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→Autopsy Findings
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).
== Images ==
File:IPLab6Scleroderma5.jpg|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.
</gallery>
== Virtual Microscopy ==
=== Lung: Scleroderma ===
<peir-vm>IPLab6Scleroderma</peir-vm>
=== Normal Lung ===
<peir-vm>UAB-Histology-00107</peir-vm>
=== Skin: Scleroderma ===
<peir-vm>IPLab6Scleroderma_Skin</peir-vm>
=== Normal Skin ===
<peir-vm>UAB-Histology-00004</peir-vm>
== Study Questions ==
* <spoiler text="What organs are usually affected in patients with scleroderma (progressive systemic sclerosis, PSS)?">Skin, gastrointestinal tract, kidneys, heart, muscles, and lungs.</spoiler>
* <spoiler text="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.</spoiler>
* <spoiler text="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.</spoiler>
* <spoiler text="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.
</spoiler>
== Additional Resources ==
=== Reference ===
* [http://emedicine.medscape.com/article/331864-overview eMedicine Medical Library: Scleroderma]
* [http://emedicine.medscape.com/article/1064663-overview eMedicine Medical Library: CREST Syndrome]
* [http://www.merckmanuals.com/professional/musculoskeletal_and_connective_tissue_disorders/autoimmune_rheumatic_disorders/systemic_sclerosis.html Merck Manual: Systemic Sclerosis]
=== Images ===
* [{{SERVER}}/library/index.php?/tags/2144-scleroderma PEIR Digital Library: Scleroderma Images]
== Related IPLab Cases ==
* [[IPLab:Lab 2:Hypertrophy|Lab 2: Heart: Myocardial Hypertrophy]]
* [[IPLab:Lab 5:α1 Antitrypsin Deficiency|Lab 5: Lung: α1-Antitrypsin Deficiency]]
{{IPLab 6}}
[[Category: IPLab:Lab 6]]