1 / 64

Scleroderma and Inflammatory Myositis

Scleroderma and Inflammatory Myositis. Kathryn Dao, MD Arthritis Center February 16, 2006. Scleroderma. “Skleros-” = hard “-derma” = skin Incidence 1-2/100,000 in USA Peak age of onset 30-50 y.o. Female:male 7-12: 1

lassie
Download Presentation

Scleroderma and Inflammatory Myositis

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript


  1. Scleroderma and Inflammatory Myositis Kathryn Dao, MD Arthritis Center February 16, 2006

  2. Scleroderma • “Skleros-” = hard “-derma” = skin • Incidence 1-2/100,000 in USA • Peak age of onset 30-50 y.o. • Female:male 7-12: 1 • Disease manifestation is a result of host factors + environment (concordance is similar in monozygotic and dizygotic twins)

  3. Scleroderma Three major disease subsets: based on extent of skin dz • Localized Scleroderma • Morphea: manifests as focal patches • Linear scleroderma: band-like (linear) areas of thickening. (Coup de Sabre) • Limited disease AKA "CREST" syndrome • Calcinosis, Raynauds, Esophageal dysmotility Sclerodactyly, Telangiectasias • Diffuse disease - skin abnormalities extending to the proximal extremities (AKA - PSS) • (Scleroderma sine scleroderma)

  4. DDX of Tight Skin • Pseudosclerodactyly • IDDM, Hypothyroidism • Drugs: Tryptophan, bleomycin, pentazocine, vinyl chloride, solvents • Eosinophilic fasciitis • Overlap syndromes • Scleredema

  5. DDX of Tight Skin • Scleromyxedema (popular mucinosis) • Scleroderma-like conditions • Eosinophil myalgia syndrome (tryptophan) • Porphyria cutanea tarda • Toxic oil syndrome • Nephrogenic fibrosing dermopathy

  6. ACR Systemic Sclerosis Preliminary Classification Criteria* • Major Criterion • Proximal Scleroderma • Minor Criteria • Sclerodactyly • Digital pitting or scars or loss of finger pad • Bibasilar pulmonary fibrosis * One major and two minor required for diagnosis

  7. Scleroderma: Onset • Raynauds • Swollen or puffy digits • Loss of skin folds, no hair growth • Digital pulp sores/scars • Arthralgias >> Arthritis

  8. SclerodermaA disorder of Collagen, Vessels • Etiology: unknown? • Autoimmune disorder suggested by the presence of characteristic autoantibodies such as ANA, anti-centromere and anti-SCL-70 antibodies. • Pathology: • Early dermal changes lymphocytic infiltrates primarily of T cells • Major abnormality is collagen accumulation with fibrosis.

  9. SclerodermaA disorder of Collagen, Vessels • Small to medium-sized blood vessels, which show bland fibrotic change Vasculopathy, NOT vasculitis! • Small thrombi may form on the altered intimal surfaces. • Microvascular disease Cold Normal Cold PSS

  10. PSS - Clinical • Skin: • Skin thickening is most noticeable in the hands, looking swollen, puffy, waxy. • Thickening extends to proximal extremity, truncal and facial skin thickening is seen. • Raynaud's phenomenon is present. • Digital pits or scarring of the distal digital pulp • Musculoskeletal: Arthralgias and joint stiffness are common. • Palpable tendon friction rubs associated with an increased incidence of organ involvement. • Muscle weakness or frank myositis can be seen.

  11. Skin Scores Extent of skin involvment predictive of survival: % Survival at 5 yr 10 yr Sclerodactyly 79-84 47-75 Truncal 48-50 22-26 J Rheumatol 1988;15:276-83.

  12. PSS - Clinical • Gastrointestinal: Esophageal dysmotility, dysphagia, malabsorptive or blind loop syndrome, constipation.

  13. Renal: Kidney involvement is an ominous finding and important cause of death in diffuse scleroderma. A hypertensive crisis (AKA renal crisis) may herald the onset of rapidly progressive renal failure.

  14. Scleroderma Renal Crisis • Risk Factors • diffuse skin involvement • rapid progression of skin thickening • disease course < 4 years • anti-RNA-polymerase III-antibodies • newly manifested anemia • newly manifested cardiac involvement • pericardial effusion • heart insufficiency • preceded high-dose corticoid therapy • pregnancy Am J Med 1984;76:779-786.

  15. Scleroderma Renal Crisis • Microangiopathic hemolytic anemia +Microscopic hematuria • Fatal before the introduction of ACE-I, CCB • Survival without ACE-I 16% @ 1 year, with ACE-I 45% at 5 years • Continue use of ACE-I even if dialysis appears imminent Ann Int Med 1990;113:352-357.

  16. Pulmonary Manifestations of PSS • Dyspnea • Pulmonary HTN primarily in CREST • Ground glass (alveolitis) • Interstitial fibrosis (bibasilar) • High resolution CT vs Gallium Scan • Major cause of death • RARE: • Pulmonary embolism • Pulmonary vasculitis

  17. PFT’s in Systemic Sclerosis • Decreased DLCO is the Earliest Marker • Increased A-a Gradient with Exercise • Restrictive Pattern •  VC,  FEV1/FVC • Pulmonary Vascular Disease •  DLCO with Normal Volumes

  18. Cardiac Findings in PSS • Myocardial fibrosis • Dilated cardiomyopathy • Cor pulmonale • Arrhythmias • Pericarditis • Myocarditis • Congestive heart failure • Myocardial infarction (Raynaud’s)

  19.  Feature LimitedCREST Diffuse PSS  Calcinosis ++ +  Arthralgia/Arthritis  ++  ++++  Pulmonary fibrosis  ++  +++  Pulmonary HTN  ++  +  Tend friction rubs  0 +++  Renal crisis 0  +  Centromere Ab  +++ +/0  Anti-Scl 70 Ab + ++ Comparison CREST v. PSS + Relative percentages: +++++ 81-100%; ++++ 61-80%; +++ 41-60%; ++ 21-40%; + 1-20%

  20. Treatment of Scleroderma • Localized: none • Raynauds: warmth, skin protection, vasodilator therapy • CREST: same as Raynauds • PSS: none proven • No Value: Steroids, Penicillamine, MTX • Cytoxan: for lung disease? • Experimental: stem cell transplant, TNF-I • Epoprostenol (Flolan): Prostacyclin • Bosentan (Tracleer): Endothelin receptor antagonist • Finger ulcers: difficult; vasodilators, Abx

  21. Inflammatory Myositis: Polymyositis/Dermatomyositis • F:M = 2:1 • Acute onset • Weakness (+ myalgia): Proximal > Distal • Skeletal muscle: dysphagia, dysphonia • Sx: Rash, Raynauds, dyspnea • 65% elevated CPK, aldolase • 50% ANA (+) • 90% +EMG • 85% + muscle biopsy

  22. Proposed Criteria for Myositis • Symmetric proximal muscle weakness • Elevated Muscle Enzymes (CPK, aldolase, AST, ALT, LDH) • Myopathic EMG abnormalities • Typical changes on muscle biopsy • Typical rash of dermatomyositis • PM Dx is Definite with 4/5 criteria and Probable with 3/5 criteria • DM Dx Definite with rash and 3/4 criteria and Probable w/ rash and 2/4 criteria

  23. Polymyositis ClassificationBohan & Peter • Primary idiopathic dermatomyositis • Primary idiopathic polymyositis • Adult PM/DM associated with neoplasia • Childhood Dermatomyositis (or PM) • often associated with vasculitis and calcinosis • Myositis associated with collagen vascular disease

  24. MYOPATHY: HISTORICAL CONSIDERATIONS • Age/Sex/Race • Acute vs. Insidious Onset • Distribution: Proximal vs. Distal • Pain? • Drugs/Pre-existing Conditions • Neuropathy • Systemic Features

  25. DDX MYOPATHIIES • Toxic/Drugs • Etoh, Cocaine, Steroids, Plaquenil, Penicillamine, Colchicine, AZT, Statins, Clofibrate, Tryptophan, Taxol, Emetine • Infectious • Coxsackie, HBV, HIV, Stept, Staph, Clostridium, Toxoplasma, Trichinella • Inflammatory Myopathies • Congenital/metabolic myopathies • Neuropathic/Motor Neuron Disorders-MG, MD • Endocrine/Metabolic-hypothyroidism • Inclusion body myositis

  26. NONMYOPATHIC CONSIDERATIONS • Fibromyalgia/Fibrositis/Myofascial Pain disorder • Polymyalgia Rheumatica • Caucasians, > 55 yrs, M=F • ESR > 100, normal strength, no synovitis • CTD (SLE, RA, SSc) • Vasculitis • Adult Still's Disease

  27. INFLAMMATORY MYOSITISImmunopathogenesis • Infiltrates - T cells (HLA-DR+) & monocytes • Muscle fibers express class I & II MHC Ags • T cells are cytotoxic to muscle fibers • t-RNA antibodies: role? FOUND IN <50% OF PTS • Infectious etiology? Viral implicated • HLA-B8/DR3 in childhood DM • DR3 and DRW52 with t-RNA synthetase Ab

  28. DERMATOMYOSITIS5 Skin Features • Heliotrope Rash: over eyelids • Seldom seen in adults • Gottrons Sign/Papules (pathognomonic): MCPs, PIPs, MTPs, knees, elbows • V-Neck Rash: violaceous/erythema anterior chest w/ telangiectasias • Periungual erythema, digital ulcerations • Calcinosis

  29. Why is it called a heliotropic rash?

  30. Calcinosis

More Related