Systemic Sclerosis
Systemic sclerosis (SSc) is a chronic, multisystem autoimmune connective‑tissue disease characterised by progressive fibrosis of skin and internal organs, vasculopathy and immune dysregulation.
What our notes say
- Systemic sclerosis (SSc) is a chronic, multisystem autoimmune connective‑tissue disease characterised by progressive fibrosis of skin and internal organs, vasculopathy and immune dysregulation. (Systemic sclerosis)
- How long does each colour last?” | Early manifestation of digital vasospasm; helps differentiate primary from secondary Raynaud’s (the latter is common in systemic sclerosis). | (Systemic sclerosis)
- | Connective tissue disease–related | Rheumatoid arthritis, systemic sclerosis, SLE, myositis, Sjögren's | (Interstitial Lung Diseases)
- CTD-ILD: commonly systemic sclerosis (NSIP), rheumatoid arthritis (UIP); treat with immunosuppression. (Interstitial Lung Diseases)
- | Autoimmune disease (e.g., rheumatoid arthritis, systemic sclerosis) | Silica acts as adjuvant, breaking tolerance | Polyarthritis, skin thickening; may worsen pulmonary fibrosis | Can appear years after exposure; prevalence higher in silicosis | (Occupational Lung Disease)
- Topics tested: rheumatoid arthritis, systemic lupus erythematosus, CREST and systemic sclerosis, myositis, vasculitis, gout and pseudogout, osteoporosis and fibromyalgia. (Internal Medicine Rheumatology CAT: 30 MCQs and 5 short answer questions)
Study material on Systemic Sclerosis
- Systemic sclerosis
- Immunopathology complete mcqs
- Immunopathology
- Gastrointestinal and liver
- Interstitial Lung Diseases
- Internal Medicine (MBIM 4100) special/supplementary exam, Paper Two: 14 August 2019
- Respiratory Pathology
Practice questions on Systemic Sclerosis
50-year-old female, systemic sclerosis, right heart failure, PCWP normal, PVR markedly elevated. Vascular pathology MOST responsible?
- Pulmonary venous hypertension from left heart disease
- Pulmonary arteriolar intimal fibrosis and medial hypertrophy (PAH)
- Multiple pulmonary emboli causing mechanical obstruction
- Pulmonary interstitial fibrosis causing capillary destruction
- Pleuropericardial effusion compressing pulmonary vessels
Answer: B. - Normal PCWP + elevated PVR = **precapillary** pulmonary hypertension - In scleroderma, this is due to **PAH**-type small vessel remodeling: intimal fibrosis and medial hypertrophy of pulmonary arterioles (Group 1 PAH associated with connective tissue disease)
A patient with systemic sclerosis develops bilateral interstitial lung disease. The most common histologic pattern in systemic sclerosis lung involvement is
- UIP pattern identical to IPF
- Cryptogenic organizing pneumonia pattern
- NSIP (nonspecific interstitial pneumonia) pattern
- Desquamative interstitial pneumonia pattern
Answer: C. NSIP is the most common histologic pattern of lung involvement in collagen vascular diseases, particularly systemic sclerosis. UIP pattern can also occur but NSIP predominates.