Systemic Sclerosis

Systemic sclerosis is a multisystem autoimmune disorder characterized by vascular abnormalities, connective tissue sclerosis and atrophy.

Cutaneous features

  • Raynaud’s phenomenon
  • ‘Hide-bound’ skin
  • Classical sclerodermoid facies- “mask-like” face, pinched or beak-like appearance of nose, radial furrows around the mouth and thinning of the upper lip
  • Pigmentation – mottled or hyperpigmentation
  • Swelling of hands & joints, atrophy
  • Finger and leg ulcers, digital gangrene, stellate scars
  • Nail fold telangiectasias
  • Calcinosis

Systemic Features

  • Gastrointestinal and Hepatic - Esophageal Dysfunction, malabsorption, primary biliary cirrhosis, Autoimmune hepatitis.
  • Arthritis, tendon friction rubs.
  • Renal-scleroderma renal crisis (most severe).
  • Lung-Interstitial lung disease, Pulmonary hyertension.
  • Cardiac-myocardial disease, pericardial involvement.
  • Muscle-SSc Associated myopathy more common in diffuse SSc.
  • Digital infarctions
  • Pulmonary hypertension
  • Myositis
  • Renal failure
  • Wound infections
  • Immune activation, fibrosis of skin and obliterative vasculopathy.
  • Females : Males - 5.2 : 1
  • Onset : Fourth decade

How is it diagnosed?

2013 ACR/EULAR criteria
  • Skin thickening of the fingers extending proximal to MCP jts is sufficient to diagnose the patient as SSc.
  • If this is not present seven other features apply with for varying weights for each -
  • Skin thickening of fingers
  • Finger tip lesions
  • Telangiectasia
  • Abnormal nail fold capillaries
  • Interstitial lung disease or pulmonary artery hypertension
  • Raynaud’s phenomenon
  • Ssc related autoantibodies (anticentromere, antitopoisomerase, antiRnp-3)

Investigations

  • Serology - ANA, anticentromere antibodies, anti-Scl 70 antibody
  • Pulmonary Function test - Detect fibrotic changes
  • Histopathology - Hyalinization and homogenisation of collagen, dermal lymphocytic infiltrate.
  • ECG - To detect rhythm and conduction abnormalities.
  • ECHO - Detect pulmonary artery hypertension.
  • GI involvement - Esophageal manometry, endoscopy, barium studies.

 

How is it treated?

  • Use of gloves
  • For Raynauds - nifedepine (mild cases), sildenafil, iloprost, low molecular weight dextran.
  • Corticosteroids
  • Immunosuppressants : Methotrexate, Cyclophosphamide
  • Penicillamine, colchicine, interferons.
  • Symptomatic treatment for pulmonary, cardiac, renal and GIT symptoms

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