Summary about Disease
Systemic sclerosis (SSc), also known as scleroderma, is a chronic autoimmune disease characterized by hardening and tightening of the skin and connective tissues. It can affect various internal organs, including the heart, lungs, kidneys, and gastrointestinal tract. The severity and progression of SSc vary widely among individuals.
Symptoms
Symptoms vary depending on the individual and the organs affected. Common symptoms include:
Skin Changes: Thickening and tightening of the skin, often starting in the fingers and hands. May include swelling, itching, and pain.
Raynaud's Phenomenon: Fingers and toes turn white or blue in response to cold or stress.
Joint Pain and Stiffness: Similar to arthritis.
Gastrointestinal Problems: Difficulty swallowing, heartburn, bloating, diarrhea, or constipation.
Lung Problems: Shortness of breath, persistent cough, and pulmonary hypertension.
Heart Problems: Arrhythmias, heart failure, pericarditis.
Kidney Problems: Scleroderma renal crisis (a rare but serious complication).
Telangiectasias: Small, widened blood vessels visible on the skin, often on the face and hands.
Fatigue: Persistent and overwhelming tiredness.
Digital Ulcers: Sores on the fingers or toes due to poor circulation.
Causes
The exact cause of SSc is unknown, but it is believed to be a combination of genetic predisposition, environmental factors, and immune system dysfunction. The immune system mistakenly attacks the body's own tissues, leading to inflammation and overproduction of collagen, which causes the hardening and thickening of the skin and other organs.
Medicine Used
There is no cure for SSc, but medications can help manage symptoms and slow the progression of the disease. Common medications include:
Immunosuppressants: To suppress the immune system (e.g., methotrexate, mycophenolate mofetil, cyclophosphamide).
Calcium Channel Blockers: To treat Raynaud's phenomenon.
Proton Pump Inhibitors (PPIs): To manage heartburn and acid reflux.
ACE Inhibitors: To treat scleroderma renal crisis.
Endothelin Receptor Antagonists and Phosphodiesterase-5 (PDE5) Inhibitors: To treat pulmonary hypertension.
Corticosteroids: To reduce inflammation.
NSAIDS (non-steroidal anti-inflammatory drugs): To help with pain.
Is Communicable
No, systemic sclerosis is not communicable or contagious. It cannot be spread from person to person through contact.
Precautions
There are no specific precautions to prevent SSc since the cause is unknown. However, people with SSc can take the following precautions to manage their symptoms and improve their quality of life:
Protect skin: Use moisturizers, avoid harsh soaps, and protect skin from the sun and cold.
Manage Raynaud's: Keep hands and feet warm, avoid smoking, and manage stress.
Physical therapy: Maintain joint flexibility and muscle strength.
Diet and nutrition: Eat a healthy diet and manage gastrointestinal symptoms.
Regular medical checkups: Monitor disease progression and adjust treatment as needed.
Vaccinations: To reduce the risk of infections, especially respiratory illnesses.
How long does an outbreak last?
SSc is a chronic disease, not a disease with outbreaks. The disease progresses over time and requires ongoing management. Disease activity can fluctuate, with periods of increased symptoms (flares) and periods of relative stability. The duration of these fluctuations varies among individuals.
How is it diagnosed?
Diagnosis typically involves:
Physical Examination: Assessing skin changes, joint mobility, and other physical signs.
Medical History: Reviewing the patient's symptoms and medical history.
Blood Tests:
Antinuclear Antibody (ANA): Often positive in SSc.
Scleroderma-specific Antibodies: Such as anti-centromere, anti-Scl-70 (anti-topoisomerase I), and anti-RNA polymerase III antibodies. These help differentiate subtypes of SSc.
Other Tests: To assess organ function (e.g., kidney, liver, lung).
Skin Biopsy: To examine skin tissue for characteristic changes.
Pulmonary Function Tests (PFTs): To assess lung function.
Echocardiogram: To evaluate heart function and pulmonary artery pressure.
High-Resolution Computed Tomography (HRCT) of the Chest: To assess lung involvement.
Esophageal Manometry/Barium Swallow: To assess esophageal function.
Timeline of Symptoms
The timeline of symptoms varies significantly among individuals. SSc typically progresses in stages:
Early Stage: Raynaud's phenomenon, puffy fingers and hands, joint pain, fatigue.
Intermediate Stage: Skin thickening progresses, internal organ involvement may begin, gastrointestinal symptoms, lung problems.
Late Stage: Skin thickening may stabilize or even improve, but internal organ damage can progress, leading to complications. It's important to note that not everyone experiences all stages or progresses through them at the same rate.
Important Considerations
Early Diagnosis and Treatment: Early diagnosis and treatment are crucial to slow the progression of SSc and prevent organ damage.
Multidisciplinary Care: SSc requires a team approach involving rheumatologists, dermatologists, pulmonologists, gastroenterologists, cardiologists, and other specialists.
Individualized Treatment: Treatment plans should be tailored to the individual's specific symptoms and organ involvement.
Monitoring for Complications: Regular monitoring for organ involvement and complications is essential.
Support Groups: Connecting with other people with SSc can provide emotional support and valuable information.
Lifestyle Modifications: Healthy lifestyle choices, such as regular exercise, a balanced diet, and stress management, can improve quality of life.