Hypertrophic Osteoarthropathy

 

• Etiology
  • Unknown
• Presents clinically as an arthritis with painful and swollen joints
• Can occur as a primary and secondary form
  • Primary form also know as pachydermoperiostitis
    • May include periostitis, clubbing, thickening of the skin
    • Involves hands
    • Familial
    • More common in males
    • Onset in adolescence with remission in adulthood
• Thoracic Causes
  • Malignant tumor (0.7-12%)
    • bronchogenic carcinoma (88%)
    • mesothelioma
    • lymphoma
    • pulmonary metastasis from
      • osteogenic sarcoma
      • melanoma
      • renal cell carcinoma
      • breast cancer
  • Benign tumor
    • benign pleural fibroma
    • tumor of ribs
    • thymoma
    • esophageal leiomyoma
    • pulmonary hemangioma
    • pulmonary congenital cyst
  • Chronic infection / inflammation
    • pulmonary abscess
    • bronchiectasis
    • Blastomycosis
    • TB (very rare)
    • cystic fibrosis
    • interstitial fibrosis
  • Congenital heart disease with R-to-L shunt
• Extrathoracic Causes
  • GI tract
    • ulcerative colitis
    • amebic + bacillary dysentery
    • intestinal TB
    • Whipple disease
    • Crohn's disease
    • gastric ulcer
    • bowel lymphoma
    • gastric carcinoma
  • Liver disease
    • biliary and alcoholic cirrhosis
    • posthepatic cirrhosis
    • chronic active hepatitis
    • bile duct carcinoma
    • benign bile duct stricture
    • amyloidosis,
    • liver abscess
  • Undifferentiated nasopharyngeal carcinoma
  • Pancreatic carcinoma
  • Chronic myelogenous leukemia
• Clinical Findings
  • Pain and swelling about the joints simulating an arthritis
  • Joints involved
    • ankles (88%)
    • wrists (83%)
    • knees (75%)
    • elbows (17%)
    • shoulders (10%)
    • fingers (7%)
  • Peripheral neurovascular disorders
    • local cyanosis
    • areas of increased sweating
    • paresthesia
    • chronic erythema
    • flushing + blanching of skin
  • Clubbing
  • Hypertrophy of extremities (soft-tissue swelling)
• Location
  • tibia + fibula (75%)
  • radius + ulna (80%)
  • proximal phalanges (60%), femur (50%)
  • metacarpus + metatarsus (40%), humerus + distal phalanges (25%)
  • pelvis (5%)
  • unilateral (rare)
• Imaging Findings
  • In diametaphyseal regions
    • Periosteal proliferation of new bone
      • Symmetrical
      • Widespread

Frontal and lateral radiograph of the distal femur shows a thick, wavy
 periosteal reaction that involves the diaphysis but spares the epiphysis

• Bone scan reveals changes early with greater sensitivity
  • Symmetric diffusely increased uptake along cortical margins of diaphysis and metaphysis of tubular bones of the extremities with irregularities
• Increased periarticular uptake (from synovitis)
• Scapular involvement in 2/3
• Mandible ± maxilla abnormal in 40%

• Thoracotomy can lead to immediate clinical remission; radiolographic remission follows

 Dahnert 5^th Edition