1. Pulmonary Manifestations of
LUPUS
Mohmeet Singh Brar
PG Resident

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. Pleuropulmonary manifestations
• Pleuritis with or without effusion
• Upper and lower airway infections
• Acute Lupus Pneumonitis
• Chronic interstitial pneumonia
• Organizing pneumonia

4. • Pulmonary hypertension
• Pulmonary embolism
• Respiratory muscle weakness (shrinking lung syndrome)
• Alveolar hemorrhage
• Mediastinal lymphadenopathy
Allergy 2005; 60: 715-734

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

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. CXR showing left sided pleural effusion.

8. CT Chest showing B/L pleural effusion

9. UPPER RESPIRATORY TRACT
INFECTION
• Cough : mc symptom
• Viral
• Patients taking corticosteroids or
immunosuppresives
• Treatment: symptomatic
cough syrups,
steam inhalation

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

13. Diffuse acinar infiltrates in the right lower zone

14. ARROW showing basal cosolidation with atelectasis

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. 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. CXR showing right lower zone
shadows
Improvement of CXR after 4
weeks of therapy

18. INTERSTITIAL LUNG DISEASE
• Upto 9 %
• Clinical features:
Chronic nonproductive cough
Dyspnea
Decreased exercise tolerance
• Diagnosis: pulmonary function tests with
exprapulmonary lupus

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

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

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. CXR showing straightening of the left heart border and
attenuation of the peripheral vessels

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. • TREATMENT:
oxygen
anticoagulants
vasodilators :bosentan,
calcium channel blockers,
prostacyclin,
endothelial antagonists,
sildenafil
Intermittent Cyclophosphamide pulse
Lupus 2004; 13:105

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

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

32. • 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

33. CXR showing B/L infiltrates in the lower
zones

34. • TREATMENT: one or combination of
high dose steroids
cyclophosphamide
• Plasmapheresis:
severe alveolar haemorrhage refractory
to corticosteroids and cytotoxic agents
Lupus 1997;6:730–3

35. 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

36. 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

37. • Treatment: cyclophosphamide pulse therapy
Vasodilators are contraindicated
Medicine (Baltimore) 2008; 87:220