Pulmonary Manifestations of SLE

1. Pulmonary Manifestations of SLE Ted Carter, MD Atlanta Medical Center

2. Pulmonary Manifestations of SLE • Pleuritic Chest Pain • Upper Respiratory Tract Infections • Acute Pneumonitis • Chronic Pneumonitis • Pulmonary Hypertension • Shrinking Lung Syndrome • Pulmonary Hemorrhage • Other

3. Pleuritic Chest Pain • Musculoskeletal pain • Pleuritis

4. Pleuritic Chest PainMusculoskeletal • 50% of SLE patients experience musculoskeletal or pleuritic chest pain • Most commonly from muscle, connective tissue, or costochondral joints (Tietze’s syndrome) • Responds to local heat, NSAIDs, topical analgesics, and Tylenol

5. Pleuritic Chest PainPleuritis • Difficult to diagnose • Rub and/or pleural effusion (20%) may be present • Effusion is usually small or moderate • Effusion is a mild exudate with elevated LDH, but no signs of marked inflammation

7. Pleuritic Chest PainEffusions • Generally, • Total white cell count is lower in SLE • Glucose levels are slightly lower than plasma levels in SLE • Low complement levels • Protein concentration is low in SLE

8. Pleuritic Chest PainEffusions • Don’t forget to exclude: • Infection • CHF • Uremia

9. Upper Respiratory Tract Infection • Usually presents as a cough • Usually viral • More frequent in SLE patients due to treatment with corticosteroids or immunosuppressive drugs

10. Pneumonitis • Acute • Chronic

11. Acute PneumonitisClinical • Uncommon, 1-12% • Fever, cough, hemoptysis, pleurisy, dyspnea • Hypoxia, basilar rales • Pleural effusion (50%) • Pulmonary infiltrates, usually lower lobes • No pathogen can be isolated

12. Acute PneumonitisPathology • Acute alveolar wall injury • Alveolar hemorrhage • Alveolar edema • Hyaline membrane formation • Immunoglobulin and complement deposition • Some authors require one of the following for diagnosis: interstitial fibrosis, vasculitis, hematoxylin bodies, interstitial pneumonitis, alveolitis, or pleuritis

13. Acute PneumonitisPrognosis and Treatment • Prognosis is generally poor • Short term mortality of 50%! • If developed during postpartum period, prognosis is very poor • Survivors have persistent PFT abnormalities with restrictive defects • Give antibiotics pending culture results • Prednisone is mainstay…1.5mg/kg qd

14. Chronic PneumonitisClinical • Up to 9% of patients with SLE • Frequently preceded by acute pneumonitis • Longstanding SLE more likely • Anti-Ro antibodies more likely • Chronic non-productive cough, dyspnea, and recurrent pleuritic chest pain • PFTs show restrictive pattern with decreased lung volume • ABGs show decreased Dlco and pO2

15. Chronic PneumonitisDiagnosis • Differentiate from Pulmonary edema ARDS Bilateral pneumonia Interstitial fibrosis Infection Malignancy Granulomatous disease

16. Chronic PneumonitisDiagnosis • HRCT useful • Look for • ground glass appearance, or • reticular pattern

17. Chronic PneumonitisGround Glass Appearance on HRCT

18. Chronic PneumonitisReticular Pattern on HRCT

19. Chronic PneumonitisDiagnosis • HRCT useful • Look for ground glass appearance, or • reticular pattern • Bronchioalveolar lavage (BAL)

20. Chronic PneumonitisDiagnosis using Bronchioalveolar Lavage • Use to exclude infection, malignancy, and granulomatous disease • >10% neutrophils suggests Chronic Pneumonitis • This finding is also observed in: Scleroderma Rheumotoid pneumonitis Idiopathic pulmonary fibrosis • Lupus is suggested by characteristic serological and extrapulmonary findings

21. Chronic PneumonitisTreatment • Oral prednisone at 1mg/kg qday • Expect slow improvement or stabilization • Immunosuppressive agents if no response

22. Pulmonary Hypertension • Rare complication of SLE • Symptoms range from dyspnea, chronic non-productive cough and chest pain to… • Fatigue, weakness, palpitations, edema, ascites and RVH • Diagnosis by echocardiogram, or right sided cardiac catheterization

23. Pulmonary Hypertension • Treatment includes oxygen, anticoagulants and vasodilators (Calcium blockers and prostacyclin infusion) • Poor prognosis with one study showing 50% 5-year mortality

24. Shrinking Lung Syndrome • Characterized by dyspnea, pleuritic chest pain, and progressive decrease in lung volume • Chest X-RAY is clear, with elevated diaphragms • Mechanism is unclear • Treatment with corticosteroids

25. Pulmonary Hemorrhage • Not necessarily associated with hemoptysis • Presenting manifestation of SLE in 10-20% of cases • CXR shows bilateral alveolar infiltrates • Unknown etiology • Bleeding can induce anemia

26. Pulmonary Hemorrhage • Diagnosis by lung biopsy • Treat with high dose corticosteroids, cyclophosphamide, and aggressive support • Plasmapheresis for patients who fail steroids • Survival ranges from 50-70%

27. Other Pulmonary Disorders • Bronchiolitis obliterans with organizing pneumonia (BOOP) • Prednisone 1mg/kg qday, or • cyclophosphamide • Acute reversible hypoxemia • Elevated C3a • Pulmonary leukoaggregation and complement activation • Corticosteroids and aspirin

28. Other Pulmonary Disorders • ARDS • Commonly due to bacteremia with Gm- bacteria • More likely in those treated with steroids within the previous month • 68% mortality

29. Other Pulmonary Disorders • Antiphospholipid antibodies • Pulmonary embolism • Thromboembolic and nonthromboembolic pulmonary hypertension • Pulmonary artery thrombosis • ARDS • Postpartum HUS • Treat with chronic anticoagulation