TY - JOUR
T1 - Systemic lupus erythematosus-associated diffuse alveolar haemorrhage
T2 - a single-centre experience in Han Chinese patients
AU - Wang, C. R.
AU - Liu, M. F.
AU - Weng, C. T.
AU - Lin, W. C.
AU - Li, W. T.
AU - Tsai, H. W.
N1 - Funding Information:
The authors are indebted to the doctors and nurses involved in the management of the reported patients at the Internal Medicine Department of NCKUH.
Publisher Copyright:
© 2018, © 2018 Informa Healthcare on license from Scandinavian Rheumatology Research Foundation.
PY - 2018/9/3
Y1 - 2018/9/3
N2 - Objective: Despite aggressive therapeutic regimens, diffuse alveolar haemorrhage (DAH) is still associated with a high mortality rate in systemic lupus erythematosus (SLE). This study was carried out in patients with SLE-associated DAH with a focus on their therapeutic modality. Method: A retrospective review was performed in 839 Han Chinese lupus patients hospitalized for their DAH manifestation from May 2006 to December 2016. Results: There were 24 episodes in 17 cases (2.0% incidence), 15 females and two males aged 19–67 years (mean ± sd 38.2 ± 15.1 years). High disease activity [Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) 12–31, 19.8 ± 5.6] was found at the onset of DAH. All patients were treated with high-dose corticosteroid, followed by pulse methylprednisolone (70.6%), plasmapheresis (41.2%), pulse cyclophosphamide (35.3%), and rituximab (23.5%). Six patients (35.3%), including three with extracorporeal membrane oxygenation, died owing to acute respiratory failure. All patients receiving rituximab treatment survived with a follow-up period of 12–58 months (40.8 ± 21.1 months), and no further relapse was noted in three cases with a history of recurrent DAH episodes. In addition, there was a significant decrease in their lupus activity (SLEDAI-2K 21.5 ± 6.0 to 6.3 ± 1.7, p = 0.0286). Conclusion: In this single-centre series with SLE-associated DAH in Han Chinese patients, a beneficial effect of rituximab therapy was observed.
AB - Objective: Despite aggressive therapeutic regimens, diffuse alveolar haemorrhage (DAH) is still associated with a high mortality rate in systemic lupus erythematosus (SLE). This study was carried out in patients with SLE-associated DAH with a focus on their therapeutic modality. Method: A retrospective review was performed in 839 Han Chinese lupus patients hospitalized for their DAH manifestation from May 2006 to December 2016. Results: There were 24 episodes in 17 cases (2.0% incidence), 15 females and two males aged 19–67 years (mean ± sd 38.2 ± 15.1 years). High disease activity [Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) 12–31, 19.8 ± 5.6] was found at the onset of DAH. All patients were treated with high-dose corticosteroid, followed by pulse methylprednisolone (70.6%), plasmapheresis (41.2%), pulse cyclophosphamide (35.3%), and rituximab (23.5%). Six patients (35.3%), including three with extracorporeal membrane oxygenation, died owing to acute respiratory failure. All patients receiving rituximab treatment survived with a follow-up period of 12–58 months (40.8 ± 21.1 months), and no further relapse was noted in three cases with a history of recurrent DAH episodes. In addition, there was a significant decrease in their lupus activity (SLEDAI-2K 21.5 ± 6.0 to 6.3 ± 1.7, p = 0.0286). Conclusion: In this single-centre series with SLE-associated DAH in Han Chinese patients, a beneficial effect of rituximab therapy was observed.
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U2 - 10.1080/03009742.2017.1420817
DO - 10.1080/03009742.2017.1420817
M3 - Article
C2 - 29916287
AN - SCOPUS:85048757851
SN - 0300-9742
VL - 47
SP - 392
EP - 399
JO - Scandinavian Journal of Rheumatology
JF - Scandinavian Journal of Rheumatology
IS - 5
ER -