RR

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 (