Pulmonary manifestations of lupus

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Pulmonary manifestations of lupus

  1. 1. Pulmonary Manifestations of LUPUS Mohmeet Singh Brar PG Resident
  2. 2. Introduction SLE( systemic lupus erythematosus) • Autoimmune • Microvascular inflammation • 10:1 female to male ratio • Antibodies directed against double stranded DNA,1 nuclear ribonucleoprotein, Smith (Sm) antigen, Ro/SS-A, and La/SS-B/Ha.
  3. 3. Pleuropulmonary manifestations • Pleuritis with or without effusion • Upper and lower airway infections • Acute Lupus Pneumonitis • Chronic interstitial pneumonia • Organizing pneumonia
  4. 4. • Pulmonary hypertension • Pulmonary embolism • Respiratory muscle weakness (shrinking lung syndrome) • Alveolar hemorrhage • Mediastinal lymphadenopathy Allergy 2005; 60: 715-734
  5. 5. Pleural involvement • Most common manifestation • Asymptomatic • Musculoskeletal pain • Pleuritic chest pain (45-60 %) • Effusions (50-70%) • Dyspnea • Fever
  6. 6. • Pleural effusion: B/L exudative, lymphocytic predominant high glucose, low LDH, complement levels LE cells specific • Treatment: Minimal: no treatment, conservative Pleuritic pain: NSAIDs Severe Ds: corticosteroids Chest tube drain/pleurodesis: rarely req
  7. 7. CXR showing left sided pleural effusion.
  8. 8. CT Chest showing B/L pleural effusion
  9. 9. UPPER RESPIRATORY TRACT INFECTION • Cough : mc symptom • Viral • Patients taking corticosteroids or immunosuppresives • Treatment: symptomatic cough syrups, steam inhalation
  10. 10. Acute lupus pneumonitis • Non specific • May simulate infections • 1-4 % of patients • Cough, dyspnoea, pleuritic pain, hypoxaemia, and fever • CXR: diffuse acinar infiltrates( u/l as well as b/l) • Sterile sputum/ ET cultures
  11. 11. • Histologicaly: alveolar wall damage and necrosis, inflammatory cell infiltration, oedema, haemorrhage,hyaline membrane • CT Scan: alveolitis (a ground glass appearance) fibrosis (a honey comb appearance)
  12. 12. • BAL: lymphocytic predominance sterile cultures • Gallium scintigraphy: increased uptake • DLCO : decreased • Video-assisted thoracoscopic biopsy or open lung biopsy: last option
  13. 13. Diffuse acinar infiltrates in the right lower zone
  14. 14. ARROW showing basal cosolidation with atelectasis
  15. 15. Treatment: • Broad spectrum antibiotic cover • Systemic prednisone (1 to 1.5 mg/kg per day in divided doses) • Intravenous pulse glucocorticoids (1 gram of methylprednisolone/ day for 3days) immunosuppressive drugs (cyclophosphamide)
  16. 16. Sick patients( tachypnea, hypoxemia) Patients with no response after 72 hours of prednisone • PROGNOSIS: Fulminant course High mortality Poor prog: BAL with eosinophils, postpartum
  17. 17. CXR showing right lower zone shadows Improvement of CXR after 4 weeks of therapy
  18. 18. INTERSTITIAL LUNG DISEASE • Upto 9 % • Clinical features: Chronic nonproductive cough Dyspnea Decreased exercise tolerance • Diagnosis: pulmonary function tests with exprapulmonary lupus
  19. 19. • PFT: restrictive pattern decreased TLC decreased DLCO • HRCT CHEST: ground glass appearance centrilobular nodules thickened bronchovascular bundles and airspaces • LUNG BIOPSY: cellular infiltration with fibrotic changes
  20. 20. Patterns include • Nonspecific interstitial pneumonia (NSIP), • Usual interstitial pneumonia(UIP), • Lymphocytic interstitial pneumonia (LIP), • Cryptogenic organizing pneumonia
  21. 21. Chronic interstitial pneumonia in a 35 year old woman with SLE.HRCT scan shows extensive ground glass opacities admixed with coarse linear bands and honeycomb cysts.
  22. 22. • TREATMENT: depends upon inflammatory or fibrotic pattern • Dual therapy • High glucocorticoids (prednisone 1 to 2 mg/kg/day) and cyclophosphamide • Transition to either azathioprine or mycophenolate mofetil after 6 to 12 months. • Nothing established for fibrotic disease • PIRFENIDONE( tyrosine kinase inhibitor): trial basis
  23. 23. PULMONARY HYPERTENSION • Rare complication • 5-10% of patients • Increases with age • 2 % have right heart failure • Dyspnea • Chest pain • Chronic non productive cough
  24. 24. • Prominent JVP • Hepatomegaly • Ascites • Peripheral edema • CXR: enlarged pulmonary arteries with clear lung fields. straightening of the left heart border and attenuation of the peripheral vessels
  25. 25. CXR showing straightening of the left heart border and attenuation of the peripheral vessels
  26. 26. • ECG: RVH • PFT: restrictive pattern decreased DLCO • 2 D ECHO: inc PAP and TR ( tricuspid valve insufficiency) • CT CHEST: dilatation of main pulmonary artery and heterogenesity of lung perfusion
  27. 27. • TREATMENT: oxygen anticoagulants vasodilators :bosentan, calcium channel blockers, prostacyclin, endothelial antagonists, sildenafil Intermittent Cyclophosphamide pulse Lupus 2004; 13:105
  28. 28. SHRINKING LUNG SYNDROME • Less common manifestation • Dyspnea, • Pleuritic chest pain • Progressive decrease in lung volume • No evidence of interstitial fibrosis or pleural disease on chest CT • myositis or myopathy affecting both diaphragms
  29. 29. • DIAGNOSIS: triad of dyspnea clear chest x-rays elevated diaphragms • TREATMENT: alone or combination of Glucocorticoids, Theophylline Immunosuppressive therapy
  30. 30. PULMONARY HEMORRHAGE • Rare complication • High mortality rates (>90%) • Clinical features: Dyspnoea Cough with hemoptysis Anemia • CXR: diffuse B/L infiltrates(LZ>UZ)
  31. 31. • BAL: bloody fluid hemosiderin-laden macrophages HPE: capillaritis immune complex deposition bland hemorrhage HIGH PROBABILITY: active lupus high titre of ds DNA antibodies lupus nephritis
  32. 32. CXR showing B/L infiltrates in the lower zones
  33. 33. • TREATMENT: one or combination of high dose steroids cyclophosphamide • Plasmapheresis: severe alveolar haemorrhage refractory to corticosteroids and cytotoxic agents Lupus 1997;6:730–3
  34. 34. Cryptogenic organizing pneumonia (COP) • BOOP • Plugs of fibrous tissue in bronchioles and alveolar ducts • Dry cough • CXR: multiple infiltrates • HRCT: dense consolidation • Treatment: oral prednisone(1 mg/Kg/day) Ann Rheum Dis 1991; 50:956
  35. 35. Pulmonary venoocclusive disease (PVOD) • Rare cause of PHTN • Dyspnea and hypoxemia • Intimal fibrosis---- occlusion of pulmonary veins • HRCT CHEST: thickened interlobular septa lymph node enlargement ground glass opacities
  36. 36. • Treatment: cyclophosphamide pulse therapy Vasodilators are contraindicated Medicine (Baltimore) 2008; 87:220

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