoral head. (c) CT scan of the right hip in the axial plane shows loss of the femoral head with two bone fragments within the hip joint (arrows).
(a) Axial proton density with fat saturation sequence through the right hip joint (a) shows destruction of the femoral head (arrow) and a complex joint effusion (arrowhead). (b) Axial T1 fat saturated sequence after gadolinium reveals synovial thickening and enhancement (arrow).
Three right hip aspirations were attempted with sufficient sample in only one attempt, which showed bloody fluid, 0.6 nucleated cells (17% neutrophils), and presence of only extracellular but not intracellular calcium pyrophosphate dehydrate (CPPD) crystals. Synovial biopsy did not reveal any crystals. Historically, CPPD crystal deposition disease can cause such acutely destructive disease on imaging and pathology [
The major differential diagnosis of this atypical case of destructive arthritis is outlined in Table
Major differential diagnosis of rapidly destructive coxarthrosis.
(1) Infectious particularly mycobacterial and fungal | |
(2) Crystal arthropathy | |
(3) Avascular necrosis | |
(4) Inflammatory such as rheumatoid arthritis | |
(5) Degenerative | |
(6) Neuropathic | |
(7) Seronegative spondyloarthropathy | |
(8) Multicentric histiocytosis | |
(9) Sarcoidosis | |
(10) Neoplastic |
In order to definitively differentiate between chronic sepsis, malignancy, and a chronic inflammatory process, an open biopsy of the hip was performed by the orthopaedic service, which showed overall morphology with features of chronic inflammation, fibrosis, multinucleated giant cell reaction with dystrophic calcification, and reactive synovial proliferation (Figure
(a) Papillary, hyperplastic, chronically inflamed synovium is shown with abundant fibrin covering the surface and multiple fragments of bone being degraded by histiocytes and multinucleated giant cells. (b) At higher magnification, fibrin is seen on the surface of the synovium with a hyperplastic synovium consistent with chronic inflammation. (c) Fibrin and bone are detailed at 40x magnification showing multiple bone fragments which is typical of a rapidly destructive joint process. (d) Bone is seen being further broken down by multinucleated giant cells.
An inflammatory etiology was most likely given multiple swollen joints, elevated inflammatory markers, constitutional symptoms, evidence of inflammatory features on imaging, and other causes excluded. Hence, a diagnosis of seronegative rheumatoid arthritis (RA) was ultimately made, fulfilling 4/7 of the 1987 RA American College of Rheumatology (ACR) classification criteria [
Rheumatoid arthritis classification criteria.
1987 American College of Rheumatology (ACR)
Morning stiffness > 1 hour (1) | |
Arthritis of 3 or more joint areas (1) | |
Symmetric arthritis (1) | |
Radiographic changes (1) | |
|
|
Total: |
2010 ACR/European League of Rheumatism (EULAR)
Greater than 10 small joints (4) | |
Abnormal CRP and ESR (1) | |
Symptoms > 6 weeks (1) | |
|
|
Total: 6 points |
Table
Inflammatory arthritis is a rare cause of RDC with only 8 case reports of patients with preexisting RA [
Another atypical feature of this patient’s presentation was an associated benign soft tissue mass with enhancement involving the articular region, acetabulum, sacroiliac region, and anterior pelvis (Figure
Total hip arthroplasties have been used as a treatment modality for severe RDC with good response in a retrospective review of total hip arthroplasties performed on patients with RDC [
To conclude, rheumatoid arthritis is a rare cause of rapidly destructive coxarthrosis (RDC). We present the first case report of RDC as the initial presentation of seronegative rheumatoid arthritis in a 57-year-old woman who required right total hip arthroplasty, but whose other active joints had a good response to DMARD and biologic therapy. This case also highlights the importance of obtaining serial imaging to evaluate a patient with persistent hip symptoms and rapid functional deterioration.
The authors declare that there is no conflict of interests regarding the publication of this paper.