Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome

et al. 2021

Front. Pediatr.

Background: Symptomatic pulmonary involvement in systemic lupus erythematosus (SLE) seems not uncommon in children. However, there are few data on the characteristics and laboratory parameters of SLE patients with pulmonary involvement.Methods: This was a hospital-based study involving 111 SLE patients from 1 January 2012 to 31 December 2016. The demographic, clinical, and laboratory data of the patients were prospectively collected. They were followed as outpatients until December 2019. Clinical characteristics and laboratory parameters of patients with and without pulmonary involvement were compared.Results: Of the 111 patients with SLE, we identified 18 patients (16.2%) with pulmonary involvement. The most common HRCT findings were ground glass opacity, interlobular septal thickening, bilateral diffuse infiltrates, and pleurisy/pleural effusion (55.6, 50, 50, and 44.4%, respectively). SLE patients with pulmonary involvement tended to have a longer disease duration (14 [12–24.5] vs. 5 [2–9] months, P < 0.01). We also observed a significant association between the presence of anti-Sm antibody, ANCA, Anti-RNP and the presence of pulmonary involvement of SLE (all P < 0.001).Conclusions: Lung involvement was frequent in SLE patients from Southeast China. Patients with a longer duration of symptoms before SLE diagnosis tended to have pulmonary involvement. When children with SLE are found to have anti-RNP antibody and positive ANCA, it should be alert to the occurrence of pulmonary involvement.

Section: Discussionsupporting

confidence: 92%

Pulmonary Involvement in Children With Systemic Lupus Erythematosus

Dai

et al. 2021

Front. Pediatr.

“…Results on SLE-associated DAH from previous studies from 2009 to 2019 are summarized in Table 4. Prevalence in our cohort was close to the ones observed in other studies, 1,2 and similar in childhood-onset SLE, 3,4 but higher than the one reported by us 9 years ago and by some other older series 5,6 (Table 4). This may be explained by a lack of recognition of DAH as SLE manifestation, especially when DAH occurred in the early course and systematic screening was not done.…”

Section: Discussionsupporting

confidence: 91%

“…In a more recent report, there were 13/19 SLE patients who had their DAH occurring within the first year after SLE diagnosis. 3 Of note, even though DAH tends to occur early, it still can present as late as 3 years after SLE diagnosis. Thus, DAH should be suspected in all SLE patients with suspicious symptoms or signs, especially in the first 3 years after SLE diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Systemic lupus erythematosus–associated diffuse alveolar hemorrhage: a single-center, matched case–control study in China

Sun

Zhou

et al. 2020

Lupus

Background This study described clinical characteristics and outcome in systemic lupus erythematosus (SLE) patients with diffuse alveolar hemorrhage (DAH), and investigated risk factors and prognostic factors for DAH. Methods We conducted a retrospective nested case–control analysis in a single-center cohort. We enrolled 94 SLE patients with DAH. For each case of DAH, two age-, sex-, and SLE courses–matched controls were randomly selected from our cohort. All patients were enrolled between 2004 and 2019 and were followed until death, end of registration with the physician’s practice, or end of January 2019. We estimated the risk factors for DAH and prognostic factors for mortality using multivariate analysis. Results We included 4744 patients diagnosed with SLE, with 94 cases of DAH, for an incidence rate of 2.0%. DAH may occur in any stage of SLE but mostly in the early phase of disease course. Lupus nephritis (LN) was the most common concomitant involvement at DAH diagnosis. By multivariate analysis, LN, anti-SSA positivity, thrombocytopenia and elevated C-reactive protein (CRP) were significantly associated with DAH in SLE patients. All-cause mortality was increased in SLE with DAH compared with SLE without DAH (adjusted hazard ratio 6.0, 95% confidence interval 2.8–13.0, p < 0.0001). Intravenous cyclophosphamide (CTX) showed an increased tendency for better survival in DAH after adjusting for Systemic Lupus Erythematosus Disease Activity Index 2000, acute kidney injury and mechanical ventilation. Conclusions LN, anti-SSA positivity, thrombocytopenia and elevated CRP were independent risk factors of DAH in lupus patients. Due to a high early death rate of DAH and little long-term damage, DAH patients may benefit from early diagnosis and intensive treatment, and CTX-based therapy can be a preferential choice.

Section: Discussionsupporting

confidence: 93%

Pulmonary manifestations in children with systemic lupus erythematosus

Jiang

Dai

et al. 2020

Preprint

Background Symptomatic pulmonary involvement in systemic lupus erythematosus (SLE) is commonly reported in children. However, few studies have assessed risk factors that predict pulmonary involvement in SLE.Methods This was a seven-year retrospective study involving 111 SLE patients. The demographic, clinical and laboratory data of the patients were collected. Logistic regression analysis was performed to identify different clinical characteristics and laboratory parameters associated with presence of high resolution computerized tomography(HRCT) chest abnormalities.Results Of the 111 patients with SLE, we identified 18 patients (16.2%) with pulmonary involvement. The most common HRCT findings were ground glass opacity, interlobular septal thickening, bilateral diffuse infiltrates and pleurisy/pleural effusion (55.6%, 50%, 50%, and 44.4%, respectively). The variables independently associated with pulmonary involvement were anti- ribonucleoprotein (RNP) antibody (OR:7.9; 95% CI: 1.1–54.6) and anti-neutrophil cytoplasmic antibodies (ANCA, OR: 73.6; 95% CI: 2.9-1894.7).Conclusions Lung involvement was frequent in SLE patients from Southeast China. Anti-RNP antibody and ANCA were significantly associated with abnormal HRCT findings.