Cases reported "Scleroderma, Systemic"

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1/27. Neuromotor disorders of the esophagus.

    Esophageal motility studies are helpful in diagnosing hypertensive and hypotensive disorders of the esophagus and its sphincters, including the exact measurement of the strength of contraction, temporal sequence and duration of the pathophysiology involved. In addition, the assessment of the extent of neuromotor involvement may be of great help to the surgeon in planning a myotomy. PH metering is probably the most accurate way to assess reflux in hypotonic states.
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2/27. Hypothenar hammer syndrome followed by systemic sclerosis.

    We describe the first case of bilateral hypothenar hammer syndrome (HHS) followed by systemic sclerosis (SSc) that was associated with silica exposure (Erasmus syndrome). The patient was a woman smoothing tiles in an earthenware factory who presented with bilateral digital ischemia associated with Raynaud's phenomenon. HHS was diagnosed, based on an angiographic study showing aneurysm of the ulnar arteries and occlusions of multiple digital arteries. Pulmonary silicosis was also diagnosed on pulmonary tomodensitometry. Two years later digital swelling with acroosteolysis developed. The FANA test was positive (titer 1:640) and anticentromere antibody tests were also positive. Esophageal manometry showed dysmotility of the lower esophagus. These findings were consistent with a diagnosis of SSc.
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3/27. The watermelon rectum.

    This is the first reported case of rectal vascular ectasia in association with gastric antral vascular ectasia (watermelon stomach). The patient, a 39-year-old woman with systemic sclerosis, reported chronic gastrointestinal blood loss and iron deficiency anemia. She had aperistalsis of the distal esophagus, decreased lower esophageal sphincter pressure leading to severe gastroesophageal reflux, and dysmotility of the small intestine. diagnosis was made by endoscopic appearance of visible linear watermelon-like vascular stripes in the gastric antrum and rectum. histology confirmed the vascular nature of this disorder, showing dilated and thrombosed capillaries in the lamina propria. Gastric biopsy showed associated fibromuscular hyperplasia in the lamina propria. However, the colonic biopsy showed only focal hyalinization in the lamina propria. The patient was successfully treated using endoscopic bipolar electrocautery. Watermelon stomach has previously been described in association with systemic sclerosis. Based on the histopathologic similarity between rectal vascular ectasia, gastric antral vascular ectasia, and systemic sclerosis, we suggest that the rectal vascular lesion may represent a component of the gastrointestinal manifestations of systemic sclerosis.
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4/27. air esophagogram and intestinal pseudoocclusion in a patient with scleroderma.

    air in the esophagus is unusual because it is collapsible. Its finding on a chest roentgenogram, particularly when not associated with a fluid level indicative of stricture, should strongly suggest systemic sclerosis (scleroderma). We describe a patient with scleroderma with intestinal pseudoocclusion and an air esophagogram. Study of chest roentgenograms of 83 patients with scleroderma, including those of 7 with pseudoocclusion, revealed no other instance of air esophagogram. This radiological sign, although rare, should suggest scleroderma and may be particularly useful in patients with "systemic sclerosis sine scleroderma."
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5/27. Primary biliary cirrhosis and scleroderma complicated by Barrett's oesophagus. A case report.

    Oesophageal problems are common in patients with scleroderma, but the association of primary biliary cirrhosis and scleroderma is uncommon. A Barrett's oesophagus identified in a patient with primary biliary cirrhosis and scleroderma is described. The Barrett's oesophagus was probably a complication of scleroderma and resulted from low lower-oesophageal sphincter pressure and severe gastro-oesophageal reflux.
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6/27. Scleroderma esophagus.

    Scleroderma (systemic sclerosis) is a connective tissue disorder characterized by thickening and fibrosis of the skin and visceral involvement that may include the heart, lungs, kidneys, and gastrointestinal tract. At least 40-50% of patients with scleroderma experience esophageal symptoms such as heartburn and dysphagia, while up to 90% of patients have esophageal dysfunction on objective testing at some point in their disease. The disease results in smooth muscle dysfunction that causes esophageal aperistalsis and reduced lower esophageal sphincter pressures. gastroesophageal reflux with poor acid clearance results with an increased incidence of complications such as peptic stricture and Barrett's esophagus. Aggressive medical therapy is necessary to prevent these and other complications of gastroesophageal reflux.
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7/27. Successful treatment of esophageal dysmotility and Raynaud's phenomenon in systemic sclerosis and achalasia by transcutaneous nerve stimulation. Increase in plasma VIP concentration.

    Dysphagia has been successfully treated by low-frequency transcutaneous nerve stimulation (TNS) in two patients with achalasia and in six patients with systemic sclerosis. A 30- to 45-min stimulation session was followed by augmentation of peristalsis in the lower half of the esophagus and relaxation of the gastroesophageal sphincter, with relief of dysphagia. The sclerotic patients were also relieved of invaliding Raynaud's phenomenon. One 30-min daily stimulation session, and later one session every 2nd or 3rd day, was sufficient to prevent relapse. After months or years of TNS treatment the stimulation could in three patients be withdrawn with no recurrence. A stimulation session produced about 30% increase in plasma vasoactive intestinal polypeptides. Activation of this neuromodulator is considered to be the cause of the beneficial effects on dysphagia and Raynaud's phenomenon.
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8/27. Esophageal-atrial fistula.

    We report an unusual case of an esophageal-atrial fistula in a patient with CREST (calcinosis, Raynaud's phenomenon, esophagitis, sclerodactyly, telangiectasia) variant of scleroderma. An ulcer in Barrett's esophagus perforated into the left atrium and led to systemic embolization and cerebral abscess. A review of similar reports of esophageal-atrial fistula reveals a symptom complex that includes chronic esophageal pathology, gastrointestinal bleeding, and neurological signs. An antemortem diagnosis has never been made.
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9/27. megacolon in a collagen vascular overlap syndrome.

    mixed connective tissue disease is a syndrome having clinical features of scleroderma, systemic lupus erythematosus, and polymyositis with a unique combination of clinical and laboratory findings. Not all patients with clinical features of more than one connective tissue disease fit the mixed connective disorders category; therefore, the term "overlap syndrome" is applied to patients with features of two or more connective tissue diseases. Gastrointestinal involvement in progressive systemic sclerosis is common, with the esophagus being the usual site, followed by the small bowel, colon, and stomach. Colonic involvement is reported to occur in 10 to 50 percent of cases and is typically manifested radiographically as wide-mouthed diverticular saculations. Symptoms secondary to colonic involvement are rare but occasionally serious, such as impaction of barium or feces. A case is described in which systemic lupus erythematosus coexisted with previously undiagnosed scleroderma involving predominantly the gastrointestinal tract and resulting in megacolon.
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10/27. Progressive systemic sclerosis with the nephrotic syndrome and acquired factor X deficiency.

    A patient with progressive systemic sclerosis (PSS) involving skin, digit, esophagus, and lung developed the nephrotic syndrome and rapidly progressive renal insufficiency. Renal pathology revealed capillary collapse, cellular crescents, arteriolar hyaline deposition, and mesangial proliferation. On immunofluorescence IgM, C3, and fibrinogen were present in mesangium and capillary walls. Prebiopsy coagulation screening revealed a factor x deficiency which caused substantial prolongation of the partial thromboplastin time without an overt bleeding diathesis. The acquired factor x deficiency resolved after fresh frozen plasma and vitamin k administration, although some spontaneous improvement was noted. nephrotic syndrome may occasionally be seen in the acute fulminant form of PSS and should not deter diagnosis of PSS.
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