Pulmonary Manifestations Of Systemic Lupus Erythematosus COMPREHENSIVE PRESENTATION ON PULMONARY MANIFESTATIONS OF SLE IT WILL BE VERY EASY TO UNDERSTAND AND LEARN AND TEACH THIS TOPIC INCLUDE ALL NEW GUIDELINES AND MANAGMENT.SLE is an autoimmune disease in which the immune system attacks its own tissues, causing widespread inflammation and tissue damage in the affected organs. It can affect the joints, skin, brain, lungs, kidneys, and blood vessels.

1. Pulmonary Manifestations Of
Systemic Lupus
Erythematosus
DR NOMAN SHAKIR
PGR MD PULMONOLOGY
BVH

2. INTRODUCTION
• Systemic lupus erythematosus – chronic, systemic, autoimmune
disease with relapsing-remitting course & characterized by production of
wide range of autoantibodies.
• people of all genders, ages, and ethnic groups are susceptible.
• Female to male ratio 9:1.
• Respiratory involvment 50 to 70%, can be initial presentation.

3. PATHOGENESIS

8. AIRWAY DISEASE

9. Parenchymal Lung Diseases
1.Acute diseases:
1.1Acute Lupus Pneumonitis:
• Histology of DAD, bronchiolitis obliterans organizing pneumonia, NSIP, or a
combination of these.

10. Diagnosis
 CBC
 MICROBIOLOGY
 ECHO
 CXR: Multiple Bilateral Patchy Infiltrates
 HRCT: Ground Glass Appearance; Patchy Areas Of Consolidation, Traction
Bronchiectasis, Or Pleural Effusions
 DLCO
 BAL
 LUNG BIOPSY: VATS Or Open Thoracotomy.

13. TREATMENT
• There have been no controlled trials addressing treatment of acute lupus pneumonitis.
• Mainstay Of Therapy: prednisone (1 to 1.5 mg/kg/day per day orally or equivalent
intravenous dosing).
• If no response in 72 hours or patient declines clinically intravenous pulse
glucocorticoids.
• In addition, immunosuppressive therapy (eg, cyclophosphamide, rituximab,
intravenous immune globulin [IVIG]) is usually initiated.

20. CHORONIC DISEASE
ILD
• Prevalence of 3 to 9%.
• NSIP most common, LIP,OP/BOOP(patchy filling of alveoli with buds of granulation tissue may extend into respiratory bronchioles, but not
terminal bronchioles) ,UIP,OB(damage of the respiratory epithelium of terminal bronchioles, leading to the formation of chronic inflammatory granulation
tissue, often laid down in a circumferential pattern causing narrowing of the airways).
• PRESENTATION Insidious onset of chronic nonproductive cough, dyspnea, and decreased
exercise tolerance, but some may .be asymptomatic.
• Physical examination typically reveals basilar late inspiratory crackles.
• DIAGNOSIS: 1-Sle-serology, 2-PFT( dec DLCO, restrictive pattern (reductions FVC & TLC), 3-
oxygen desaturation 6MWT),
• Imaging: 4-CXR,5-HRCT strandred NSIP(Diffuse GGOs),UIP(Patchy GGOs,reticular
pattern,honey combing & traction bronchectasis),OP(Patchy areas of air-space consolidation
with or without air bronchograms or GGOs),LIP(Lung cysts,centrilobular nodules,septal
thickening&GGOs),6-BAL,7-ECHO.

25. Managment
 Supportive care –cigarette smoking cessation Supplemental O2 Vaccinations influenza
and Streptococcus pneumoniae and SARS-CoV-2.
 SLE-ILD – For (eg, NSIP, LIP) prednisone 0..5 to: 1 mg/kg per day. Steroid sparing agents:For
patients with mild to moderate ILD, mycophenolate or azathioprine is a reasonable first
choice; cyclophosphamide or rituximab might be preferred for more severe or rapidly
progressive disease.
 Flare of ILD –characterized by hypoxemia and severe impairment on pulmonary function tests
intravenous methylprednisolone 1 g daily for three days, or prednisone 1 to 2 mg/kg/day in
less acute patients) and cyclophosphamide (usually intravenously) or rituximab with transition
to either azathioprine or mycophenolate mofetil after 6 to 12 months.
 OP-Oral prednisone (1 mg/kg per day for a month gradual tapering) ,although the addition of
another immunosuppressive agent is typically necessary.
 Fibrotic ILD –UIP&fibrotic NSIP are fibrotic ILDs. Nintedanib has been approved for use in
progressive fibrosing interstitial lung disease by the FDA. lung transplantation.

26. Vascular Diseases
• Acute reversible hypoxemia:Hypoxemia & widened alveolar–arterial oxygen
gradient, but normal CXR & VQ SCAN. Postulated that there is complement-
activated neutrophil aggregation in the pulmonary vasculature. Improves with
immunosuppressive therapy. antiphospholipid syndrome, considered only after
excluding thromboembolic disease.
• THROMBOEMBOLIC DISEASE with SLE have an increased risk of venous
thromboembolic disease. SLE is associated with a number of risk factors for VTE,
such as membranous nephropathy and the presence of antiphospholipid
antibodies in some patients.

27. PAH
• 1-9%
• Mild PH, medial hypertrophy.With progression,marked intimal fibrous thickening
and eventually plexiform lesions.
• Association: interstitial lung disease, serositis (particularly pericardial effusion),
active smoking, Raynaud phenomenon, and anti-RNP antibodies
• Clinical manifestations — Dyspnea, palpitations, fatigue, and impaired exercise
tolerance, weakness, syncope, edema.Physical Findings loud S2, peripheral
edema, ascites, hepatomegaly. Although Early PH Can Be Asymptomatic.
• Diagnosis: PFTs PFTs in PAH is normal spirometry with a reduced DLCO. The
6MWD is reduced, O2 desaturation ≥5 mmHg is common.
• Echocardiogram –Doppler estimate greater (ePASP) than 35 or tricuspid
regurgitant velocity (TRV) of >2.8 m/s is suggestive of PH.
• Right heart catheterization –mean pulmonary artery pressure (mPAP) of ≥20
mmHg at rest with a mean pulmonary capillary wedge pressure <15 mmHg (to
exclude occult left ventricular dysfunction).

28. TREATMENT
• General measures —Oxygen, Exercise, Diuretics, And Sometimes
Anticoagulation, Pulmonary Rehabilitation.
• Approach to advanced therapy:
• World Health Organization Functional Classification of Pulmonary Arterial
Hypertension

29. • Functional Classes II And III, a vasoreactivity test is used to decide whether to
initiate a three to six month trial of a calcium channel blocker (dihydropyridine
or diltiazem).
• If the vasoreactivity test is negative the next step is usually to select one of the
alternate agents
• Combination therapy – The therapy with the best evidence for benefit in PAH
(class II and III) in general is the combination of ambrisentan (an endothelin-1
antagonist) plus tadalafil (a phosphodiesterase-5 inhibitor), and the benefit
appears to extend to those with CTD-associated PAH.
• Endothelin antagonists –nonselective agents, bosentan and macitentan, selective
agent ambrisentan.
• Phosphodiesterase-5 inhibition – Sildenafil, tadalafil, and vardenafil are orally
administered PDE5 inhibitors that prolong the vasodilatory effect of nitric oxide.
• Prostacyclin pathway agonists –intravenous prostacyclin
(epoprostenol), treprostinil (inhaled, subcutaneous, or intravenous),
inhaled iloprost, and oral selexipag.

30. • Immunosuppressive therapy for selected patients –concomitant PH and
interstitial lung disease (ILD) or pulmonary vasculitis
• One study randomly assigned 34 patients with SLE and moderate pulmonary
hypertension to receive either intravenous cyclophosphamide (0.5 g/m2 per
month) or oral enalapril (10 mg per day) for six months.
• Prognosis — The presence of pulmonary hypertension appears to increase
mortality in patients with SLE

31. PLEURAL DISEASE

35. Shrinking Lungs Syndrome

37. • Although there is a reduction in static lung volumes, the diffusing capacity,
when corrected for alveolar volume, remains normal, thereby
distinguishing respiratory muscle dysfunction from interstitial lung
disease.
• The likely explanation for this is a reduction in the transdiaphragmatic
pressure generated during maximal inspiration, which in turn reduces
static lung compliance, producing the linear atelectasis seen on the chest
radiograph.
• Association with the development of shrinking lung syndrome include:
pleuritis, double-stranded DNA antibody and RNP antibody seropositivity,
serositis, and a prolonged course of SLE.
• Rituximab has been used successfully in patients with respiratory muscle
dysfunction with significant physiologic improvement.

40. Thank you