Systemic lupus erythematosus and scleroderma

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a. Systemic lupus erythematosus. There are no well-documented cases of occult malignancy presenting as SLE. Although antinuclear antibodies can be present in patients with solid neoplasms or leukemias who lack other evidence of a rheumatic syndrome, the significance of this is not understood. LE cells or antinuclear antibodies can be seen in patients with lymphoma or angioimmunoblastic lymphadenopathy with dysproteinemia (AILD). Diagnostic confusion may arise, as lymphadenopathy and splenomegaly are features also common in SLE.

A "lupuslike antibody syndrome," manifested by typical SLE serologic and laboratory abnormalities but lacking clinical criteria for SLE, has been associated with certain tumors and myelodysplastic disorders. For example, hypernephroma has been found in a peripartum woman, but this may have been an incidental finding because a similar lupuslike antibody syndrome can occur during pregnancy. Other tumors include gastric, cervical, and breast carcinomas, gastrointestinal lymphoma, AILD, and testicular seminoma.

In general, a diagnosis of clinical SLE should not prompt a search for occult malignancy. However, lupuslike serology and unexplained Coombs'-positive anemia, thrombocytopenia, or circulating anticoagulants without clinical features of SLE warrant consideration of an occult neoplasm. In addition, in a patient with a known tumor who presents with pleural effusions, pericarditis, or nondeforming polyarthritis, an associated lupuslike illness should be considered.

b. Scleroderma. Rarely, hematologic malignancies, bladder cancer, and breast or stomach carcinoma become apparent shortly after the onset of scleroderma. The scleroderma may improve following treatment of these malignancies. Carcinoid tumors are associated with scleroderma-like changes of the lower extremities. POEMS may also be associated with scleroderma-like skin changes. Scleroderma may be erroneously diagnosed before these disorders are considered.

7. Miscellaneous. Rheumatic syndromes that may be a harbinger of neoplasia include eosinophilic fasciitis and the following disorders. Patients should be followed for development of hematologic disorders (e.g., aplastic anemia and lymphoid malignancies).

I. Reflex sympathetic dystrophy may occur secondary to brain tumor, ovarian carcinoma, or Pancoast lung tumor.

J. Erythromelalgia (burning, red, warm feet) may be associated with myeloproliferative disorders in 10% of cases.

K. Sweet's syndrome, or acute neutrophilic dermatosis, is associated with malignancy in at least 15% of cases, most commonly with acute myelogenous leukemia.

L. Palmar fasciitis and polyarthritis. Originally described in women with ovarian carcinoma, this differs from reflex sympathetic dystrophy. Palmar fasciitis and polyarthritis has also been associated with other malignancies. Plantar fasciitis may be associated with this syndrome. Polychondritis may rarely predate the discovery of a neoplasm.

M. Osteomalacia may develop in patients with a variety of benign or malignant mesenchymal tumors, giant cell tumor, hemangioma, angiosarcoma, hemangiopericytoma, neurilemmoma, and nonossifying fibroma. Epidermal nevus syndrome is also associated with this condition. Patients exhibit hypophosphatemia, normal or slightly decreased serum calcium, and usually normal serum parathyroid hormone concentrations. Abnormalities revert to normal following successful removal of tumor tissue.

Unclassified rheumatic disorders in hospitalized patients may be associated with an underlying occult neoplasia in up to 24% of cases during a 2-year period. The frequency may be higher in men and patients over 50 years old. The malignancy is usually discovered on routine physical examination.

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