Reumatizam, Vol. 64 No. suppl. 1, 2017.
Review article
SYSTEMIC SCLEROSIS: AN OVERVIEW
Dušanka Martinović Kaliterna
Abstract
Systemic sclerosis (SSc) is considered as autoimmune disease of connective tissue. It is characterised with obliterative
and proliferative micro vascular involvement, activation of the immune system and excessive fi brosis of skin
and internal organs. Th e possibility of genetic infl uences in disease progression and the role of SSc antibodies for diagnosis
are exploring. Th e criteria for SSc established in 2013 by the American College of Rheumatology/European League
Against Rheumatism are more sensitive than previous. Modifi ed Rodnan Skin Score remains the best method for the
objective assessment of skin. Micro vascular changes are observed by videocapillaroscopy what is useful for early diagnosis
and prognosis. Digital ulcers are considered as an early manifestation of vasculopathy. Lung complications including
interstitial lung disease and pulmonary artery hypertension, can be superimposed and they are considered as
the major cause of death in SSc. Myocardial fi brosis is associated with diastolic dysfunction and high risk of cardiac
arrhythmias. Since the induction of ACE-inhibitors the kidney complications like renal crisis are less but the patients
on glucocorticoids are on the great risk for kidney damage. Oesophageal and ano-rectal involvement are the earliest
involvement of gastrointestinal tract. Barrett’s oesophagus, GAVE or watermelon stomach as well as intestinal pneumatosis,
pseudodiverticulosis andcolonis telangiectasias may appear. Immunosuppressive therapy is recommended in
diff use cutaneous SSc with rapidly progressive interstitial lung disease, starting with cyclophosphamide and next
switching to azathioprine or mycophenolate mofetil. Endothelin1 receptor antagonist improved digital ulcers and
PAH. Combination therapy of siledanfi l and bosentan or ambrisentan and tanadanafi l is recommended for PAH. Th e
results of hematopoietic stem cell transplantation are doubtful. Platelet-derived growth factor and transforming growth
factor-β are infl uenced by tyrosine kinase. Imatinib, the tyrosine kinase inhibitor showed the improvement of lung
function. Fresolimumab, a monoclonal antibody to transforming growth factor-β, improved skin disease. SSc fi broblasts
produce high levels of interleukin-6. Tocilizumab, monoclonal antibody against interleukin-6 is expected to
improve the disease, but the risk of gastrointestinal complications appears. Intravenous immunoglobulin’s showed effectiveness
in skin and gastrointestinal changes. Th e recommended therapies for Raynaud phenomenon are calcium
channel blockers and for second line serotonin uptake antagonists.
Keywords
Scleroderma, systemic – complications, diagnosis, etiology, therapy; Transforming growth factor beta; Lung diseases, interstitial – etiology; Hypertension, pulmonary – etiology; Raynaud disease – etiology; Skin ulcer – etiology; Telangiectasis – etiology; Fibrosis – etiology; Myocardium – pathology; Gastrointestinal diseases – etiology; Immunosuppressive agents – therapeutic use; Endothelin a receptor antagonists – therapeutic use; Antibodies, monoclonal – therapeutic use
Hrčak ID:
188329
URI
Publication date:
25.10.2017.
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