l muscle weakness in the setting培训讲义.pptVIP

A 41 Year-old woman with proximal muscle weakness in the setting of “rheumatoid arthritis” and an abnormal chest x-ray Rheumatology Rounds December 15, 2006 Lisa Christopher-Stine, MD, MPH Assistant Professor of Medicine Co-Director, The Johns Hopkins Myositis Center No Relevant Financial Relationships with Commercial Interests Objectives To review the historical, physical examination, and pathological findings of a patient who presents with upper extremity proximal weakness ? To explore the differential diagnosis of this case To gain insight into the meaning of the associated autoantibodies noted ? To review the current literature related to this clinical entity ? To explore therapeutic treatment options Case Presentation Referral: Proximal myopathy in the setting of a diagnosis of rheumatoid arthritis 41 year-old woman w/ complicated medical history 1984: unrestrained passenger in MVA; leg weakness; ? Upper arm soreness Circa 1988: Wheezing and SOB, attributed to smoking 1988: c/o arm weakness, soreness; neuro eval History 1988: CXR shows hilar adenopathy noted on pre-employment physical examination 1989: Knee and ankle swelling; rheum eval reveals high RF ? Dx with RA and Rx w/ naproxen 1989: Dx with “asthma”; Rx nebs, inhalers 1988-1994: Notes “slow and steady decline of muscle strength” in the shoulder girdle 1989: Left quad muscle bx nondiagnostic 1994: Left neck lymph node biopsy History 1994-2006 Rapid deterioration in strength; arthritis and arthralgias; enlarging goiter; jaw weakness 1995: Second muscle bx of deltoid 2000: ? Right foot drop noted 2005: Consultation in JHU Arthritis Center 2006: Referral for myopathy evaluation Family History Sister: occular sarcoid, facial palsy, arthritis Maternal cousin with sarcoid Maternal grandfather: RA Medications Prednisone (1994- present) MTX and CSA – short duration Etanercept 2000-2002; restarted 2 wks ago Cyclophosphamide 2002-2004 Mycophenolate mofetil 2 g/day 12/04 12/05: IVIG x 1 course Physical Ex