Case report, case study
Very Early Diagnosis of Systemic Sclerosis in Clinical Practice – Case Report and Review of the Literature
Ivan Marković
; Special Hospital for Respiratory Diseases Rockefellerova, Zagreb, Croatia;
Anja Ljilja Posavec
; Polyclinic for the respiratory tract diseases, Zagreb, Croatia
Jadranka Morović-Vergles
; Division of Clinical Immunology, Al- lergology and Rheumatology, Department of Internal Medicine, School of Medicine University of Zagreb, Dubrava University Hospital, Zagreb, Croatia
Joško Mitrović
; Division of Clinical Immunology, Al- lergology and Rheumatology, Department of Internal Medicine, School of Medicine University of Zagreb, Dubrava University Hospital, Zagreb, Croatia
Abstract
Systemic sclerosis (SSc) is a systemic autoimmune disease charac-
terised by generalized microangiopathy and fibrosis of skin and internal organs.
The 2013 American College of Rheumatology (ACR) / European League Against
Rheumatism (EULAR) criteria have contributed considerably to classifying pa-
tients with SSc in earlier stages, but they still lack sensitivity for a very early stage
of the disease. Criteria for a very early diagnosis of SSc (VEDOSS) have been pro-
posed by EULAR Scleroderma Trial and Research group (EUSTAR) which include
three red flags: Raynaud’s phenomenon, puffy fingers and antinuclear antibody
positivity, plus SSc specific antibodies positivity and/or abnormal nailfold capil-
laroscopy.
We report a case of a 54-year-old female patient with 6-week history of puffy
fingers, Raynaud phenomenon and positive antinuclear antibodies. Further
workup revealed early pathologic capillary pattern by nailfold capillaroscopy
and positive anticentromere antibodies. Screening for internal organ involve-
ment detected no heart, lung, or upper gastrointestinal tract involvement. The
patient was started on pentoxifylline with further follow-up.
The aim of the implementation of VEDOSS criteria is to diagnose SSc at the earli-
est possible stage, so that subclinical internal organ involvement could be de-
tected and appropriate treatment started at a potentially reversible stage.
Keywords
Systemic sclerosis; Raynaud phenomenon; antibodies; antinuclear
Hrčak ID:
299675
URI
Publication date:
14.12.2022.
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