Dados do Trabalho

Title

CANCER IN PATIENTS WITH SYSTEMIC SCLEROSIS: A SERIES OF FOUR CASES

Background

Current therapies have improved the prognosis of renal crises and lung diseases in systemic sclerosis (SSc) patients. Emerging causes of morbidity and mortality include infections, cardiovascular diseases and neoplasia. Further, recent evidence suggests that SSc patients have increased risk of developing cancer. Our aim was to describe a series of cancer in patients with SSc in treatment in our referral center.

Case report

Four cases of cancer in SSc patients are described. Case 1: a female, 43 years, black with 8 years dSSc, nucleolar antinuclear antibody (ANA) nucleolar pattern (1:1280 titer) negative anti-topoisomerase presented with fever and multiple lymphadenopathy. Biopsy confirmed malignant Non-Hodgkin lymphoma. She had received two 1g cyclophosphamide injections for interstitial lung disease. Case 2: a black female 61 years patient with dSSc, 15 years disease duration, nucleolar ANA and negative anti-topoisomerase and previous treatment with 6 pulse venous cyclophosphamide for interstitial lung disease presented with fatigue, severe weight loss and malaise. Lung computed tomography disclosed a pulmonary large nodule. Biopsy confirmed lung adenocarcinoma. Diffuse metastatic disease was identified during stratification. Case 3: a female black 56 years, 20 years limited disease duration, centromere ANA pattern presented with a breast single nodule. Radical mastectomy was performed and histopathology confirmed an adenocarcinoma. Further chemotherapy with cyclophosphamide and hormone therapy with tamoxifen was performed. Case 4: a female, black, 32 years old, 3 years disease duration female patient with dSSc, anti-ribonucleoprotein antibody presented with a large breast nodule. Needle biopsy disclosed high risk lesion and surgery is scheduled. She had pulmonary artery hypertension treated with macitentan and multiple mononeuropathy treated with 4 pulse venous cyclophosphamide in 2016. She also had received 4 months mycophenolate mofetil interrupted due to gastrointestinal intolerance.

Conclusion

An increased risk of cancer in SSc patients is recently being reported in literature. According to other studies, in our series lung, breast and lymphoproliferative cancers occurred. Of note, none of our patients had received high cyclophosphamide dose and cancer occurred on both dSSc and lSSc patients. The awareness of cancer risk in SSc patients may lead to the development of screening programs and improve patients care.