Internal Organ Damage in Systemic Sclerosis: We Can’t Live without Each Other

<jats:p>Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by a triad of pathological processes: fibrosis, microangiopathy, immune-inflammatory activity. The disease is highly clinically variable and often accompanied by multisystem involvement, which determines the prognosis and outcome. The lungs are the most frequently affected organs: interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are observed in a significant proportion of patients. ILD can present asymptomatically or with progressive dyspnea, cough, with typical radiographic findings. PAH develops as a consequence of vasculopathy and is one of the most severe complications of SSc, associated with a high mortality rate. The cardiovascular system is also frequently involved in the pathological process. Patients may experience myocarditis, myocardial fibrosis, conduction disturbances, pericarditis. These changes are often asymptomatic, emphasizing the need for regular instrumental monitoring. The gastrointestinal tract is affected in over 90% of cases. The esophagus is most commonly affected, manifesting as gastroesophageal reflux disease, impaired peristalsis, stenosis. Involvement of the stomach, small intestine, colon is also possible, significantly reducing the quality of life of patients. The kidneys in SSc can be involved in the so-called “scleroderma renal crisis” – an acute condition characterized by a sharp rise in blood pressure, impaired renal function, microangiopathic hemolysis. Thus, systemic sclerosis is a multiorgan disease with a high degree of disability, requiring a multidisciplinary approach to diagnosis, monitoring, and treatment.</jats:p>