Systemic sclerosis (scleroderma) is a connective tissue disease caused by autoimmune dysfunction, characterized by excessive collagen deposition leading to fibrosis of the skin and internal organs, accompanied by microvascular lesions and immune abnormalities. The pathological process includes microvascular injury, fibroblast activation, and immune-mediated tissue remodeling, resulting in progressive organ function loss. Diagnosis requires a combination of clinical and auxiliary examinations.
If systemic sclerosis is not recognized and treated early, microvascular lesions will worsen, and fibrosis will continue to progress. This increases the risk of severe complications such as interstitial lung disease, pulmonary hypertension, and renal crisis. Long-term cumulative damage may lead to respiratory failure, heart failure, and renal insufficiency, which can be disabling or even life-threatening. Therefore, early diagnosis and intervention are crucial for prognosis.
1. Clinical manifestations and history
Symptoms such as Raynaud’s phenomenon, onset of skin sclerosis, digestive system symptoms, or respiratory disorders provide preliminary diagnostic clues and serve as the entry point for screening.
2. Physical examination and skin assessment
Assessment of skin thickness, fingertip ulcers, changes in skin elasticity, and joint involvement helps determine disease course and activity.
3. Antibody testing
Antinuclear antibodies (ANA), anti-Scl-70 (topoisomerase I), and anti-RNA polymerase III are highly valuable for diagnosis and phenotype prediction.
4. Microvascular and skin pathology examination
Capillaroscopy can detect abnormal capillary morphology, while skin biopsy can assess the degree of fibrosis and exclude other lesions.
5. Imaging and functional tests
High-resolution CT can identify interstitial lung disease, esophageal manometry can evaluate swallowing disorders, and echocardiography can screen for pulmonary hypertension and cardiac involvement.
6. Multidisciplinary comprehensive evaluation
Collaboration among rheumatology, respiratory, cardiology, nephrology, and gastroenterology departments is recommended to regularly evaluate disease progression and formulate treatment plans.
7. Serum and inflammatory markers
Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and immune-related cytokines help assess disease activity and monitor treatment response.
8. Pulmonary function tests and six-minute walk test
These assess respiratory function prognosis and pulmonary hypertension risk, serving as important tools for long-term follow-up.
9. Complication screening and risk stratification
Timely screening for high-risk complications such as pulmonary hypertension, renal crisis, and infection allows early intervention and optimized treatment.
Systemic sclerosis is a multi-organ, rapidly progressive autoimmune fibrotic disease. Without timely diagnosis and intervention, it can lead to irreversible organ damage and significantly increased mortality risk. Experts at United Life International Medical Center emphasize strengthening primary care recognition and multidisciplinary collaboration, enhancing early diagnostic capabilities, and using diagnostic tools for comprehensive disease evaluation to optimize treatment strategies, improve prognosis, and enhance quality of life.