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Paget Disease Presenting as a Chronic Symmetrical Polyarthritis

Paget Disease Presenting as a Chronic Symmetrical Polyarthritis

ABSTRACT: This patient's symptoms were attributed to osteoarthritis initially and then to rheumatoid arthritis. She had a history of asymptomatic Paget disease of bone. Physical examination revealed tenderness, swelling, and crepitation in both knees. Chondrocalcinosis was seen on pelvic, knee, and hand x-ray films; the sacroiliac joints were normal. This is typical of calcium pyrophosphate dihydrate (CPPD) crystal deposition disease. Because it was thought that Paget disease of bone played a role in CPPD disease development, treatment was aimed at both conditions. With this regimen, the symptoms resolved. It may be hypothesized that Paget disease played a role in the development of CPPD disease in this patient and management of Paget disease may lead to control of the associated CPPD disease symptoms.


J.S. is a 69-year-old woman who presented with bilateral knee pain that she had had for 5 years. Her symptoms had been attributed to osteoarthritis (OA) initially and then to rheumatoid arthritis (RA). She had a history of asymptomatic Paget disease of bone for which she was not being treated. Chondrocalcinosis was seen on pelvic, knee, and hand x-ray films, and her sacroiliac joints were normal, typical of calcium pyrophosphate dihydrate (CPPD) crystal deposition disease. Paget disease of bone was thought to have played a role in CPPD disease development in this patient; therefore, treatment was aimed at both conditions. In this article, we discuss the hypothesis that in some patients, management of Paget disease may help control symptoms of associated CPPD disease.

Case Report

J.S. described her bilateral knee pain as a severe dull pain that did not worsen at any time during the day. The pain was associated with morning stiffness and swelling and caused impairment in her performance of activities of daily living. She reported good pain relief with NSAIDs and intra-articular corticosteroid knee injections.

The patient's initial diagnosis of OA was changed to RA 1 year ago after her rheumatoid factor was found to be positive (titer, 1:2). At the time of presentation, she was not receiving disease-modifying antirheumatic drugs but occasionally was using ibuprofen for relief of her knee pain.

The diagnosis of Paget disease of bone had been made incidentally 10 years earlier, when J.S. underwent a head CT scan for another reason. A nuclear bone scan showed focal uptake in the skull, T8 and L5 vertebrae, and sacrum. J.S. was not offered treatment for Paget disease at the time of diagnosis because she was asymptomatic.

Our physical examination of J.S. revealed tenderness, swelling, and crepitation in both knees. There was no warmth or erythema of the knees, and range of motion and muscle strength were normal. Rash and hepatosplenomegaly were absent.

Laboratory test results were normal for thyroid function and negative for hepatitis B and C. Also normal were uric acid; parathyroid hormone; antinuclear, anti-dsDNA, and Lyme antibody; and complement levels. Serum alkaline phosphatase levels were elevated, with a value of 132 U/L (normal, 35 to 104 U/L). The bone-specific alkaline phosphatase level was elevated at 23.7 U/L (normal, 0 to 21.3 U/L).

FIGURE 1

X-ray films of both of the patient's hands show chondrocalcinosis of the triangular fibrocartilage.

X-ray films of the patient's pelvis, sacroiliac joints, knees, feet, and hands were obtained. The pelvic x-ray film showed chondrocalcinosis in both hip joints and the pubis symphysis. The sacroiliac joints were normal. In the knee joints, there was severe chondrocalcinosis bilaterally involving the articular fibrocartilage. X-ray films of the patient's hands showed chondrocalcinosis of the triangular fibrocartilage (Figure 1). These radiographic findings were typical of CPPD disease.

FIGURE 2

Shown are calcifications in the left midfoot dorsally that were aspirated using ultrasonographic guidance.

No evidence of RA was seen with ultrasonographic examination of the patient's hands and feet. Granular material was seen in the left midfoot (Figure 2). This material was aspirated; positively birefringent, rhomboid, needle-shaped crystals revealed with examination of the aspirate under the microscope were in keeping with a diagnosis of CPPD disease.

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