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Pulmonary Manifestations of
LUPUS
Mohmeet Singh Brar
PG Resident
Introduction
SLE( systemic lupus erythematosus)
• Autoimmune
• Microvascular inflammation
• 10:1 female to male ratio
• An...
Pleuropulmonary manifestations
• Pleuritis with or without effusion
• Upper and lower airway infections
• Acute Lupus Pneu...
• Pulmonary hypertension
• Pulmonary embolism
• Respiratory muscle weakness (shrinking lung syndrome)
• Alveolar hemorrhag...
Pleural involvement
• Most common manifestation
• Asymptomatic
• Musculoskeletal pain
• Pleuritic chest pain (45-60 %)
• E...
• Pleural effusion: B/L
exudative, lymphocytic predominant
high glucose, low LDH, complement levels
LE cells specific
• Tr...
CXR showing left sided pleural effusion.
CT Chest showing B/L pleural effusion
UPPER RESPIRATORY TRACT
INFECTION
• Cough : mc symptom
• Viral
• Patients taking corticosteroids or
immunosuppresives
• Tr...
Acute lupus pneumonitis
• Non specific
• May simulate infections
• 1-4 % of patients
• Cough, dyspnoea, pleuritic
pain, hy...
• Histologicaly:
alveolar wall damage and necrosis,
inflammatory cell infiltration,
oedema, haemorrhage,hyaline membrane
•...
• BAL: lymphocytic predominance
sterile cultures
• Gallium scintigraphy: increased uptake
• DLCO : decreased
• Video-assis...
Diffuse acinar infiltrates in the right lower zone
ARROW showing basal cosolidation with atelectasis
Treatment:
• Broad spectrum antibiotic cover
• Systemic prednisone (1 to 1.5 mg/kg per day
in divided doses)
• Intravenous...
Sick patients( tachypnea, hypoxemia)
Patients with no response after 72 hours of
prednisone
• PROGNOSIS:
Fulminant cours...
CXR showing right lower zone
shadows
Improvement of CXR after 4
weeks of therapy
INTERSTITIAL LUNG DISEASE
• Upto 9 %
• Clinical features:
Chronic nonproductive cough
Dyspnea
Decreased exercise tolerance...
• PFT: restrictive pattern
decreased TLC
decreased DLCO
• HRCT CHEST: ground glass appearance
centrilobular nodules
thicke...
Patterns include
• Nonspecific interstitial pneumonia (NSIP),
• Usual interstitial pneumonia(UIP),
• Lymphocytic interstit...
Chronic interstitial pneumonia in a 35 year old woman
with SLE.HRCT scan shows extensive ground glass
opacities admixed wi...
• TREATMENT: depends upon inflammatory or
fibrotic pattern
• Dual therapy
• High glucocorticoids (prednisone 1 to 2
mg/kg/...
PULMONARY HYPERTENSION
• Rare complication
• 5-10% of patients
• Increases with age
• 2 % have right heart failure
• Dyspn...
• Prominent JVP
• Hepatomegaly
• Ascites
• Peripheral edema
• CXR: enlarged pulmonary arteries with clear
lung fields.
str...
CXR showing straightening of the left heart border and
attenuation of the peripheral vessels
• ECG: RVH
• PFT: restrictive pattern
decreased DLCO
• 2 D ECHO: inc PAP and TR ( tricuspid valve
insufficiency)
• CT CHES...
• TREATMENT:
oxygen
anticoagulants
vasodilators :bosentan,
calcium channel blockers,
prostacyclin,
endothelial antagonists...
SHRINKING LUNG SYNDROME
• Less common manifestation
• Dyspnea,
• Pleuritic chest pain
• Progressive decrease in lung volum...
• DIAGNOSIS: triad of
dyspnea
clear chest x-rays
elevated diaphragms
• TREATMENT: alone or combination of
Glucocorticoids,...
PULMONARY HEMORRHAGE
• Rare complication
• High mortality rates (>90%)
• Clinical features:
Dyspnoea
Cough with hemoptysis...
• BAL: bloody fluid
hemosiderin-laden macrophages
HPE: capillaritis
immune complex deposition
bland hemorrhage
HIGH PROBAB...
CXR showing B/L infiltrates in the lower
zones
• TREATMENT: one or combination of
high dose steroids
cyclophosphamide
• Plasmapheresis:
severe alveolar haemorrhage refra...
Cryptogenic organizing pneumonia
(COP)
• BOOP
• Plugs of fibrous tissue in bronchioles and
alveolar ducts
• Dry cough
• CX...
Pulmonary venoocclusive disease
(PVOD)
• Rare cause of PHTN
• Dyspnea and hypoxemia
• Intimal fibrosis---- occlusion of pu...
• Treatment: cyclophosphamide pulse therapy
Vasodilators are contraindicated
Medicine (Baltimore) 2008; 87:220
Pulmonary manifestations of lupus
Pulmonary manifestations of lupus
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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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