Summary and the Case
Background: A 58-year-old woman presented with arthritis of the small joints of her hands and rapidly progressive joint contractures. She was wheelchair bound within 2 months of the onset of her symptoms. Physical examination revealed synovitis of the small joints of her hands and palmar fasciitis. The patient had been diagnosed with pancreatic carcinoma approximately 1 year before the presentation of her rheumatic symptoms, and had undergone radical pancreatico-duodenectomy.
Investigations: Physical examination; routine laboratory work, including full blood count and measurement of erythrocyte sedimentation rate and C-reactive protein; serological tests for rheumatoid factor, antinuclear antibodies and extractable nuclear antibodies; measurement of serum tumor markers; radiological investigations, including X-rays of her hands and feet, whole-body CT-scans and radioisotope bone scan.
Diagnosis: The patient's rheumatic presentation was diagnosed as a paraneoplastic syndrome associated with pancreatic carcinoma.
Management: The patient's condition was managed with corticosteroids and methotrexate. No residual tumor or evidence of metastatic disease have been detected in the 1.5 years since the initial presentation of her rheumatic symptoms.
A 58-year-old woman presented to the Accident and Emergency department with rapidly progressive joint flexures. A rheumatological consultation was sought. The patient's first clinical manifestation was the development of purple erythematous papules over the palmar surface of her hands. She reported a 2-month history of increasing pain and swelling of the small joints of her hands and wrists, and painful and stiff elbows and knees. The patient also experienced profound early morning stiffness, but her clinical presentation was atypical, with left-side joints markedly more affected than those on her right side. Rapid progression of flexion contractures involving her hands, wrists, elbows and knee joints resulted in the patient being wheelchair bound upon presentation to the Accident and Emergency department, less than 2 months after the onset of her symptoms. The patient's baseline level of physical activity, before presentation of her rheumatic symptoms, involved independent mobilization at home and independence in her activities associated with daily living. Physical examination revealed bilateral erythema and palpable thickening of palmar fascia, synovitis of the metacarpal phalangeal (MCP) and proximal interphalangeal (PIP) joints, and flexion contractures of all fingers (~60° at MCP joints and ~30° at PIP joints), wrists (~20° bilaterally), elbows (~45° bilaterally) and knee (~45° bilaterally) joints.
Approximately 1 year before the presentation of her rheumatic symptoms, the patient presented with weight loss and jaundice, and was diagnosed with lymph-node-positive pancreatic carcinoma. She was treated with radical pancreatico-duodenectomy. The patient subsequently underwent gastrojejunostomy for anastomotic stricture and had a protracted postoperative convalescence, but made a complete recovery. She did not develop diabetes postsurgery, and never required insulin or other hypoglycemia medications. Before the onset of pancreatic carcinoma, the patient had been healthy and had no comorbidities.
At the initial rheumatological consultation, the results of laboratory investigations, including full blood count, erythrocyte sedimentation rate, serum C-reactive protein level and serological tests for rheumatoid factor, antinuclear antibodies and extractable nuclear antibodies, were unremarkable ( Table 1 ). Anti-cyclic citrullinated peptide antibodies were not measured. Radioisotope bone scan and radiological examination of hands, wrists and knees were normal. There was no evidence of residual tumor or metastasis detected on CT of the abdomen, pelvis, thorax and brain, and the patient's tumor marker levels were normal.
A presumptive diagnosis of rheumatoid arthritis with atypical features was initially made, and the patient was administered intravenous steroids and methotrexate; however, the atypical clinical picture against the background of malignancy, the rapid progression of symptoms and the subsequent findings from normal investigations led us to revisit the diagnosis. The patient's inflammatory arthritis responded to low-dose maintenance steroids and methotrexate but, unfortunately, she had residual disabling flexion contractures; these contractures were treated with extensive physiotherapy. The patient was diagnosed as having paraneoplastic palmar fasciitis and polyarthritis syndrome. Although many case reports claim a gradual resolution of rheumatic symptoms with successful management of the associated neoplasm, we could not detect any residual tumor or evidence of metastatic disease in our patient.
Nat Clin Pract Rheumatol. 2008;4(5):274-277. © 2008
Nature Publishing Group
Cite this: A Paraneoplastic Case of Palmar Fasciitis and Polyarthritis Syndrome - Medscape - May 01, 2008.