Systemic Lupus Erythematosus-related ILD
DIAGNOSTICS
SYMPTOMS
SLE
Malar rash, joint pain, and extreme fatigue are common presenting symptoms among men and women.8
Some presenting symptoms are more commonly associated with a specific gender:9-11
More Common in Women
- Raynaud's phenomenon
- Photosensitivity
- Mucosal ulceration
- Malar rash
More Common in Men
- Renal disease
- Skin manifestations
- Cytopenias
- Serositis
- Neurologic involvement
- Thrombosis
- Cardiovascular disease
- Hypertension
- Vasculitis
SLE-ILD
- Symptomatic ILD typically occurs in ~3%-8% of patients with SLE1
- Approximately 1 in 3 patients has interstitial abnormalities on HRCT12
- Patients with SLE who have multiple end-organ manifestations are more likely to have ILD12
Common SLE-ILD Presenting Symptoms:13
- Acute dyspnea
- Cough
- Fever
- Pleuritic pain
- Occasionally pulmonary hemorrhage
ILD in SLE patients can have 2 differential diagnoses: alveolar hemorrhage (rare), and lupus pneumonitis, which occurs in 1%-10% of SLE patients.1
DIAGNOSIS FOR SLE1 | DIAGNOSIS FOR SLE-ILD1,2,12,14 | |||
---|---|---|---|---|
Established diagnosis of SLE OR medical history + thorough physical exam + serologies (Antinuclear Antibody (ANA), anti-dsDNA (double stranded DNA), anti-Sm (autoantibodies to the nuclear Sm (Smith) proteins). |
PFTs | HRCT | Lung Biopsy | BAL |
Restrictive defect (or mixed, if other comorbidities present); reduced diffusing capacity of the lung for carbon monoxide (DLCO) | Nonspecific Interstitial Pneumonia (NSIP), Organizing Pneumonia (OP), Lymphoid Interstitial Pneumonia (LIP), Usual Interstitial Pneumonia (UIP) - very rare |
Rarely used | Primarily for exclusion of other causes |