JNUH

Systemic Sclerosis

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

 

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

Consult with experienced Doctors

JNU is home to some of the most eminent doctors in the world, most of whom are pioneers in their respective arenas and are renowned for developing innovative and revolutionary procedures
Gajanand Ojha

Dr. Gajanand Ojha

Professor

Qualification

MBBS, MD

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Priyanka Sharma

Dr. Priyanka Sharma

Associate Professor

Qualification

MBBS, MD

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Neha Agarwal

Dr. Neha Agarwal

Assistant Professor

Qualification

MBBS, MD

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Vikarn Garg

Dr. Vikarn Garg

Assistant Professor

Qualification

MBBS, MD

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Amit Kumar Malhotra

Dr. Amit Kumar Malhotra

Assistant Professor

Qualification

MBBS, MD

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