Corticosteroid in Combination with Leflunomide and Mesenchymal Stem Cells for Treatment of Pediatric Idiopathic Pulmonary Hemosiderosis

Xu, Lü-Hong; Ou, Rongqiong; Bao-jing, WU; Wang, Haiyan; Fang, Jian-Pei; Tan, Weiping

doi:10.1093/tropej/fmx002

Cited by 11 publications

(10 citation statements)

References 26 publications

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“…The combination of novel and traditional therapies reduces drugs dose and the side effects of corticosteroids. A clinical study has proved the safety of this new therapy 46 . However, further studies are required for its application in silicosis.…”

Section: Discussionmentioning

confidence: 97%

Mesenchymal stem cells ameliorate silica‐induced pulmonary fibrosis by inhibition of inflammation and epithelial‐mesenchymal transition

Wei

Zhao

Guo

et al. 2021

J Cellular Molecular Medi

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Silicosis is a devastating occupational disease caused by long‐term inhalation of silica particles, inducing irreversible lung damage and affecting lung function, without effective treatment. Mesenchymal stem cells (MSCs) are a heterogeneous subset of adult stem cells that exhibit excellent self‐renewal capacity, multi‐lineage differentiation potential and immunomodulatory properties. The aim of this study was to explore the effect of bone marrow‐derived mesenchymal stem cells (BMSCs) in a silica‐induced rat model of pulmonary fibrosis. The rats were treated with BMSCs on days 14, 28 and 42 after perfusion with silica. Histological examination and hydroxyproline assays showed that BMSCs alleviated silica‐induced pulmonary fibrosis in rats. Results from ELISA and qRT‐PCR indicated that BMSCs inhibited the expression of inflammatory cytokines TNF‐α, IL‐1β and IL‐6 in lung tissues and bronchoalveolar lavage fluid of rats exposed to silica particles. We also performed qRT‐PCR, Western blot and immunohistochemistry to examine epithelial‐mesenchymal transition (EMT)–related indicators and demonstrated that BMSCs up‐regulate E‐cadherin and down‐regulate vimentin and extracellular matrix (ECM) components such as fibronectin and collagen Ⅰ. Additionally, BMSCs inhibited the silica‐induced increase in TGF‐β1, p‐Smad2 and p‐Smad3 and decrease in Smad7. These results suggested that BMSCs can inhibit inflammation and reverse EMT through the inhibition of the TGF‐β/Smad signalling pathway to exhibit an anti‐fibrotic effect in the rat silicosis model. Our study provides a new and meaningful perspective for silicosis treatment strategies.

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Section: Discussionmentioning

confidence: 97%

Mesenchymal stem cells ameliorate silica‐induced pulmonary fibrosis by inhibition of inflammation and epithelial‐mesenchymal transition

Wei

Zhao

Guo

et al. 2021

J Cellular Molecular Medi

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“…Azathioprine allowed to reduce gradually the doses of methylprednisolone. Mesenchymal stem cells (25) can also be used as an additional therapy option. As we had no experience in applying this method, we did not offer it to the patient.…”

Section: Discussionmentioning

confidence: 99%

Cryobiopsy in the diagnosis of idiopathic pulmonary hemosiderosis: a case report

et al. 2019

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“…Anemia (91.6%) and dyspnea (85.5%) are more common presenting symptoms in adults than hemoptysis 3 . Among pediatric patients the prevalence of anemia and dyspnea ranges from 64%−100% (average 92.2%) and 15%−85% (average 45%), respectively 18,23,26,27,39,40,46–51 . Cough is more common among children than adults (65.6% vs. 26.2%).…”

Section: Clinical Aspects In Iphmentioning

confidence: 99%

“…Chest pain is present in a minority of adult patients (10.7%) 3 . Nearly half of adult patients have systemic symptoms, 3 likely comparable to pediatric patients 39,47,49,52 …”

Section: Clinical Aspects In Iphmentioning

confidence: 99%

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Updates in idiopathic pulmonary hemosiderosis in 2022: A state of the art review

Saha

Aiman

Chong

et al. 2022

Pediatric Pulmonology

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This manuscript reports the recent advances in idiopathic pulmonary hemosiderosis (IPH), a rare cause of diffuse alveolar hemorrhage in children and adults. This narrative review of the literature summarizes different aspects of IPH, including proposed pathogenesis, patient demographics, clinical and radiological characteristics, treatment, and prognosis. Additionally, the association between Celiac Disease (CD) and IPH is carefully evaluated. IPH is a frequently misdiagnosed disease. The delay in the diagnosis of IPH is often significant but fortunately, appears to have decreased in recent years. IPH in adults and children have distinct demographic preferences. The autoantibodies are common in IPH but with a definite difference between the adult and pediatric populations. The definitive diagnosis of IPH requires lung biopsy and careful exclusion of all competing diagnoses, even with lung biopsy showing bland pulmonary hemorrhage. The presence of nonspecific inflammatory cells or lymphoid aggregates may suggest a secondary immunologic phenomenon and needs careful evaluation and follow-up. A substantial number of patients suffer from coexisting CD, also known as Lane−Hamilton syndrome (LHS), and all patients with IPH need to be evaluated for LHS by serology. Although strict gluten free diet can manage the majority of patients with LHS, other patients generally require immunosuppressive therapy. The corticosteroids are the backbone of IPH therapy. Recently utilized experimental treatment options include mesenchymal stem cell transplant, liposteroid and bronchial artery embolization. The immunosuppression should be adjusted to achieve optimal disease control. Patients may progress to endstage lung disease despite all measures, and lung transplantation may be the only viable option.

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Corticosteroid in Combination with Leflunomide and Mesenchymal Stem Cells for Treatment of Pediatric Idiopathic Pulmonary Hemosiderosis

Abstract: Treatment with corticosteroid, leflunomide and MSCs for pediatric IPH was safe and effective.

Cited by 11 publications

References 26 publications

Mesenchymal stem cells ameliorate silica‐induced pulmonary fibrosis by inhibition of inflammation and epithelial‐mesenchymal transition

Mesenchymal stem cells ameliorate silica‐induced pulmonary fibrosis by inhibition of inflammation and epithelial‐mesenchymal transition

Cryobiopsy in the diagnosis of idiopathic pulmonary hemosiderosis: a case report

Updates in idiopathic pulmonary hemosiderosis in 2022: A state of the art review

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