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Fellow’s Conference 11/10/10 Cheryl Pirozzi, MD

Pulmonary Manifestations of Polymyositis/Dermatomyositis Sjögren’s Syndrome and Ankylosing Spondylitis. Fellow’s Conference 11/10/10 Cheryl Pirozzi, MD. Case 1. 41 yo woman p/w weakness in shoulders and thighs, and progressive DOE. Exam reveals crackles and the following rash:. jfponline.com.

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Fellow’s Conference 11/10/10 Cheryl Pirozzi, MD

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  1. Pulmonary Manifestations ofPolymyositis/DermatomyositisSjögren’s SyndromeandAnkylosing Spondylitis Fellow’s Conference 11/10/10 Cheryl Pirozzi, MD

  2. Case 1 • 41 yo woman p/w weakness in shoulders and thighs, and progressive DOE. Exam reveals crackles and the following rash: jfponline.com dermatology.cdlib.org

  3. Case 1 • The serology most closely associated with her disease and ILD is • A) Anti- Jo-1 • B) c-ANCA • C) Anti-Ro (SS-A) • D) HLA-B27

  4. Case 1 The serology most closely associated with her disease and ILD is A) Anti- Jo-1 B) c-ANCA C) Anti-Ro (SS-A) D) HLA-B27

  5. Polymyositis and Dermatomyositis Inflammatory myopathies Clinical features: • Polymyositis: • Symmetrical proximal muscle weakness • Myositis on muscle biopsy and EMG • ↑ serum muscle enzymes • Dermatomyositis: the above plus rash: • Gottron’s papules • Violaceous heliotrope rash Bohan A, Peter J: Polymyositis and dermatomyositis. N Engl J Med  1975; 292:403-40

  6. Polymyositis and Dermatomyositis Lung manifestations of PM/DM: • Diffuse lung disease: • Interstitial pulmonary fibrosis • Acute pneumonitis (with diffuse alveolar damage) • Organizing pneumonia • Pulmonary vasculitis and alveolar hemorrhage • Respiratory muscle weakness • Aspiration pneumonia Murray and Nadel 5th edition

  7. Polymyositis and DermatomyositisDiffuse lung disease • Diffuse lung disease • Most common manifestation, up to 32% of pts • Pulmonary fibrosis: • NSIP most common pattern • Acute pneumonitis – rapidly progressive • DAD histopath • Organizing pneumonia Semin Arthritis Rheum 2003; 32:273-284.

  8. Polymyositis and DermatomyositisDiffuse lung disease • Am J Respir Crit Care Med 2001; 164:1182-1185 • 70 pts with ILD and PM or DM • Surgical lung biopsies in 22 pts • NSIP in 82% • diffuse alveolar damage (DAD) in 9% • BOOP in 4% • UIP in 4%

  9. Polymyositis and DermatomyositisDiffuse lung disease • Nonspecific interstitial pneumonia (NSIP) • most common pattern • Path: Lymphoplasmocytic infiltration, thickening of alveolar structures • HRCT: Patchy interstitial pattern with GGOs Murray and Nadel 5th edition

  10. Polymyositis and DermatomyositisDiffuse lung disease Clinical features • Presenting sxs: dyspnea, nonproductive cough, DOE, hemoptysis (if capillaritis) • Pulm sxs can develop before systemic dz or at any point • Severity of pulm involvement does not correlate with severity of musculoskeletal sxs Murray and Nadel 5th edition

  11. Polymyositis and DermatomyositisDiffuse lung disease Evaluation • PFTs: restrictive pattern, ↓DLCO • If hemorrhage or severe myopathy, DLCO may be disproportionately preserved • BAL: lymphocytosis and neutrophilia (worse prognosis) Murray and Nadel 5th edition

  12. Polymyositis and DermatomyositisDiffuse lung disease Evaluation • Labs: • AutoAbs to tRNA synthetases correlate with ILD: • Jo-1 (in 50-100% of pts with ILD) • (antisynthetase syndrome = myositis, ILD, and arthritis) • Other: Ku, PL-12, PL-7, EJ, OJ • Low CK associated with more severe ILD Murray and Nadel 5th edition

  13. Polymyositis and Dermatomyositis Other lung manifestations of PM/DM: • Respiratory muscle weakness • Aspiration pneumonia • Hypercapneic respiratory failure • Bilateral diaphragm paralysis Murray and Nadel 5th edition

  14. Polymyositis and Dermatomyositis Treatment • Corticosteroids: • PO prednisone 0.75-1.0 mg/kg/d • IV steroids in severe/rapidly progressive dz • Taper after 1 month, depending on response • If steroid resistant or unable to tolerate: • Cyclophosphamide, Cyclosporine A, Azathioprine, MTX, IVIG Murray and Nadel 5th edition

  15. Polymyositis and Dermatomyositis Am J Respir Crit Care Med 2001; 164:1182-1185. Prognosis • About 50% of pts with ILD have good response to steroids • Similar survival to idiopathic NSIP • 60% at 5 yrs

  16. Case 2 • 50 yo woman has long h/o dry eyes, dry mouth, and Anti-Ro (SS-A) Abs. She presents with 2 month h/o progressive DOE. Exam reveals crackles. PFTs show a restrictive pattern and decreased DLCO. HRCT and lung biopsy are most likely to show which of the following? • A) Lymphocytic interstitial pneumonia • B) UIP • C) NSIP • D) diffuse interstitial amyloidosis • E) Lots of aspirated peanuts

  17. Case 2 • 50 yo woman has long h/o dry eyes, dry mouth, and Anti-Ro (SS-A) Abs. She presents with month h/o progressive DOE. Exam reveals crackles. PFTs show a restrictive pattern and decreased DLCO. HRCT and lung biopsy are most likely to show which of the following? • A) Lymphocytic interstitial pneumonia • B) UIP • C) NSIP • D) diffuse interstitial amyloidosis • E) Lots of aspirated peanuts

  18. Sjögren's syndrome • Autoimmune disorder of lymphocytic infiltration of the lachrymal, salivary, conjunctival, and pharyngeal mucosal glands • Cardinal clinical features: keratoconjunctivitis sicca (dry eyes) and xerostomia (dry mouth) • Primary Sjögren's syndrome (sicca sxs in isolation) or secondary (associated with CTDs such as RA, SSc, or SLE). • Anti-Ro (SS-A) or anti-La (SS-B) Abs

  19. Sjögren's syndrome • Pulm involvement is common (25%-75% in pSS) • Due to lymphocytic infiltration similar to that in salivary gland • Wide spectrum of lung processes: • Diffuse interstitial lung disease • Tracheobronchial and small airway disease • ILD and airway disease occur together in about 50% • Respiration. 2009 Apr 22;78(4):377-386 Constantopoulos. Chest 1985 Aug;88(2):226-9 Gardiner P. Primary Sjögren's syndrome. Baillieres Clin Rheumatol  1993; 7:59-7

  20. Sjögren's syndrome- Diffuse lung disease • Interstitial lung disease (25% in pSS) • Clinical presentation: p/w dry cough, dyspnea, crackles • Evaluation: • BAL: lymphocytic alveolitis, occas neutrophil predominance • PFTs: restrictive defect, ↓ DLCO • CXR: reticular or nodular opacities with basilar prominence • HRCT • Lung biopsy (not always needed) • Multiple distinct histiologic patterns Chest. 2006 Nov;130(5):1489-95 Am J Respir Crit Care Med 2005 Mar 15;171(6):632-8

  21. Sjögren's syndrome- Diffuse lung disease • ILD: Many diffferent histiologic patterns: • Nonspecific interstitial pneumonia (NSIP) (20-60%) • Lymphocytic interstitial pneumonia (14-20%) • Pseudolymphoma • Primary pulmonary lymphoma • Organizing pneumonia • usual interstitial pneumonia (UIP) • diffuse interstitial amyloidosis Chest. 2006 Nov;130(5):1489-95 Am J Respir Crit Care Med 2005 Mar 15;171(6):632-8

  22. Sjögren's syndrome- Diffuse lung disease • Parambil. Chest. 2006 Nov;130(5):1489-95 • CXR, HRCT, and biopsy of 18 pts with pSS ILD

  23. Sjögren's syndrome- Diffuse lung disease • Nonspecific interstitial pneumonia (NSIP) • most common pattern • Lymphoplasmocytic infiltration of interstitial compartment • HRCT: Patchy interstitial pattern with GGOs Respiration. 2009 Apr 22;78(4):377-386

  24. Sjögren's syndrome- Diffuse lung disease • Lymphocytic interstitial infiltration: • Lymphocytic interstitial pneumonia • Diffuse lymphocytic infiltrate, most prominent around bronchioles • HRCT: thin-walled cysts, GGO, centrilobular and subpleural nodules Murray and Nadel 5th edition

  25. Sjögren's syndrome- Diffuse lung disease • Lymphocytic interstitial infiltration: • Pseudolymphoma: nodular lymphoid hyperplasia- infiltrates of mature lymphocytes • Pulm lymphoma: interstitial vs discrete masses • Prevalence of lymphoma increased 40-50x in Sjogrens, mst common NHL Chest. 2006 Nov;130(5):1489-95

  26. Sjögren's syndrome- Diffuse lung disease • Other: follicular bronchiolitis, Organizing pneumonia, usual interstitial pneumonia (UIP), diffuse interstitial amyloidosis • UIP Chest. 2006 Nov;130(5):1489-95

  27. Sjögren's syndrome- Large and/or small airways disease • Chronic bronchitis/lymphocytic bronchiolitis: lymphocytic infiltration around bronchioles • Can present like COPD. • HRCT: bronchial thickening, bronchiectasis, centrilobular nodules, mosaic attenuation • PFTs: If primarily small airway dz- obstruction. If both ILD and small airway dz- mixed pattern. • Path: Follicular bronchiolitis with lymphoid infiltrates Respiration. 2009 Apr 22;78(4):377-386

  28. Sjögren's syndrome- Large and/or small airways disease • Xerotrachea: loss of mucous secretion in trachea • Up to 25% pts, atrophy of mucous glands with lymphoplasmocytic infiltrate • Chronic severe dry cough Murray and Nadel 5th edition

  29. Sjögren's syndrome- Treatment • No prospective randomized trials of rx in SS and ILD • If asymptomatic, follow • Rx if symptomatic and functional deterioration • Prednisone 1 mg/kg/d PO x 6 months with taper If resistant or not tolerant of steroids: • Azathioprine • Cyclophosphamide • Cyclosporine Hunninghake, GW, Fauci, AS. Am Rev Respir Dis 1979; 119:471 UpToDate.com

  30. Case 3 • 35 yo man with a h/o back pain and recurrent uveitis p/w SOB and hemoptysis. Chest CT shows cavitary lesions in the RUL and LUL. These are most likely to be colonized by which pathogen? • A) TB • B) Aspergillus fumigatus • C) beta hemolytic streptococcus • D) Mycobacterium avium intracellulare • E) Coccidioidomycosis

  31. Case 3 35 yo man with a h/o back pain and recurrent uveitis p/w SOB and hemoptysis. Chest CT shows cavitary lesions in the RUL and LUL. These are most likely to be colonized by which pathogen? A) TB B) Aspergillus fumigatus C) beta hemolytic streptococcus D) Mycobacterium avium intracellulare E) Coccidioidomycosis

  32. Ankylosing Spondylitis • Seronegativespondyloarthritis • 0.05-1.5% of population, male:female = 10:1 • Associated with HLA-B27 • Inflammation-> fibrosis-> ankylosis of vertebral joints • Peripheral joint arthritis (1/3) • Extra-articular features: aortic regurg, uveitis, pulmonary disease and chest wall restriction Murray and Nadel Mayo Clin Proc. 1977 Oct;52(10):641-9

  33. Ankylosing Spondylitis • Lung disease occurs in up to 30% of patients • Subclinical lung abnormalities are found on HRCT in 40-88% of pts • Pulmonary abnormalities: • Upper lobe fibrosis • Upper lobe fibrobullous disease • Small airway disease • Bronchiectasis • Paraseptal emphysema • Pneumothorax • Chest wall restriction • Murray and Nadel • Mayo Clin Proc. 1977 Oct;52(10):641-9 • Sampaio-Barros. Clin Rheumatol (2007) 26: 225–230

  34. Ankylosing Spondylitis • Upper zone fibrosis • Most common finding, usually asymptomatic • Unilateral -> progresses to bilateral fibrobullous dz Kanathur N. Clin Chest Med - 01-SEP-2010; 31(3): 547-54 Murray and Nadel Mayo Clin Proc. 1977 Oct;52(10):641-9 Rev. Bras. Reumatol. [online]. 2009, vol.49, n.5 [cited  2010-11-09], pp. 630-637

  35. Ankylosing Spondylitis Kanathur N. Clin Chest Med - 01-SEP-2010; 31(3): 547-54 Murray and Nadel Mayo Clin Proc. 1977 Oct;52(10):641-9 ERJ March 1, 2004 vol. 23 no. 3 488-489 • Apical fibrobullous disease with upper lobe cavitation • Fungal or mycobacterialsuperinfection of cavities in up to 1/3 of pts • Aspergillus is most common pathogen • Others: atypical mycobacteria, candida • Can be complicated by life-threatening hemoptysis or spontaneous pneumothorax

  36. Ankylosing Spondylitis • Small airway disease • Bronchiectasis • Paraseptal emphysema • Spontaneous pneumothorax – 0.29% of AS pts, increased risk with apical fibrobullous dz • Sampaio-Barros. Clin Rheumatol (2007) 26: 225–230 Kanathur N. Clin Chest Med - 01-SEP-2010; 31(3): 547-54 Murray and Nadel Mayo Clin Proc. 1977 Oct;52(10):641-9

  37. Ankylosing Spondylitis • Chest wall restriction due to costovertebral ankylosis- usually mildly reduced lung volumes • OSA: 12-23% of pts • Increased risk with ↑ dz duration, older pts • May be due to restrictive pulm dz, airway obstruction due to TMJ involvement, or compression of medullary respiratory center by cervical spinal dz Kanathur N. Clin Chest Med - 01-SEP-2010; 31(3): 547-54 Murray and Nadel Mayo Clin Proc. 1977 Oct;52(10):641-9

  38. Ankylosing Spondylitis Evaluation • CXR: diffuse reticular opacities in upper zones, symmetrical • HRCT • Sampaio-Barros P. Clin Rheumatol (2007) 26: 225–230 • 52 asymptomatic AS pts • 40% HRCT abnormalities Murray and Nadel

  39. Ankylosing Spondylitis Kanathur N. Clin Chest Med - 01-SEP-2010; 31(3): 547-54 Murray and Nadel Evaluation BAL: often normal, may show lymphocytic alveolitis Sputum cx for mycobacterium or fungi Histology: mix of lymphocytic infiltration, fibrosis, bullous change PFTs: may have mild restriction (mostly due to ↓ thoracic cage compliance rather than apical fibrosis). DLCO usually nl.

  40. Ankylosing Spondylitis Treatment • No treatment has been shown to alter the clinical course of apical fibrobullous disease • Rx pulmonary superinfections with antifungal or antibacterial agents (systemically or into cavities) • Medical rx of Aspergillus infected cavitary lesions often unsuccessful, may require surgical excision. Kanathur N. Pulmonary manifestations of ankylosing spondylitis. Clin Chest Med - 01-SEP-2010; 31(3): 547-54

  41. Conclusions • Pulmonary disease is common in PM/DM, Sjogrens, and ankylosing spondylitis. • In polymyositis and dermatomyositis the most common form of pulm disease is ILD, usually NSIP. • In Sjogren’s the most common abnormality is ILD, which occurs in a wide variety of histologic patterns, most commonly NSIP, then LIP. • In ankylosing spondylitis the most common finding is upper lobe fibrosis, which can be complicated by cavitation and superinfection.

  42. References • Rosenow E, Strimlan CV, Muhm JR, Ferguson RH: Pleuropulmonary manifestations of ankylosing spondylitis. Mayo Clin Proc  1977; 52:641-649 • Sampaio-Barros P, Cerqueira E, Rezende S, et al: Pulmonary involvement in ankylosing spondylitis. Clin Rheumatol (2007) 26: 225–230 • Boushea, DK, Sundstrom, WR. The pleuropulmonary manifestations of ankylosing spondylitis. Semin Arthritis Rheum 1989; 18:277.   • Bronchoalveolar lavage and transbronchial biopsy in spondyloarthropathies. Kchir MM; Mtimet S; Kochbati S; Zouari R; Ayed M; Gharbi T; Hila. J Rheumatol 1992 Jun;19(6):913-6 • Bohan A, Peter J. Polymyositis and dermatomyositis. N Engl J Med  1975; 292:403-407 • Douglas WW, Tazelaar HD, Hartman TE, et al: Polymyositis-dermatomyositis–associated interstitial lung disease. Am J Respir Crit Care Med 2001; 164:1182-1185. • Schnabel A, Reuter M, Biederer J, et al: Interstitial lung disease in polymyositis and dermatomyositis: Clinical course and response to treatment. Semin Arthritis Rheum 2003; 32:273-284. • Shi JH; Liu HR; Xu WB; Feng RE; Zhang ZH; Tian XL; Zhu YJ. Pulmonary Manifestations of Sjogren's Syndrome. Respiration. 2009 Apr 22;78(4):377-386.

  43. References • Kanathur N. Pulmonary manifestations of ankylosing spondylitis. Clin Chest Med - 01-SEP-2010; 31(3): 547-54 • Hunninghake, GW, Fauci, AS. Pulmonary involvement in the collagen vascular disease. Am Rev Respir Dis 1979; 119:471 • T. Franquet , N.L. Müller and J.D. Flint. A patient with ankylosing spondylitis and recurrent haemoptysis. European Respiratory Journal. March 1, 2004 vol. 23 no. 3 488-48 • Ribeiro de Carvalho, Deheinzelin D, Kairalla R, King T. Interstitial lung disease associated with Sjögren's syndrome. UpToDate.com

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