Abstract Introduction Remitting seronegative symmetrical synovitis with pitting edema (RS(3)PE) is an uncommon, steroid-responsive syndrome of older adults, often mistaken for benign rheumatologic disease. However, emerging evidence shows up to one-third of cases signal an occult malignancy. Its striking presentation—symmetric polyarthritis, dramatic hand edema, and seronegativity—makes RS(3)PE a clinical red flag. Prompt recognition is crucial, which may serve as the first clue to an underlying cancer. Case Presentation 57 year old African American male with an extensive smoking history, alcohol abuse, CHF, HLD, and HTN, presented with severe swelling of the lips, diffuse joint pains accompanied by edema of hands up to the forearms, excessive lacrimation, conjunctival injection, and peau d’orange appearance the skin, ongoing for one month. Given his age 50, smoking history and presenting symptoms of polyarthralgias, unintentional weight loss, synovitis of the small joints, marked pitting edema of the upper extremities, conjunctivitis, a clinical diagnosis of RS(3)PE was reached. A diagnostic workup was initiated for suspected underlying autoimmune or rheumatologic disease. Additionally, malignancy had to be ruled out as well. To start, ESR and CRP were positive while ANA, RF, complements and C1 esterase inhibitors returned negative. X-rays of affected joints including bilateral hands and wrists, showed no erosive lesions. A CT scan of the chest, abdomen, and pelvis revealed extensive adenopathy of the hemithorax, abdomen, and pelvis along with splenomegaly. Pulmonary infiltrates were patchy and somewhat nodular in several areas that raised suspicion for lymphoma. Lymph node biopsy pathology revealed NK/T-cell lymphoma. The patient subsequently underwent steroid, R-CHOP and radiation therapy, significantly improving his edema and arthralgias. Discussion The symptoms and unique findings that make up RS(3)PE appear to represent a steroid-responsive disease that may be a harbinger of an underlying malignancy. Studies suggest that inflammatory processes of malignancy involving VEGF and TNF alpha may play a causative role in RS(3)PE. Literature says that 20-30% of the malignancies are associated with RS(3)PE, including hematologic malignancies such as myelodysplastic syndrome, Non-Hodgkin’s lymphoma, T-cell lymphoma, and chronic lymphocytic leukemia. Treatment is focused on low dose steroids, anti-inflammatories, as well as treating the underlying disease. Conclusion Our case demonstrates a strong correlation between NK/T-cell lymphoma and RS(3)PE syndrome. In patients with RS(3)PE syndrome, it is important to exclude autoimmune or rheumatologic illnesses. Early treatment with steroids is shown to be beneficial. This abstract is funded by: None
Chaudhari et al. (Fri,) studied this question.