Pulmonary Manifestations of SLE

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Pulmonary Manifestations of SLE

• Ted Carter, MD
• Atlanta Medical Center

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Pulmonary Manifestations of SLE

• Pleuritic Chest Pain
• Upper Respiratory Tract Infections
• Acute Pneumonitis
• Chronic Pneumonitis
• Pulmonary Hypertension
• Shrinking Lung Syndrome
• Pulmonary Hemorrhage
• Other

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Pleuritic Chest Pain

• Musculoskeletal pain
• Pleuritis

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Pleuritic Chest PainMusculoskeletal

• 50 of SLE patients experience musculoskeletal or
  pleuritic chest pain
• Most commonly from muscle, connective tissue, or
  costochondral joints (Tietzes syndrome)
• Responds to local heat, NSAIDs, topical
  analgesics, and Tylenol

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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

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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

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Pleuritic Chest PainEffusions

• Dont forget to exclude
• Infection
• CHF
• Uremia

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Upper Respiratory Tract Infection

• Usually presents as a cough
• Usually viral
• More frequent in SLE patients due to treatment
  with corticosteroids or immunosuppressive drugs

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Pneumonitis

• Acute
• Chronic

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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

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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

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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 mainstay1.5mg/kg qd

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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

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Chronic PneumonitisDiagnosis

• Differentiate from
• Pulmonary edema
• ARDS
• Bilateral pneumonia
• Interstitial fibrosis
• Infection
• Malignancy
• Granulomatous disease

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Chronic PneumonitisDiagnosis

• HRCT useful
• Look for
• ground glass appearance, or
• reticular pattern

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Chronic PneumonitisGround Glass Appearance on
HRCT
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Chronic PneumonitisReticular Pattern on HRCT
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Chronic PneumonitisDiagnosis

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

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Chronic PneumonitisDiagnosis using
Bronchioalveolar Lavage

• Use to exclude infection, malignancy, and
  granulomatous disease
• gt10 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

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Chronic PneumonitisTreatment

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

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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

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Pulmonary Hypertension

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

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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

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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

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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

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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

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Other Pulmonary Disorders

• ARDS
• Commonly due to bacteremia with Gm- bacteria
• More likely in those treated with steroids
  within the previous month
• 68 mortality

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Other Pulmonary Disorders

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