Further Evaluation of Unspecified Rheumatic Syndrome

Stephen A. Paget, MD, FACP, FACR

Disclosures

July 10, 2002

Question

Please recommend further diagnostic tests/evaluations that should be considered for a patient who has had the following rheumatic syndrome for 2 years:

Palmer and plantar fasciitis with asymmetric finger arthritis and noduli in the subcutis and on tendons of fingers and feet, vision disturbances with retinal autoantibody p35K, and a strongly reactive bone marrow with infiltration of small polyclonal B and T cells. There is also sensory neuropathy with intense skin pain and no neuron autoantibodies. All the changes could be paraneoplastic, but extensive investigation for the past 2 years has not shown any evidence of malignancy. All other rheumatic/hematologic tests are negative.

Response from Stephen A. Paget, MD, FACP, FACR

This patient clearly has a systemic inflammatory disorder manifested by joint and soft tissue inflammation with nodularity, an autoimmune disorder of the eyes, a neuropathy, and a bone marrow demonstrating an activated, polyclonal inflammatory state. Because patients do not read medical textbooks, they often present with manifestations that do not fit neatly into the clinical picture described in the chapters on joint, autoimmune, and inflammatory disorders. This patient provides a classic example.

Although the presence of nodules, arthritis, and neuropathy are consistent with rheumatoid arthritis, the asymmetry of the chronic finger arthritis, the prominence of fasciitis, and the presence of an autoantibody found in patients with uveitis would make the astute clinician consider a diagnosis of a seronegative spondyloarthropathy. Negative serologic tests such as rheumatoid factor and antinuclear antibody would not support a diagnosis of rheumatoid arthritis or an overlap autoimmune disorder. Sarcoidosis should be considered in the differential diagnosis, given the nodular lesions, joint disease, neuropathy, and possible infiltrative skin and subcutis disease and uveitis. Despite the 2-year history of symptoms, subacute or chronic infections such as tuberculosis must always be considered, despite the bone marrow being negative for granuloma.

Facts that are not available but which could add to the clinical equation would be the age and sex of the patient and the presence or absence of Raynaud's phenomenon, psoriasiform or vasculitic skin rash, and dry eyes and/or dry mouth, pulmonary problems, bowel inflammation, or sacroiliac joint inflammation. I assume that the complete blood cell count, erythrocyte sedimentation rate, C-reactive protein, and immunoelectrophoresis were either normal or noncontributory. An HLA-B27 test is rarely needed in the setting of a characteristic clinical presentation of a seronegative spondyloarthropathy, but it could be helpful in this type of very atypical case, if not already performed. An angiotensin-converting enzyme level would also be a reasonable addition.

Finally, a biopsy of 1 of the nodular lesions and skin would be very helpful. This could rule out some of the more atypical granulomatous, infiltrative, or neoplastic disorders that could stimulate this type of systemic illness. These disorders would include infections such as tuberculosis, fungus, or leprosy, sarcoidosis, or a cancer. The finding of a typical rheumatoid nodule would be helpful. It is likely that this biopsy would have a significant impact on helping to define the diagnosis and the subsequent therapeutic modalities.

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