Treatment Approach for SLE with Lung Involvement

Initial Assessment and Treatment Strategy

The European League Against Rheumatism recommends treatment goals that aim at remission or low disease activity and prevention of flares in all organs, maintained with the lowest possible dose of glucocorticoids 3
The American College of Rheumatology suggests hydroxychloroquine is recommended for all SLE patients at a dose not exceeding 5 mg/kg/real body weight as the backbone of treatment 3
For acute severe or organ-threatening lung manifestations, pulses of intravenous methylprednisolone provide immediate therapeutic effect 3, 4

The American College of Rheumatology conditionally recommends mycophenolate, azathioprine, rituximab, and cyclophosphamide as first-line treatment options for patients with SLE and interstitial lung disease 1, 2
Cyclophosphamide is recommended for severe organ-threatening disease or as "rescue" therapy 1, 2, 3

For SLE-ILD, first-line therapy should include mycophenolate, azathioprine, rituximab, or cyclophosphamide 1, 2
For patients with SLE-ILD progression despite first-line therapy, consider switching to or adding mycophenolate, rituximab, or cyclophosphamide 2
For rapidly progressive ILD, pulse intravenous methylprednisolone is recommended as first-line treatment 2
For confirmed or suspected MDA-5 RP-ILD, triple combination therapy is conditionally recommended over monotherapy 2

For patients with inadequate response to standard therapy, rituximab may be considered in organ-threatening, refractory disease 3

Regular assessment of disease activity using validated indices is recommended 5
Monitor complement levels and anti-dsDNA antibodies to assess disease activity 5
Screen for infections, which are a common complication of immunosuppressive therapy 5, 6
Ophthalmological screening for patients on hydroxychloroquine should be performed at baseline, after 5 years, and yearly thereafter 3

First-line therapy includes mycophenolate, azathioprine, rituximab, or cyclophosphamide with appropriate glucocorticoid dosing 1, 2
If inadequate response, consider switching immunosuppressants or adding a second agent 2
Consider adding nintedanib for patients with progressive disease 2
For severe, refractory disease, cyclophosphamide as "rescue" therapy may be considered 3
Consider rituximab if not previously used for severe, refractory disease 3