Pulmonary manifestations of lupus

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Pulmonary Manifestations of LUPUS Mohmeet Singh Brar PG Resident

Transcript of Pulmonary manifestations of lupus

Page 1: Pulmonary manifestations of lupus

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..!!!