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hypertrophic pulmonary osteoarthropathy (Bamberger-Marie syndrome)

An expansion of long bones, either the distal ends or the entire shafts. Etiology: 1) carcinoma of the lung & other malignancies a) adenocarcinoma of the lung b) large cell carcinoma of the lung 2) other associated disorders a) chronic pulmonary infections 1] cystic fibrosis (severe) 2] bronchiectasis b) congenital heart disease with associated hypoxemia - right to left shunts c) bacterial endocarditis d) sarcoidosis e) pulmonary or intestinal tuberculosis f) myxedema g) amebiasis h) hepatic or biliary cirrhosis i) inflammatory bowel disease j) subphrenic or liver abscess k) aortic aneurysm Pathology: 1) erosions of articular cartilage 2) thickening & villous proliferation of synovial membranes 3) thickened & raised periosteum Clinical manifestations: 1) oligo or polyarthritis a) symmetrical arthralgias b) large joint involvement (knees, elbows, ankles, wrists) c) synovial effusions, pitting edema 2) digital clubbing 3) bone pain, periostitis, tibial tenderness 4) alleviation of pain by elevating affected extremities 5) gynecomastia 6) fever Laboratory: - increased erythrocyte sedimentation rate (ESR) - increase serum C-reactive protein Radiology: 1) periosteal new bone formation at the ends of long bones 2) bone scan shows increased uptake of radionuclide by affected periosteum 3) pelvis, joints, skull, vertebrae may show uptake of radionuclide 4) chest X-ray may reveal pulmonary lesion 5) 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: 1) resection of tumor 2) somatostatin analog (octreotide) 3) ipsilateral vagotomy

General

osteoarthropathy lung disease syndrome

References

  1. Stedman's Medical Dictionary 26th ed, Williams & Wilkins, Baltimore, 1995
  2. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 720-21, 866, 770
  3. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, American College of Physicians, Philadelphia 1998
  4. NEJM Knowledge+ Question of the Week. Oct 31, 2023 https://knowledgeplus.nejm.org/question-of-week/776/ - Martinez-Lavin M. Hypertrophic osteoarthropathy. Best Pract Res Clin Rheumatol. 2020 Jun;34(3):101507. PMID: 32291203 Review.