Systemic Sclerosis

Clinic

• Systemic scleroderma, or systemic sclerosis, is an autoimmune rheumatic disease
• SS is characterized by excessive production and accumulation of collagen, called fibrosis, in the skin and internal organs.
• 2 major subgroups are limited and diffuse.
• Limited form affects areas below the elbows and knees with or without involvement of the face.
• Diffuse form also affects the skin above the elbows and knees and can also spread to the torso.
• Visceral organs, including the kidneys, heart, lungs, and GI tract can also be affected by the fibrotic process.

Signs and symptoms

Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasia (CREST syndrome) are associated with limited scleroderma. Other symptoms include:

• Hardening and scarring,Tight, reddish, or scaly.

• Raynaud's phenomenon (Over 80% )
• Calcinosis near the elbows, knees, or other joints.

• Arthralgia which can lead to arthritis, esp small joints of the hand, may be restricted by calcinosis or skin thickening.
• Myopathy

• Pulmonary hypertension that can lead to right-sided heart failure.
• Aspiration pneumonia, pulmonary hemorrhage and pneumothorax

• Chronic GI Reflux, Dysphagia
• Reflux esophagitis due to esophageal strictures
• Decreased motility of esophagus and the lower esophageal sphincter, leading to dysphagia and chest pain.
• Visceral myopathy of duodenum can lead to dilated, atonic duodenum.
• Small intestine bacterial overgrowth leads to malabsorption that can cause pseudo-obstruction or ischemic colitis.
• Rarer complications include pneumatosis cystoides intestinalis

• The most important involvement of kidneys is scleroderma renal crisis (SRC): Malignant hypertension, Hyperreninemia, Azotemia, and Microangiopathic hemolytic anemia, Hematuria and Proteinuria.

Causes

• No clear cause
• Genetic predisposition appears to be limited
• Evidence implicating CMV and parvovirus B19
• One of the suspected mechanisms behind the autoimmune phenomenon