Systemic Sclerosis Classification Prognosis and Complications

Systemic Sclerosis (Scleroderma)

• Systemic Sclerosis Classification Epidemiology Pathology Prognosis and Complications

It is a chronicChronic Hepatitis Classification viral hepatitis Clinical features and Treatment. Read more ... » multisystem disorder.

There is thickening of the skin due to accumula­tion of connective tissue affecting the structure and function of gastrointestinal system (GITAmoebiasis infection Diagnosis Treatment protozoan Entamoeba histolytica Symptoms and Causes. Read more ... »), lungs, heart, kidneys and other organs.

There is vascular damage, and other immune processes resulting in damage of connective tis­sues. Skin and internal organs are involved to different extents.

Diffuse cutaneous scleroderma

• There is symmetric skin thickening of upper and lower limbs, face and trunk.
• KidneyDiabetic Nephropathy and Renal complications of DM (Diabetes Mellitus). Read more ... » may be inyolved.

Limited cutaneous scleroderma

• There is symmetric skin thickening of distal extremi­ties a nd face.
• Features of CREST syndromeMetabolic,Insulin Resistance Syndrome X Causes Symptoms. Read more ... » are present – calcino­sis, Raynaud’s phenomenon, oesophageal dysmotility, sclerodactyly, telangiectasia.
• There is pulmonary arterial hypertensionThyroid Disorders Examination and Laboratory tests. Read more ... ». Biliary cirrhosisBiliary Cirrhosis (Primary and Secondary) Symptoms Diagnosis and Treatment. Read more ... ».

Systemic sclerosis sine scleroderma

• There is systemic sclerosis without any skin involve­ment.
• Localized scleroderma, occurs in young women and children.

en Coup de saber

• There is a linear scleroderma of face on the forehead with hemiatrophy of the same side of face.
• The linear lesion resembles a wound from a sword, hence called en coup de saber.

Mixed connective tissue disease (MCTD)

• There is SLE, scleroderma, polymyositis, rheumatoid arthritis, high titres of RNP (ribonucleo protein).
• There is eosinophilia myalgia syndrome.

Systemic Sclerosis Classification Epidemiology Pathology Prognosis and Complications

Classification of localized scleroderma

• Bullous morphea
  Bullous involvement
  May occur in other forms of localized scleroderma
• Deep morphea
  Least common form of localized scleroderma in children
  Most disabling form of localized scleroderma
  Primary site of involvement is the panniculus (subcutaneous tissue)
• Generalized morphea
  Multiple plaques that are confluent or affect three or more anatomic sites
  Often located on the trunk or limbs
  Similar clinicalPericardiaI Diseases Diagnosis Causes and Physical findings and PericardiaI Diseases Treatment. Read more ... » and histologic features as plaque morphea
• Linear scleroderma
  Most common form of localized scleroderma in children
  One or more elongated sclerotic areas of skin
  Lesions are typically asymmetric and oriented along the affected limb
  Associated with growth impairment of the involved extremity
  Lesions on the face or scalp are called en coup de sabre
• Plaque morphea
  One or more circumscribed patches of skin with sclerotic changes in two or less anatomic sites
  Involvement is confined to the superificial panniculus (subcutaneous tissue)

Classification of systemic sclerosis

• Diffuse cutaneous scleroderma
  Raynaud’s phenomenon followed, within one year, by puffy or hidebound skin changes
  Truncal and acral skin involvement; tendon friction rubs
  Nailfold capillary dilatation and capillary drop-out
  Early and significant incidence of renalDiabetic Nephropathy and Renal complications of DM (Diabetes Mellitus). Read more ... », interstitial lung, diffuse gastrointestinal, and myocardial diseasePericardiaI Diseases Diagnosis Causes and Physical findings and PericardiaI Diseases Treatment. Read more ... »
  Anti-Scl-70 (30 percent) and anti-RNA polymerase-I, II, or III (12 to 15 percent) antibodies
• Environmentally induced scleroderma
  Generally diffuse distribution of skin sclerosis and a history of exposure to an environmental agent suspected of causing scleroderma
• Limited cutaneous scleroderma
  Raynaud’s phenomenon for years, occasionally decades
  Skin involvement limited to hands, face, feet, and forearms (acral distribution)
  Dilated nailfold capillary loops, usually without capillary drop-out
  A significant (10 to 15 percent) late incidence of pulmonary hypertension, with or without skin calcification, gastrointestinal disease, telangiectasias (CREST syndrome), or interstitial lung disease
  Renal disease rarely occurs
  Anticentromere antibody (ACA) in 70 to 80 percent
• Overlap syndromes
  Features of systemic sclerosis which coexist with those of another autoimmune rheumatic disease such as systemic lupus erythematosus, rheumatoid arthritis, dermatomyositis, vasculitis, or Sjögren’s syndrome.
• Pre-scleroderma
  Raynaud’s phenomenon
  Nailfold capillary changes and evidence of digital ischemia
  Specific circulating autoantibodies – anti-topoisomerase-I (Scl-70), anti-centromere (ACA), or anti-RNA polymerase-I, II, or III
• Scleroderma sine scleroderma
  Presentation with pulmonary fibrosis or renal, cardiacPericardiaI Diseases Diagnosis Causes and Physical findings and PericardiaI Diseases Treatment. Read more ... », or gastrointestinal disease
  No skin involvement
  Raynaud’s phenomenon may be present
  Antinuclear antibodies may be present – anti-Scl-70, ACA, or anti-RNA polymerase-I, II, or III

Systemic Sclerosis Epidemiology

• Predominant age:
  • Young adult (16–40 years); middle-aged (40–75 years), peak onset 30–50 years
  • SymptomsPericardiaI Diseases Diagnosis Causes and Physical findings and PericardiaI Diseases Treatment. Read more ... » usually appear in the 3rd–5th decades.
• Predominant sex: Female > Male (4:1)
• · Most common in 3^rd to 5^thdecade.
• · More common in women.
• · Antinuclear antibodies (ANA), Human leucocyte antigen (HLAL Anticentromere antibodies (ACA) are all associated with the disease.
• · It is more common in coal and gold miners.
• · On ingestion of rapeseed oil, mixed in cooking oil, a scleroderma-like syndrome develops with the following features:
• - Interstitial pneumonitis
• - Eosinophilia
• - Arthralgias
• - Arthritis
• - Myositis
• - Skin thickening
• - Raynaud’s phenomenon
• - Pulmonary hypertension
• - Resorption of digits.
• · It can also occur due to silicon breast implants.

Systemic Sclerosis Pathology

• There is accumulation of collagen and other extracel­lular matrix proteins.
• Immunologic mechanisms are involved.
• There is vascular injury to blood vessels of skin, GIT, kidneys, heartPericardiaI Diseases Diagnosis Causes and Physical findings and PericardiaI Diseases Treatment. Read more ... » and lungs.

Systemic Sclerosis Prognosis

• Possible improvement, but incurable
• Prognosis is poor if cardiac, pulmonary, or renal manifestations present early.

Systemic Sclerosis Complications

• ArrhythmiaSick Sinus Syndrome SSS Symptoms Causes Diagnosis with Treatment. Read more ... »
• CardiomyopathyCardiomyopathy Clinical features Etiology Diagnosis Dilated Hypertrophic Restrictive cardiomyopathy with Treatment. Read more ... »
• Death
• Disability
• Esophageal dysmotility
• Flexion contractures
• Megacolon
• Obstructive bowel
• Pneumatosis intestinalis
• Possible association with lung and other cancers
• Pulmonary hypertension
• Reflux esophagitis
• Renal failure
• Respiratory failureRespiratory Failure Causes Clinical Features with Management. Read more ... »