Patient with Idiopathic Pulmonary Fibrosis with Consequent Initial Findings of Sjögren’s Syndrome: Case Report

  • Pauls Bērziņš (Speaker)
  • Skuja, I. (Co-author)

Activity: Talk or presentation types › Poster presentation

Description

Keywords: idiopathic pulmonary fibrosis, primary Sjögren's syndrome. Idiopathic pulmonary fibrosis (IPF) is a type of interstitial lung disease (ILD). It's imperative to diagnose IPF as early as possible. Lung fibrosis is irreversible for there are no pathogenetic treatment yet.Most common ILD pattern is usual interstitial pneumonia (UIP) and is often associated with autoimmunity, like primary Sjögren's syndrome (pSS). ILD is usually late complication of pSS complication, but in 10% to 51% of cases it can initiate years after the first presentation of ILD. This case shows the importance of a multidisciplinary approach in early diagnosis for IPF with late onset pSS. A 56y.o. male, after a second pneumonia in that year, visited a general practitioner with complaint of light dyspnea. Inspiratory crackles on auscultation. Chest x-ray showed no infiltrative changes as two months earlier, but peripheral small foci changes. A lung CT visualized hilar and peripheral reticulation. ILD could not be ruled out. The CT was forwarded to Switzerland, Basel University clinic for a second look - consensus for an UIP was reached.Video assisted thoracoscopy with wedge biopsies histologically fitted ATS/ERS criteria for UIP. DLCO was just below 70%. The multidisciplinary council decided for a treatment for IPF with Pirfenidone. A year later the patient subjectively improved, noting oral and ocular dryness. Elevated anti-SSA and salivary gland ultrasonography suggested diagnosis of pSS, On follow up DLCO fell to 49% and diffuse progression of fibrosis was found on CT scan. Pirfenidone therapy was continued despite its low efficacy and lack of alternatives. Treatment for Sjögren-associated IPF is empiric as no studies are conclusive of yet.The role of antifibrotics in such cases remains unclear.  A better understanding of molecular mechanisms involved in the pathogenesis of ILD and pSS should facilitate the development of novel pathogenetic therapies. . .. .. .
Period29 Mar 2023
Event titleRSU International Research Conference 2023: Knowledge for Use in Practice
Event typeConference
OrganiserRīga Stradiņš University
LocationRiga, LatviaShow on map
Degree of RecognitionInternational

Related content