CREST syndrome

Connective Tissue Diseases. Scleroderma is the connective tissue disease most frequently associated with intestinal dysmotility and bacterial overgrowth [159, 160]. Although the motility of the esophagus is most frequently affected, and a prerequisite for the label CREST syndrome, small bowel involvement is seen in a proportion of these patients. When present, intestinal clearance is usually impaired because of shallow contractions resulting in ineffective peristalsis and clearance. This can lead to overgrowth with Gram-negative bacilli, in part responsible for the malabsorption [161]. 

Four women with CREST syndrome or systemic sclerosis had pain and eventually contracture of the masseter muscles during infusion of iloprost for severe attacks of Raynaud s phenomenon (9). The adverse effect was quickly reversed by reducing the infusion rate. There were no electrocardiographic or cardiac enzyme changes. The mechanism of this effect is obscure. 

Martin Gabriel, J.C., Solis Herruzo, J.A. Primary biliary cirrhosis and CREST syndrome (case report). Rev. Esp. Enferm. Dig. 2004 96 219-220... 

A 58-year-old Japanese woman with CREST syndrome (calcinosis, Raynaud s syndrome, esophageal sclerosis, sclerodactyly, and telangiectasia) developed progressive renal dysfunction (16). Renal biopsy showed changes typical of Chinese herb nephropathy. Analyses of Chinese herbs she had taken for several years demonstrated the presence of aristolochic acid. Oral prednisolone improved her renal function and anemia. 

Nishimagi E, Kawaguchi Y, Terai C, Kajiyama H, Hara M, Kamatani N. Progressive interstitial renal fibrosis due to Chinese herbs in a patient with calcinosis Raynaud esophageal sclerodactyly telangiectasia (CREST) syndrome. Intern Med 2001 40(10) 1059-63. 

The cause of systemic sclerosis is unknown. Ninety-five percent of patients have identifiable autoantibodies. There are two major subsets of the disease, fimited cutaneous and diffuse systemic sclerosis. Patients with limited cutaneous involvement often have the CREST syndrome (calcinosis, Raynaud s, esophageal dysmotility, sclerodactyly, and felangiectasias), whereas patients with diffuse systemic sclerosis have a more aggressive disease with renal, cardiac, or pulmonary involvement. The prevalence of the disease is estimated to be between 138 and 286 cases per 1 million population. The wide range may be due to differences in diagnostic criteria, regional variation, or sample sizes used to estimate the prevalence. 

Yousem SA. The pulmonary pathologic manifestations of the CREST syndrome. Hum Pathol 1990 21 467-474. 

Anti-centromere antibodies react with antigen located in the kinetochore region of chromosomes and are strongly associated with CREST syndrome (i.e. calcinosis -i- Raynaud phenomenon + oesophageal hypomotility -I- skin abnormalities -i- telangiectasia). Those antibodies are only found in 10% of patients with diffuse disease. 

Malignancies, particularly of neural crest origin, are known to affect brain function adversely through remote (presumably hormonal) effects on neural tissue. For example, ovarian adenocarcinoma can selectively induce a profound cerebellar syndrome caused by the selective death of Purkinje cells (presumably from a neurotoxic hormonal factor). Such phenomena simply illustrate the complicated nature of CNS functioning and the need to be cautious about explanations in the absence of systematic data. 

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