title: Systemic sclerosis refractory disease: from the skin to the heart.
abstract: Systemic sclerosis or scleroderma (SSc) is an heterogeneous disease involving the connective tissue and the microvasculature with fibrosis and vascular occlusion. It is difficult to define refractory SSc once it is itself a paradigm of a refractory condition: there is no evidence of when to act to stop the progression to fibrosis and irreversible microvascular damage. There is no definition of refractory disease in SSc and to propose a definition we used mainly the Medsger severity index and the EULAR 2009 treatment recommendations from the skin to the heart through peripheral vascular, musculoskeletal, gastrointestinal, renal, pulmonary hypertension and interstitial lung disease. We used some clinical setting reflecting the different reasoning when there is probable refractory disease and finally we briefly pointed out some available treatment options to refractory disease. With this reflection, we would like to open paths to a broader discussion.
authors.affiliation: Unidade de Imunologia Clínica, Hospital de Santo António, Centro Hospitalar do Porto, Portugal. isabel.almeida40@gmail.com
authors.forename: Isabel
authors.initials: I
authors.lastname: Almeida
authors.forename: Raquel
authors.initials: R
authors.lastname: Faria
authors.forename: Pedro
authors.initials: P
authors.lastname: Vita
authors.forename: Carlos
authors.initials: C
authors.lastname: Vasconcelos
mesh_terms: D000933:Antifibrinolytic Agents / Q000627:therapeutic use*; D002986:Clinical Trials as Topic; D003238:Connective Tissue / Q000187:drug effects / Q000473:pathology; D018450:Disease Progression; D004351:Drug Resistance; D005355:Fibrosis; D006801:Humans; D006976:Hypertension, Pulmonary; D007155:Immunologic Factors / Q000627:therapeutic use*; D055806:Microvessels / Q000187:drug effects / Q000473:pathology; D012008:Recurrence; D051437:Renal Insufficiency; D012595:Scleroderma, Systemic / Q000175:diagnosis* / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology; D012883:Skin Ulcer; D033581:Stem Cell Transplantation
chemical_list: D000933:Antifibrinolytic Agents; D007155:Immunologic Factors