Post on 07-May-2015
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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