IPLab:Lab 6:Scleroderma

Clinical Summary

This 29-year-old black female had a history of scleroderma involving the lung, kidney, heart, and skin. Her main clinical problems centered on her restrictive lung disease. She was able to live at home with supplemental oxygen but recently she had developed edema, chest pain, weakness, light-headedness, and a loss of appetite. The patient was admitted to the hospital with a working diagnosis of congestive heart failure brought on by her lung disease. Echocardiographic evaluation revealed a pericardial effusion that was tapped. Soon after this procedure her respiratory status degenerated and she required intubation. Despite aggressive supportive treatment for her cardiac and pulmonary problems, she could not be weaned from the ventilator. Two weeks after admission she became febrile and Gram positive cocci were isolated from sputum culture. She was placed on antibiotics but her condition deteriorated and she developed bradycardia followed by electromechanical dissociation (EMD).

Autopsy Findings

Upon opening the thorax there was 600 cc of cloudy serous fluid in each hemithorax and 100 cc of similar fluid in the pericardial sac. The heart weighed 530 grams and there was thickening of both the left and right ventricular walls. The liver weighed 1880 grams and was congested. The spleen weighed 200 grams and was also congested. The combined lung weight was 1875 grams; the lungs were markedly fibrotic with severe emphysema. In addition, dermal thickening was evident throughout the body and the wall of the esophagus was thickened and firm.

Images

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  This is a gross photograph of cut section of the lungs from this patient. Note the extensive fibrosis of the lung parenchyma.

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  This is a gross photograph of a cut section of one lung from this patient. Note the extensive fibrosis lower lobe (arrows).

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  This is a closer view of the cut section of lung from this patient. Note the extensive fibrosis and the severe emphysematous changes.

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  This is a closer view of the cut section of lung from this patient showing the extensive fibrosis and the severe emphysematous change.

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  This is a gross photograph of the heart from this case. There is thickening of the left ventricular wall and some thickening of the right ventricle as well.

Study Questions

• What organs are usually affected in patients with scleroderma (progressive systemic sclerosis, PSS)?

Skin, gastrointestinal tract, kidneys, heart, muscles, and lungs.

• What are the common clinical ramifications of PSS?

The striking cutaneous involvement is the primary clinical problem in many early cases. Raynaud's phenomenon, dysphagia due to esophageal fibrosis and hypomotility, abdominal pain, and intestinal obstruction are caused by fibrosis and scaring of these tissues. The respiratory problems are caused by pulmonary fibrosis. Chronic pulmonary fibrosis may lead to right-sided heart failure.

• What is the etiology of PSS?

The exact etiology of PSS is not known. PSS is associated with excessive fibrosis, changes in the microvasculature, and a variety of immunologic abnormalities including T cell and humoral abnormalities that either cause or are caused by cytokines. The antigens that trigger the immune response have not been identified.

• What is the CREST syndrome?

Some patients with a milder form of PSS may demonstrate the CREST syndrome. CREST stands for calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, telangiectasia, and the presence of anticentromere antibodies. These patients usually have less skin involvement of skin, and the more serious visceral problems do not develop until later in the course of the disease.

Additional Resources

Reference

• eMedicine Medical Library: Scleroderma
• eMedicine Medical Library: CREST Syndrome
• Merck Manual: Systemic Sclerosis