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Systemic sclerosis is a rare autoimmune disorder often complicated by interstitial lung disease, sometimes as the initial or sole manifestation, which can delay diagnosis when classic features are absent. A man in his 70s presented with hypoxemic respiratory failure and fibrotic CT findings; bronchoalveolar lavage supported alveolitis, immunology confirmed systemic sclerosis sinescleroderma, and treatment with immunosuppression plus nintedanib stabilized disease before eventual deterioration.",{"@graph":69,"@context":123},[70,84,106],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/a-case-report-on-a-challenge-from-diagnosis-to-treatment-interstitial-lung-disease-as-the-first-manifestation-of-systemic-sclerosis-sine-scleroderma/454962/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/a-case-report-on-a-challenge-from-diagnosis-to-treatment-interstitial-lung-disease-as-the-first-manifestation-of-systemic-sclerosis-sine-scleroderma/454962.png","ImageObject",300,407,{"name":92,"@type":93},"McGucket","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-30","2026-09-30",true,{"@type":102,"interactionType":103,"userInteractionCount":105},"InteractionCounter",{"@type":104},"ViewAction",11,{"@type":107,"mainEntity":108},"FAQPage",[109,115,119],{"name":110,"@type":111,"acceptedAnswer":112},"Why is determining the etiology of interstitial lung disease important?","Question",{"text":113,"@type":114},"An accurate etiology guides specific immunomodulatory therapy and strongly affects prognosis.","Answer",{"name":116,"@type":111,"acceptedAnswer":117},"What made diagnosis challenging in this case?",{"text":118,"@type":114},"Systemic sclerosis sine scleroderma was suspected despite the absence of overt scleroderma or vasculopathy, meaning typical clinical clues were initially lacking.",{"name":120,"@type":111,"acceptedAnswer":121},"What treatment was used and what was the outcome?",{"text":122,"@type":114},"The patient received corticosteroids and antibiotics initially, later started mycophenolate mofetil and combined it with nintedanib, achieving early stabilization; the patient ultimately died two years after presentation due to progressive, complicated pulmonary disease.","https://schema.org",{"og:url":83,"og:type":125,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":127,"canonical":83},"index,follow",{"doc_id":129,"site_id":62},454962,1790954209,{"code":4,"msg":5,"data":132},{"doc_id":129,"user_id":133,"nickname":92,"user_avatar":134,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":135,"file_id":136,"file_url":137,"file_type":138,"file_size":139,"view_count":105,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":34,"language":140,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":141,"faqs":142,"seo_title":143,"seo_description":67,"update_tm":144,"read_time":145,"preview_page_count":4},1236954412713,"https://us-avatar.wpscdn.com/davatar_29158cc5080c5b710cf443261637dec0","Review began 11/12/2025  \nReview ended 11/30/2025  \nPublished 12/08/2025  \n© Copyright 2025  \nViana et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 4.0. , which permits unrestricted use , distribution , and reproduction in any medium , provided the original author and source are credited.  \nDOI: 10.7759/cureus.98714  \nOpen Access Case Report  \nA Case Report on a Challenge From Diagnosis to Treatment: Interstitial Lung Disease As the First Manifestation of Systemic Sclerosis Sine Scleroderma  \nSusana Viana 1, Andreia Sá Lima 1, Verónica Guiomar 1, Inês Neves 2 , Rui Môço 1  \n1. Internal Medicine Department, Pedro Hispano Hospital, Matosinhos Local Health Unit, Matosinhos, PRT 2. Pulmonology Department, Pedro Hispano Hospital, Matosinhos Local Health Unit, Matosinhos, PRT  \nCorresponding author: Susana Viana, [susana.viana@ulsm.min-saude.pt](susana.viana@ulsm.min-saude.pt)  \nAbstract  \nInterstitial lung disease comprises a heterogeneous group of conditions that may progress to pulmonary fibrosis. Establishing an accurate etiology is critical, as it dictates specific immunomodulatory therapy and profoundly influences prognosis. Systemic sclerosis is a rare autoimmune condition frequently complicated by interstitial lung disease, either during its course or, less commonly, as its initial or sole manifestation. Diagnosing systemic sclerosis in the absence of typical scleroderma features can be particularly challenging.  \nWe present a rare case of pulmonary involvement as the initial manifestation of systemic sclerosis sinescleroderma. A man in his 70s presented to the Emergency Department due to gradually increasing dyspnea over the preceding two months. Upon admission, he was found to have hypoxemic respiratory failure. Thoracic computed tomography showed a fibrotic pulmonary pattern characterized by peripheral reticulation, honeycombing, and traction bronchiectasis. Bronchoscopy with bronchoalveolar lavage showed intense neutrophilic and mild eosinophilic alveolitis, along with increased alveolar macrophages. Initial clinical improvement was observed following treatment with corticosteroids and antibiotics, as infection could not be excluded. Following discharge and corticosteroid tapering, the patient 's clinical condition worsened. Further immunological evaluation revealed positive antinuclear, anti-centromere, and RNA polymerase III antibodies. Pulmonary function tests demonstrated a mild restrictive pattern and a severely reduced diffusing capacity for carbon monoxide. In the absence of overt scleroderma or vasculopathy, a diagnosis of interstitial lung disease associated with systemic sclerosis sine scleroderma was established. Treatment with mycophenolate mofetil was initiated, and later combined with nintedanib, resulting in initial disease stabilization. Unfortunately, the patient succumbed to progressive and complicated pulmonary disease two years after initial presentation.  \nThis case underscores the importance of maintaining a high index of suspicion when evaluating patients with interstitial lung disease, even in the absence of cutaneous manifestations of systemic sclerosis.  \nCategories: Internal Medicine, Rheumatology, Pulmonology  \nKeywords: interstitial pulmonary fibrosis, mycophenolate mofetil (mmf), nintedanib, progressive interstitial lung  \ndisease, systemic sclerosis interstitial lung disease(ssc-ild), systemic sclerosis sine scleroderma (ssssc)  \nIntroduction  \nInterstitial lung disease (ILD) refers to a broad and heterogeneous group of diffuse parenchymal lung  \ndisorders that may progress to pulmonary fibrosis [1] . Although idiopathic pulmonary fibrosis is the most common form, approximately 15% of ILD cases are associated with an underlying connective tissue disease (CTD) [2,3] . Among CTDs, systemic sclerosis (SSc) is particularly relevant, as ILD represents one of its most common and severe organ manifestations, affecting up to 80-90%","cbCaidBqP8wQJ1Zr","https://ap.wps.com/l/cbCaidBqP8wQJ1Zr","pdf",1979590,"English","# Abstract\n# Introduction\n# Case Presentation","[{\"question\":\"Why is determining the etiology of interstitial lung disease important?\",\"answer\":\"An accurate etiology guides specific immunomodulatory therapy and strongly affects prognosis.\"},{\"question\":\"What made diagnosis challenging in this case?\",\"answer\":\"Systemic sclerosis sine scleroderma was suspected despite the absence of overt scleroderma or vasculopathy, meaning typical clinical clues were initially lacking.\"},{\"question\":\"What treatment was used and what was the outcome?\",\"answer\":\"The patient received corticosteroids and antibiotics initially, later started mycophenolate mofetil and combined it with nintedanib, achieving early stabilization; the patient ultimately died two years after presentation due to progressive, complicated pulmonary disease.\"}]","A Case Report on a Challenge From Diagnosis to Treatment - Interstitial Lung Disease as the First Manifestation of Systemic Sclerosis Sine Scleroderma | PDF",1790741790,18]