Pp. 123-142 (20)
Systemic sclerosis (SSc) is a connective tissue disease characterized by
fibrosis of the skin and internal organs, changes in the microvasculature and in cellular
and humoral immunity.
The types of disorders include: localized scleroderma (morphea, linear scleroderma, en
coup de sabre) and systemic sclerosis (diffuse cutaneous systemic sclerosis [dsSSc],
limited cutaneous systemic sclerosis [lcSSc] and systemic sclerosis sine scleroderma).
LcSSc is a milder form with slow progression of skin involvement and visceral
complications after 10-15 years, but with serious pulmonary arterial hypertension.
DsSSc has fast progression of skin sclerosis, from weeks to months, and includes
injuries to organs such as the lung, heart and kidney, which may lead to organ failure.
The presence of ANA was found in more than 90% of patients with SSc, anti-Scl-70
antibodies in patients with dcSSc in 30-40%, anticentromere antibodies (ACA) in
patients with lcSSc in 80-90%.
Fibrosis is the end-stage representation of SSc pathogenesis.
Severe forms of the disease and rapidly progressive diffuse SSc with pulmonary,
cardiac, and renal involvement, are associated with very high mortality rates, estimated
at 40-50% in 5 years. No treatment effective therapy exists to stop fibrosis and disease
progression beside autologous haemopoietic stem cell transplantation (AHSCT).
Cyclophospamide has moderate efficacy in patients with ILD. Methotrexate may be
used for the treatment of arthritis. Oesophageal dysmotility and reflux are treated with
promotility agents, and proton pump inhibitors. In PAH endothelin receptor
antagonists, with PDE5 inhibitor and prostaglandins are used. The most important
manifestation is scleroderma renal crisis and is now treated using ACE inhibitors.
ACE inhibitors, AHSCT, ANA, Anticentromere antibodies (ACA),
Anti-RNA polymerases II and III antibodies, Anti-Scl-70 antibodies, Calcinosis,
CREST syndrome, Cyclophospamide, Diffuse cutaneous systemic sclerosis
(dsSSc), Endothelin receptor antagonists, Fibrosis, Limited cutaneous systemic
sclerosis (lcSSc), Localized scleroderma, Oesophageal dysmotility, PAH, PDE5
inhibitor, Prostaglandins, Proton pump inhibitors, Raynaud’s phenomenon,
Sclerodactyly, Systemic sclerosis (SSc), Teleangiectasis, TGFβ.
Head of Rheumatology and Internal Diseases Department CSK MSWiA Warsaw Poland.