{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,21]],"date-time":"2026-03-21T21:54:02Z","timestamp":1774130042732,"version":"3.50.1"},"reference-count":22,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2021,10,4]],"date-time":"2021-10-04T00:00:00Z","timestamp":1633305600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>Interstitial lung disease (ILD) as an initial manifestation of lupus is rare, especially in young children. Here, we report a case of a 3-year-old boy who presented with fever, shortness of breath, and facial erythema. Clinical examination suggested a diagnosis of active systemic lupus erythematosus (SLE) with butterfly rash, anemia, positive antinuclear antibody, positive anti-double-stranded DNA, and hypocomplementemia. On retrospective review of the patient\u2019s records, multiple chest computed tomography (CT) images showed non-specific interstitial pneumonia + organizing pneumonia pattern, with no further autoimmune work-up during the visit to a respiratory department. In our opinion, persistent interstitial pneumonia may be a clue to connective tissue disease. The patient received steroid treatment for 1 year, and the radiological and immunological resolution was noted. However, he still suffered from cough and dyspnea. After a 1-year follow-up, he was hospitalized again for SLE relapse. While continuing corticosteroid therapy, the patient was given combination therapy consisting of cyclosporine A (CsA) and monthly-pulse cyclophosphamide for 6 months, and decreased proteinuria was noted. However, the patient\u2019s respiratory symptoms and pulmonary radiologic findings did not improve significantly. With continued steroid therapy, the patient was started on a daily regimen of CsA and pirfenidone. Both drugs were sufficiently effective to allow gradual reduction of steroid dosage. After 2 years of treatment, marked improvements in symptoms, pulmonary function and chest CT images were observed. Our experience with this case emphasizes that prompt work-up for connective tissue disease (CTD) should be considered in young children with ILD, and pirfenidone might be a useful add-on therapy with immunosuppressive agents for refractory CTD-ILD in pediatric patients. Nevertheless, further clinical trials including larger numbers of patients need to assess the efficiency and safety of this combination therapy for refractory CTD-ILD.<\/jats:p>","DOI":"10.3389\/fimmu.2021.708463","type":"journal-article","created":{"date-parts":[[2021,10,4]],"date-time":"2021-10-04T07:27:54Z","timestamp":1633332474000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":10,"title":["Case Report: Successful Treatment of Refractory Interstitial Lung Disease With Cyclosporine A and Pirfenidone in a Child With SLE"],"prefix":"10.3389","volume":"12","author":[{"given":"Linxia","family":"Deng","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yaxian","family":"Chen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiufen","family":"Hu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jianhua","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yu","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2021,10,4]]},"reference":[{"key":"B1","volume-title":"Pulmonary Pathophysiology: The Essentials","author":"West","year":"2008"},{"key":"B2","article-title":"Interstitial Lung Disease as Initial Manifestation of Systemic Lupus Erythematosus","volume":"11","author":"Kumar","year":"2013","journal-title":"J Nepal Health Res Council"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1177\/0961203320967439","article-title":"Bronchiolitis Obliterans Organizing Pneumonia as the Pulmonary Manifestation of Lupus: A Review of Three Cases","volume":"30","author":"Baisya","year":"2021","journal-title":"Lupus"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.20224","article-title":"Bronchiolitis Obliterans Organizing Pneumonia as an Initial Manifestation in Systemic Lupus Erythematosus","volume":"40","author":"Takada","year":"2005","journal-title":"Pediatr Pulmonol"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1136\/thorax.55.2.159","article-title":"3rd. 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