Hypertrophic Osteoarthropathy

Transcription

1 September 2005 Hypertrophic Osteoarthropathy Roxanne Landesman, Harvard Medical School Year III

2 Hypothetical Patient A patient presents with persistent right ankle pain, and no history of trauma. As the 3 rd year medical student reviews the plain film with the radiologist, he is immediately concerned about the patient s 2

3 Hypothetical Case A patient presents with persistent right ankle pain, and no history of trauma. As the 3 rd year medical student reviews the plain film with the radiologist, he is immediately concerned about the patient s Lungs! He recommends a plain film of the contralateral ankle, and later, a chest film. 3

4 Right ankle Opaque line of new bone formation, Separated from underlying cortex by narrow radiolucent line Ossifying periostitis dirt/museum/p7-262.html 4

5 Later The contralateral ankle showed similar signs. A chest film revealed a small mass in the left lung. The patient s cancer was detected solely because of ankle pain! The medical student had remembered Hypertrophic pulmonary osteoarthropathy. 5

6 Hypertrophic Osteoarthropathy Clinical Syndrome: (HOA) Pain in the extremities (often lower) +/- Clubbing of fingers and toes +/- Articular symptoms (30-40% of HOA pts) Pain, tenderness, swelling May mimic rheumatoid arthritis +/- limb skin thickening 6

7 Agenda Introduction to HOA Radiologic menu of tests Radiologic findings Concise presentation of three patients findings Summary of HOA findings Diseases associated with HOA Pathophysiology of HOA? Take home message 7

8 Classification of HOA 3-5% Primary Secondary 95-97% =Pachydermoperiostosis Genetic: autosomal dominant Associated with: Intrathoracic, extrathoracic; neoplastic,, infectious, inflammatory disease processes Lung cancers mesothelioma tuberculosis lymphoma Inflammatory bowel dz Cystic fibrosis Hepatobiliary dz thymoma 8

9 Patients with HOA May or may not have bony symptoms Pain, when present, may be minor to severe May or may not have clubbing of the digits May be patients with a known associated disease (lung cancer, IBD, ) May be patients whom you suspect have a new metastasis to the lung May appear perfectly healthy and present to you only for bone pain. 9

10 Radiologic Examinations of Choice Plain film Fast, simple, cheap, readily available Likely test for patients presenting with limb pain Nuclear medicine: bone scan Most sensitive May also detect metastases CT Detect underlying cause of 2 HOA MRI Non-standard Non yield findings specific for HOA 10

11 HOA: Radiologic Findings Ossifying periostitis [usually] symmetric Long bones most common Distal shaft, progressing proximally [usually] may also affect metacarpals, metatarsals, scapulae, other bones Radiological Differential Diagnosis: Thyroid acropachy, chronic venous stasis, hypervitaminosis A, infantile hyperostosis (Caffey Dz), even shin splints Must combine with clinical history and presentation to achieve specific diagnosis. 11

12 Patient A Patient A 53yo male presented 1 year ago with hematuria, diagnosed with renal cell carcinoma Nephrectomy performed; bone scan negative for metastases Lung metastases detected by CT at 6 month f/u; patient asymptomatic for lung disease Over the next six months, patient develops increasingly severe pain bilaterally in knees, lower limbs, wrists, hands uncontrolled by oxycodone, celecoxib, indomethacin [new] clubbing was recently noted Current bone scan: 12

13 Increased tracer uptake in distal femurs, fibulae, tibiae, Renal osteodystrophy vs. 2 2 HOA? - Lab values and history of pulmonary mets 2 HOA more likely BIDMC PACS 13

14 2 HOA parallels 1 disease All HOA findings (clinical, radiographic, scintigraphic) generally disappear with effective treatment of primary disease (often within days!) Recurrence of primary disease is often heralded by recurrence of HOA. 14

15 Patient B 53-year-old male smoker presented with bilateral lower-limb pain of the hips, knees, and ankles 15

16 Patient B, continued Biopsy confirmed squamous carcinoma 16

17 Patient C 29yo female with inflammatory bowel disease c/o 3 months of worsening shin pain Lateral view showing increased activity in the tibial periosteum ( railroad sign ) BIDMC PACS 17

18 HOA, Summary of Classic Findings Periostitis Commonly: Bilateral Long bones +/- bone and joint pain +/- Clubbing BIDMC PACS +/- other skin changes 18

19 2 HOA: Associated Disease Processes Neoplasms or states of chronic infection or inflammation: Pulmonary Bronchogenic carcinoma (1-12% of these pts) Other primary lung tumors Metastases Cystic Fibrosis Infections: TB, histo, blasto, pneumocystis Pleural: mesothelioma, pleural fibroma, Cardiac: cyanotic heart dz with right to left shunt, Intestinal and hepatic: cirrhosis, hepatocellular carcinoma, Approx 90% are intra- thoracic lymphoma, other neoplasms, polyposis, inflammatory bowel dz, dysentery, 19

20 HOA: Pathophysiology Unknown/Debated Latest theories note that most associated dz processes involve some degree of R L shunting of blood, thus allowing large platelets to escape fragmentation in the pulmonary microvasculature and thus impact in distal capillary beds. There they may stimulate endothelial cell activation via PDGF, etc, thus stimulating connective tissue matrix synthesis leading to clubbing, HOA has been induced in dogs by creating R L shunts 20

21 A Final Point If you detect any aspect of the HOA syndrome, especially in a previously healthy patient, Be on guard for the possibility of a serious associated disease process! Focus on the chest, but don t forget other possible sites. 21

22 References Ali A, Tetalman MR, Fordham EW et al. Distribution of hypertropic pulmonary osteoarthropathy. Am J Radiol 1980; 134: Martinez-Lavin M: Hypertrophic osteoarthropathy. Curr Opin Rheumatol 9:83 86, 1997 Andres R, Saenz A et al. Case 4. Hypertrophic osteoarthropathy associated with pulmonary metastasis of uterine leiomyosarcoma. J Clin Oncol Sep 15;21(18): Faulhaber PF. Nuclear and SPECT Teaching Files - Case One -Hypertrophic Osteoarthropathy. accessed 17 Sep 2005 Rothschild BM, Rothschild C: Recognition of hypertrophic osteoarthropathy in skeletal remains. J Rheumatol 1998 Nov; 25(11):

23 Acknowledgements Thanks to J. Anthony Parker, MD PhD Larry Barbaras, our webmaster Pamela Lepkowski for her wise advice 23