Pulmonary Manifestations of LUPUS

Mohmeet Singh BrarPG Resident

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.

Pleuropulmonary manifestations

• Pleuritis with or without effusion

• Upper and lower airway infections

• Acute Lupus Pneumonitis

• Chronic interstitial pneumonia

• Organizing pneumonia

• Pulmonary hypertension

• Pulmonary embolism

• Respiratory muscle weakness (shrinking lung syndrome)

• Alveolar hemorrhage

• Mediastinal lymphadenopathy

Allergy 2005; 60: 715-734

Pleural involvement

• Most common manifestation• Asymptomatic• Musculoskeletal pain• Pleuritic chest pain (45-60 %)• Effusions (50-70%)• Dyspnea• Fever

• 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

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• Treatment: symptomatic cough syrups, steam inhalation

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

• 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)

• BAL: lymphocytic predominance sterile cultures• Gallium scintigraphy: increased uptake• DLCO : decreased• Video-assisted thoracoscopic biopsy or open

lung biopsy: last option

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 pulse glucocorticoids (1 gram of

methylprednisolone/ day for 3days) immunosuppressive drugs (cyclophosphamide)

Sick patients( tachypnea, hypoxemia)Patients with no response after 72 hours of

prednisone• PROGNOSIS: Fulminant course High mortalityPoor prog: BAL with eosinophils, postpartum

CXR showing right lower zone shadowsImprovement of CXR after 4 weeks of therapy

INTERSTITIAL LUNG DISEASE

• Upto 9 % • Clinical features: Chronic nonproductive cough Dyspnea Decreased exercise tolerance• Diagnosis: pulmonary function tests with

exprapulmonary lupus

• 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

Patterns include • Nonspecific interstitial pneumonia (NSIP), • Usual interstitial pneumonia(UIP),• Lymphocytic interstitial pneumonia (LIP),• Cryptogenic organizing pneumonia

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.

• 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

PULMONARY HYPERTENSION

• Rare complication• 5-10% of patients• Increases with age• 2 % have right heart failure• Dyspnea• Chest pain• Chronic non productive cough

• 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

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 CHEST: dilatation of main pulmonary artery

and heterogenesity of lung perfusion

• TREATMENT: oxygen anticoagulants vasodilators :bosentan, calcium channel blockers, prostacyclin, endothelial antagonists, sildenafilIntermittent Cyclophosphamide pulse

Lupus 2004; 13:105

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

• DIAGNOSIS: triad of dyspnea clear chest x-rays elevated diaphragms • TREATMENT: alone or combination of Glucocorticoids, Theophylline Immunosuppressive therapy

PULMONARY HEMORRHAGE

• Rare complication• High mortality rates (>90%)• Clinical features: Dyspnoea Cough with hemoptysis Anemia• CXR: diffuse B/L infiltrates(LZ>UZ)

• BAL: bloody fluid hemosiderin-laden macrophagesHPE: capillaritis immune complex deposition bland hemorrhageHIGH PROBABILITY: active lupus high titre of ds DNA antibodies lupus nephritis

CXR showing B/L infiltrates in the lower zones

• TREATMENT: one or combination of high dose steroids cyclophosphamide• Plasmapheresis: severe alveolar haemorrhage refractory to corticosteroids and cytotoxic agents

Lupus 1997;6:730–3

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

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

• Treatment: cyclophosphamide pulse therapy

Vasodilators are contraindicated

Medicine (Baltimore) 2008; 87:220

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