17/07/2023
Systemic sclerosis, also known as scleroderma, is a chronic autoimmune disease that affects the connective tissues of the body.
It is characterized by excessive production and accumulation of collagen, a fibrous protein, in the skin and various internal organs. This abnormal collagen buildup leads to hardening and thickening of the affected tissues.
There are two main types of systemic sclerosis: limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous systemic sclerosis (dcSSc).
lcSSc typically involves the skin of the hands, face, and feet, while dcSSc affects larger areas of the skin and may involve internal organs such as the lungs, heart, kidneys, and gastrointestinal tract.
The exact cause of systemic sclerosis is unknown, but it is believed to involve a combination of genetic predisposition and environmental triggers.
It is thought to be an autoimmune disorder, in which the immune system mistakenly attacks the body's own tissues.
Symptoms of systemic sclerosis can vary widely depending on the organs involved, but common manifestations include thickening and hardening of the skin, particularly in the fingers and face, sensitivity to cold or stress leading to Raynaud's phenomenon, gastrointestinal issues like acid reflux and difficulty swallowing, lung problems causing shortness of breath and cough, kidney involvement resulting in hypertension and urine production problems, joint and muscle pain, and persistent fatigue.
Diagnosis involves a combination of clinical evaluation, medical history, and various tests such as blood tests and imaging studies to assess organ involvement.
While there is no cure for systemic sclerosis, treatment aims to manage symptoms, slow disease progression, and prevent complications.
This may involve medications to suppress the immune system, relieve symptoms, and manage organ-specific complications.
Physical therapy, occupational therapy, and lifestyle modifications are also recommended.
Individuals with systemic sclerosis should work closely with healthcare professionals specializing in the disease to receive appropriate care and monitoring.