Abstract

Paraneoplastic rheumatic syndromes comprise a heterogeneous group of disorders characterized by typical rheumatic manifestations but without direct invasion by the tumor or metastases. The clinical features and malignancy-associated risk factors of 21 patients with paraneoplastic rheumatic syndromes, including 11 men and 10 women with a mean age of 56.3 ± 13.1 years, were characterized by a retrospective review. All patients were diagnosed with malignancy within 2 years of rheumatism diagnosis. Patients suffering from solid malignancies accounted for the majority (62%); hematological malignancies were observed in the remainder. Arthritis (48%), lymph node enlargement (38%), skin rash (38%), weight loss (29%), fever/chills (24%), fatigue (24%), muscle soreness (24%) and smoking history (29%) were common findings. Except for 8 patients (38%) who tested positive for anti-nuclear antibody (ANA) and 9 positive for rheumatoid factor (RF), all patients tested negative for anti-extractable nuclear antigen (ENA) antibodies. Rheumatic disorders with a typical clinical presentation in older patients and nonspecific systemic features should alert clinicians to search for an occult malignancy. Patients with rheumatic disease must be closely followed to screen for malignancies, particularly within 2 years of rheumatism diagnosis.

Highlights

  • Paraneoplastic syndromes involve symptoms mediated by hormones and cytokines from a tumor or are the consequence of humoral or cellular immune mechanisms directed against tumor cells, though direct invasion by the tumor or metastases does not occur

  • An incidence of paraneoplastic rheumatic disease of 2.65%–23.1% has been reported, and all types of rheumatism or rheumatic symptoms are present in malignant diseases[10,11,12,13]

  • Previous studies have shown that malignancy typically occurs within 24 months in patients with paraneoplastic rheumatic disease[14,15]

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Summary

Introduction

Paraneoplastic syndromes involve symptoms mediated by hormones and cytokines from a tumor or are the consequence of humoral or cellular immune mechanisms directed against tumor cells, though direct invasion by the tumor or metastases does not occur. Several studies have indicated connections between malignancy and rheumatic manifestations[1,2,3]. In the case of paraneoplastic rheumatic syndromes, rheumatic symptoms can coincide, precede, or follow the diagnosis of cancer or herald its recurrence[4], generally at no longer than 2 years before the diagnosis of associated cancer[5,6,7]. Malignancies are associated with a wide variety of paraneoplastic rheumatic manifestations, which may arise in joints, fasciae, muscles, vessels or bones[8,9]. This study aimed to examine the relationship between rheumatic manifestations and malignancy to further improve our understanding of paraneoplastic rheumatic disease and to avoid misdiagnosis

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