Systemic Sclerosis: A Rare but Serious Autoimmune Disease

Systemic sclerosis, also known as scleroderma, is a rare autoimmune disease that primarily affects the skin but can also involve internal organs such as the lungs, heart, kidneys, and digestive system. Although uncommon, it is a serious condition that requires timely recognition and management.

What is systemic sclerosis?

The word scleroderma literally means "hard skin." In this condition, the body’s immune system becomes overactive and produces excess collagen. This leads to thickening and tightening of the skin and, in more severe cases, fibrosis (scarring) of vital organs. It mostly affects women between the ages of 30 and 50, but men and children can also be affected.

Symptoms and warning signs

Patients may first notice coldness and color changes in their fingers or toes when exposed to cold (Raynaud’s phenomenon). Other common symptoms include:

• Tight, shiny, or thickened skin, especially of the hands and face
• Joint stiffness and muscle weakness
• Difficulty swallowing or heartburn
• Shortness of breath or persistent cough (if the lungs are affected)
• High blood pressure due to kidney involvement

Because symptoms vary from person to person, systemic sclerosis can be difficult to diagnose early.

Why early diagnosis matters

Although there is no cure, modern treatments can slow disease progression and improve quality of life. Early detection allows doctors to screen for lung, heart, or kidney involvement and start treatment before irreversible damage occurs.

Treatment and care

Treatment focuses on managing symptoms and preventing complications. Options include:

• Medications to control the immune system
• Drugs to improve circulation and treat Raynaud’s
• Targeted therapies for lung involvement, including biologic drugs and antifibrotic agents
• Supportive measures such as physiotherapy, skin care, and dietary modifications
• Regular monitoring of lungs, heart, and kidneys

Supportive care, such as protecting the skin from cold, avoiding smoking, and maintaining a balanced lifestyle, is equally important.

Living with systemic sclerosis

With proper medical care and awareness, many patients live full and productive lives. Patient support groups and counseling can also help individuals cope with the emotional and physical challenges of the disease.

Author: 

Dr. Farzana Shumy 

MD (Rheumatology), MRCP (UK), FCPS (Medicine), FRCP (Edin), 

FACR fellow (America), APLAR Fellow (Japan), EULAR Certified (Rheumatology) 

Consultant- Rheumatology 

Evercare Hospital Dhaka