Which malignancies are associated with preexisting CTD

Which malignancies are associated with preexisting CTD?

Malignancies associated with preexisting connective tissue diseases are outlined in the below table

Preexisting CTD Associated with Malignancy

Disease (Risk)MalignancyClinical Risks
Rheumatoid arthritisLymphoproliferative disorders (2–3 times increased risk)
Lung cancer (2 times increased risk)
Melanoma and other skin cancers
Longer disease duration, high disease activity immunosuppression, Felty’s syndrome
Systemic lupus erythematosusLymphoproliferative disorders (2–4 times increased risk)
Cervical cancer
Adenopathy, ↑ spleen
HPV-related
Discoid lupusSquamous cell epitheliomaIn plaques >20 years
Sjögren’s syndrome (1.5×)Lymphoproliferative disorders, hematologic malignancies (4%–10% lifetime risk)Palpable purpura, cutaneous ulcers, cryoglobulinemia, adenopathy, ↑ spleen, germinal centers MSG
Systemic sclerosis (1.4×)Alveolar cell carcinoma, nonmelanoma skin cancer, adenocarcinoma of esophagus, lymphoproliferative disordersPulmonary fibrosis, areas of skin fibrosis, Barrett’s metaplasia, anti-RNAP III
DM (4× to 7×)Adenocarcinoma, melanoma, lymphoproliferative disorders, nasopharyngeal tumors (Asians)Older age, ulcerative skin lesions, anti-p155/p140, anti-NXP-2
PM (1.6×)AdenocarcinomaPM < DM
ANCA vasculitis (1.7×)Various cancersSome due to cyclophosphamide
Paget’s diseaseOsteogenic sarcomaOccurs in 1% of lesions
Eosinophilic fasciitisLymphoproliferative disordersAplastic anemia, thrombocytopenia

ANCA, Antineutrophil cytoplasmic antibody; HPV, human papillomavirus; MSG, minor salivary gland biopsy.

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