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Published on: June 16, 2020
A 5-year follow-up of pulmonary function tests in childhood-onset systemic lupus erythematosus: a single-center
A J Altchek1, L N Moorthy1, M Ramagopal1
1Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, USA.
Insights
Pulmonary function test (PFT) abnormalities are common in childhood-onset systemic lupus erythematosus (cSLE), often presenting as restrictive lung disease or impaired diffusing capacity. Early detection through PFTs is crucial for managing respiratory complications in these patients.
Area of Science:
- Pediatric Rheumatology
- Pulmonology
- Systemic Lupus Erythematosus
Background:
- Pulmonary involvement significantly impacts morbidity and mortality in childhood-onset systemic lupus erythematosus (cSLE).
- Respiratory symptoms may be absent, yet pulmonary function test (PFT) abnormalities can still be present.
Purpose of the Study:
- To characterize PFT abnormalities in patients diagnosed with cSLE.
- To identify common patterns of lung dysfunction in this pediatric population.
Main Methods:
- Retrospective review of 42 patients with cSLE aged 6 years and older.
- Data collected between July 2015 and July 2020.
- Analysis of PFT results to identify abnormalities.
Main Results:
- 10 out of 42 patients (23.8%) exhibited abnormal PFTs.
- Common abnormalities included restrictive lung disease and impaired diffusing capacity.
- Patients with restrictive patterns showed reduced total lung capacity (TLC).
Conclusions:
- The primary PFT abnormalities in cSLE are restrictive lung disease and altered diffusing capacity.
- These findings underscore the importance of PFT screening in cSLE patients.
- Early identification of pulmonary dysfunction aids in comprehensive patient management.
Significance:
Pulmonary involvement in childhood-onset systemic lupus erythematosus (cSLE), contributes to significant morbidity and mortality. Manifestations include chronic interstitial pneumonitis, pneumonia, pleuritis, alveolar hemorrhage, and shrinking lung syndrome. However, many patients can be asymptomatic from a respiratory standpoint and still have pulmonary function test (PFT) abnormalities. Our aim is to describe PFT abnormalities in patients with cSLE.
Methods:
We completed a retrospective review of 42 patients with cSLE followed at our center. These patients were at least 6 years old (so they could complete PFTs). We collected data from July 2015 to July 2020.
Results:
Out of the 42 patients, 10 (23.8%) had abnormal PFTs. These 10 patients had a mean age at diagnosis of 13 ± 2.9 years. Nine were female. One-fifth (20%) self-identified as Hispanic, 20% as Asian, 10% as Black or African American, and the remaining 50% as "Other." Of the 10, 3 had restrictive disease only, 3 with diffusion impairment only, and 4 with both restrictive lung disease and diffusion impairment. Patients with restrictive patterns had a mean total lung capacity (TLC) of 72.5 ± 5.8 throughout the study period. The average diffusing capacity for carbon monoxide corrected for hemoglobin (DsbHb) among patients with diffusion limitation during the study period was 64.8 ± 8.3.
Conclusions:
The most common PFT abnormalities seen in patients with cSLE are alterations in diffusing capacity as well as restrictive lung disease.
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