pulmonary manifestations of lupus
TRANSCRIPT
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Pulmonary Manifestations of LUPUS
Mohmeet Singh BrarPG Resident
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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.
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Pleuropulmonary manifestations
• Pleuritis with or without effusion
• Upper and lower airway infections
• Acute Lupus Pneumonitis
• Chronic interstitial pneumonia
• Organizing pneumonia
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• Pulmonary hypertension
• Pulmonary embolism
• Respiratory muscle weakness (shrinking lung syndrome)
• Alveolar hemorrhage
• Mediastinal lymphadenopathy
Allergy 2005; 60: 715-734
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Pleural involvement
• Most common manifestation• Asymptomatic• Musculoskeletal pain• Pleuritic chest pain (45-60 %)• Effusions (50-70%)• Dyspnea• Fever
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• Pleural effusion: B/L exudative, lymphocytic predominant high glucose, low LDH, complement levels LE cells specific• Treatment: Minimal: no treatment, conservativePleuritic pain: NSAIDsSevere Ds: corticosteroidsChest tube drain/pleurodesis: rarely req
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CXR showing left sided pleural effusion.
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CT Chest showing B/L pleural effusion
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UPPER RESPIRATORY TRACT INFECTION
• Cough : mc symptom• Viral• Patients taking corticosteroids or
immunosuppresives• Treatment: symptomatic cough syrups, steam inhalation
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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
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• 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)
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• BAL: lymphocytic predominance sterile cultures• Gallium scintigraphy: increased uptake• DLCO : decreased• Video-assisted thoracoscopic biopsy or open
lung biopsy: last option
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Diffuse acinar infiltrates in the right lower zone
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ARROW showing basal cosolidation with atelectasis
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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)
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Sick patients( tachypnea, hypoxemia)Patients with no response after 72 hours of
prednisone• PROGNOSIS: Fulminant course High mortalityPoor prog: BAL with eosinophils, postpartum
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CXR showing right lower zone shadowsImprovement of CXR after 4 weeks of therapy
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INTERSTITIAL LUNG DISEASE
• Upto 9 % • Clinical features: Chronic nonproductive cough Dyspnea Decreased exercise tolerance• Diagnosis: pulmonary function tests with
exprapulmonary lupus
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• 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
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Patterns include • Nonspecific interstitial pneumonia (NSIP), • Usual interstitial pneumonia(UIP),• Lymphocytic interstitial pneumonia (LIP),• Cryptogenic organizing pneumonia
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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.
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• 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
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PULMONARY HYPERTENSION
• Rare complication• 5-10% of patients• Increases with age• 2 % have right heart failure• Dyspnea• Chest pain• Chronic non productive cough
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• 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
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CXR showing straightening of the left heart border and attenuation of the peripheral vessels
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• 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
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• TREATMENT: oxygen anticoagulants vasodilators :bosentan, calcium channel blockers, prostacyclin, endothelial antagonists, sildenafilIntermittent Cyclophosphamide pulse
Lupus 2004; 13:105
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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
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• DIAGNOSIS: triad of dyspnea clear chest x-rays elevated diaphragms • TREATMENT: alone or combination of Glucocorticoids, Theophylline Immunosuppressive therapy
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PULMONARY HEMORRHAGE
• Rare complication• High mortality rates (>90%)• Clinical features: Dyspnoea Cough with hemoptysis Anemia• CXR: diffuse B/L infiltrates(LZ>UZ)
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• BAL: bloody fluid hemosiderin-laden macrophagesHPE: capillaritis immune complex deposition bland hemorrhageHIGH PROBABILITY: active lupus high titre of ds DNA antibodies lupus nephritis
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CXR showing B/L infiltrates in the lower zones
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• TREATMENT: one or combination of high dose steroids cyclophosphamide• Plasmapheresis: severe alveolar haemorrhage refractory to corticosteroids and cytotoxic agents
Lupus 1997;6:730–3
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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
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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
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• Treatment: cyclophosphamide pulse therapy
Vasodilators are contraindicated
Medicine (Baltimore) 2008; 87:220
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Thanks..!!!