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RESEARCH PRODUCT

Interstitial lung disease in systemic sclerosis: current and future treatment.

Vasiliki LiakouliPaola CiprianiFrancesco CarubbiRoberto GiacomelliPiero RuscittiPaola Di BenedettoOnorina BerardicurtiSalvatore Di BartolomeoGiuliana GugginoFrancesco CicciaGiovanni Triolo

subject

0301 basic medicinemedicine.medical_specialtyPathologyFibrosiImmunologyInterstitial lung diseaseSclerodermaPulmonary function testingSclerodermaPathogenesis03 medical and health sciencesSystemic sclerosi0302 clinical medicineRheumatologyFibrosisPredictive Value of TestsRisk FactorsInternal medicineCase fatality ratemedicineAnimalsHumansImmunology and AllergyMolecular Targeted Therapyskin and connective tissue diseasesLungCause of death030203 arthritis & rheumatologyScleroderma Systemicintegumentary systembusiness.industryInterstitial lung diseaseHematopoietic Stem Cell Transplantationrespiratory systemmedicine.diseaseRheumatologyRespiratory Function Testsrespiratory tract diseasesTreatmentSettore MED/16 - Reumatologia030104 developmental biologyEarly DiagnosisTreatment OutcomebusinessLung Diseases InterstitialTomography X-Ray ComputedImmunosuppressive AgentsLung Transplantation

description

Systemic sclerosis (SSc) has the highest fatality rate among connective tissue diseases and is characterized by vascular damage, inflammation and fibrosis of the skin and various internal organs. Interstitial lung disease (ILD) frequently complicates SSc and can be a debilitating disorder with a poor prognosis. ILD is the most frequent cause of death in SSc, and the management of SSc–ILD patients is a great challenge. Early detection of pulmonary involvement based on a recent decline of lung function tests and on the extent of lung involvement at high-resolution computed tomography is critical for the best management of these patients. This article summarizes classification, pathogenesis, diagnosis, prognosis, survival and finally current and future treatment options in SSc–ILD.

yearjournalcountryeditionlanguage
2017-01-01
10.1007/s00296-016-3636-7http://hdl.handle.net/11697/123703