What are the lung manifestations of Systemic Lupus Erythematosus (SLE)?

From the Guidelines

Systemic Lupus Erythematosus (SLE) patients are at high risk of developing pulmonary manifestations, with pleural involvement being the most common, affecting up to 90% of patients, and interstitial lung disease (ILD) being a rare but severe manifestation, particularly in men and with increasing age and disease stage. The pulmonary manifestations of SLE can affect any component of the lung structure and are associated with a significantly worse prognosis and higher mortality 1.

Key Pulmonary Manifestations

• Pleural involvement: the most common pulmonary manifestation, with prevalence rates varying according to study population and detection methods
• Interstitial lung disease (ILD): rare, particularly as an initial manifestation, and usually less severe than in other connective tissue diseases (CTDs), with a prevalence of 1% to 15% in SLE patients
• Diffuse ILD or chronic pneumonitis: occurs in about 1% to 15% of SLE patients, with nonspecific interstitial pneumonia (NSIP) being the most frequent pattern

Risk Factors for ILD

• Previous episodes of acute lupus pneumonitis
• Raynaud phenomenon
• Gastroesophageal reflux disease
• Tachypnea
• Abnormal nail-fold capillaries
• Elevated CRP
• Anti-Sm and anti-U1-RNP seropositivity

Management and Treatment

The management and treatment of SLE-related pulmonary manifestations depend on the specific manifestation and severity of the disease. Early detection and treatment of ILD are crucial to improve prognosis and reduce mortality. Treatment options may include corticosteroids, immunosuppressants, and vasodilators, depending on the specific manifestation and severity of the disease 1.

From the Research

SLE and Lung Manifestations

• Systemic lupus erythematosus (SLE) is a complex autoimmune disease with multiorgan manifestations, including pleuropulmonary involvement, which affects 20-90% of patients 2, 3.
• The pulmonary manifestations of SLE are wide-ranging and debilitating, including disorders of the lung parenchyma, pleura, and pulmonary vasculature 2.
• The most common lung manifestations of SLE include:
  • Pleuritis and pleural effusion
  • Interstitial lung disease (ILD)
  • Acute lupus pneumonitis
  • Shrinking lung syndrome
  • Pulmonary arterial hypertension (PAH)
  • Pulmonary embolism
  • Diffuse alveolar hemorrhage (DAH) 3, 4, 5
• The diagnosis of lung involvement in SLE is based on a combination of clinical presentation, imaging studies (such as chest X-ray and high-resolution CT), pulmonary function tests, and laboratory tests 3, 6.
• Treatment of lung manifestations in SLE is mainly based on glucocorticoids and immunosuppressants, with a variable response according to the primary pathologic process 6.
• The prognosis of lung involvement in SLE varies depending on the specific manifestation, with some conditions (such as DAH) having a high mortality rate 3.