Rheumatic Paraneoplastic Syndromes and Malignancies With Rheumatic Manifestations
Introduction
- Complex relationship between cancer and rheumatic diseases.
- Bidirectional influences:
- Rheumatic diseases can increase cancer risk (inflammation, treatments).
- Cancer or treatments can cause autoimmune/rheumatic manifestations.
- Focus: features of rheumatic paraneoplastic syndromes that indicate underlying malignancy.
Paraneoplastic Syndromes
- Definition: Rare syndromes mediated by factors from tumors, not directly by the tumor.
- Clinical manifestations often involve:
- Joints
- Muscles
- Fasciae
- Vessels
- Bones
- Causality must be shown according to Bradford Hill Criteria:
- Strength, specificity, and temporal relationship between rheumatic symptoms and tumor diagnosis crucial.
- Symptoms should appear simultaneously or within 3 years of malignancy detection.
- Symptoms often resolve after successful elimination of the tumor.
Key Points
- Knowledge of paraneoplastic syndromes enhances diagnosis and treatment timelines.
- Common musculoskeletal manifestations linked to malignancies:
- Paraneoplastic arthritis
- Palmar fasciitis and polyarthritis syndrome (PFPAS)
- Remitting seronegative symmetric synovitis with pitting edema (RS3PE)
- Erythromelalgia
- Paraneoplastic vasculitis
- Hypertrophic osteoarthropathy
- Eosinophilic fasciitis
- Tumor-induced osteomalacia
- Cancer-associated myositis and scleroderma
Paraneoplastic Arthritis (PA)
- Characteristics:
- Acute, asymmetric polyarthritis (34%), oligoarthritis (48%), monoarthritis (18%).
- Markedly elevated inflammation markers.
- Diagnostic challenge:
- 23% test positive for rheumatoid factor.
- Atypical joint involvement patterns and poor response to corticosteroids suggest malignancy.
Palmar Fasciitis and Polyarthritis Syndrome (PFPAS)
- Symptoms:
- Swollen, painful palms and fingers (