hypertrophic pulmonary osteoarthropathy (Bamberger-Marie syndrome)

Introduction

An expansion of long bones, either the distal ends or the entire shafts.

Etiology

• carcinoma of the lung & other malignancies
  • adenocarcinoma of the lung
  • large cell carcinoma of the lung
• other associated disorders
  • chronic pulmonary infections
    • cystic fibrosis (severe)
    • bronchiectasis
  • congenital heart disease with associated hypoxemia
    • right to left shunts
  • bacterial endocarditis
  • sarcoidosis
  • pulmonary or intestinal tuberculosis
  • myxedema
  • amebiasis
  • hepatic or biliary cirrhosis
  • inflammatory bowel disease
  • subphrenic or liver abscess
  • aortic aneurysm

Pathology

• erosions of articular cartilage
• thickening & villous proliferation of synovial membranes
• thickened & raised periosteum

Clinical manifestations

• oligo or polyarthritis
  • symmetrical arthralgias
  • large joint involvement (knees, elbows, ankles, wrists)
  • synovial effusions, pitting edema
• digital clubbing
• bone pain, periostitis, tibial tenderness
• alleviation of pain by elevating affected extremities
• gynecomastia
• fever

Laboratory

• increased erythrocyte sedimentation rate (ESR)
• increase serum C-reactive protein

Radiology

• periosteal new bone formation at the ends of long bones
• bone scan shows increased uptake of radionuclide by affected periosteum
• pelvis, joints, skull, vertebrae may show uptake of radionuclide
• chest X-ray may reveal pulmonary lesion
• CT of lung for lung carcinoma

Differential diagnosis

• Paget's disease of bone
  • uptake of radionuclide
  • no clubbing of fingers
  • no hemoptysis, blood-tinged sputum

Management

• resection of tumor
• somatostatin analog (octreotide)
• ipsilateral vagotomy

More general terms

• osteoarthropathy
• lung disease
• syndrome