"Primary" Pulmonary Hypertension And Raynaud's Phenomenon

Winters, William L.

doi:10.1001/archinte.1964.03860120133017

Cited by 40 publications

(8 citation statements)

References 18 publications

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“…Patients with the CREST syndrome or Raynaud's phenome- non can have the same panoply of vascular abnormalities in the lung. [42][43][44] Moderate or marked pulmonary hypertensive changes were found in 14 of 30 cases of scleroderma studied at autopsy in this hospital. 36 The pulmonary vascular changes observed in the current case were independent of pulmonary interstitial fibrosis, which is the form of the disease most commonly detected radiographically.…”

Section: Pathological Discussionmentioning

confidence: 80%

Case 4-1999

Landzberg¹,

Roberts²,

Mark³

1999

N Engl J Med

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A 38-year-old woman (gravida 3, para 2) was admitted to the hospital because of left-sided chest pain.The patient, who worked as a day-care provider, had been well until seven months earlier, when headaches and pain in the neck and shoulder began. One month later she was discovered to be pregnant. A test for antinuclear antibodies was positive, at a titer of 1:5120, with a nucleolar pattern of staining. Systemic lupus erythematosus was diagnosed, and prednisone was administered. Three months before admission, severe myalgias and progressive dysphagia developed, with a weight loss of about 7 kg.Four days before admission, the patient had sudden chest pain that became more severe and pleuritic and that was accompanied by dyspnea. She was admitted to another hospital. The results of laboratory tests at this time and throughout the course of the illness are presented in Tables 1, 2, and 3. A specimen of arterial blood, drawn while the patient was breathing room air, revealed that the partial pressure of oxygen was 76.3 mm Hg, the partial pressure of carbon dioxide was 32.1 mm Hg, and the pH was 7.44. A radionuclide ventilation-perfusion scan showed mismatch defects in the left lung. A pulmonary angiographic study revealed questionable "pruning" of the distal pulmonary arteries, without evidence of emboli. The pulmonary arterial pressure was 53/24 mm Hg, the right ventricular pressure was 49/9 mm Hg, and the right atrial pressure was 11 mm Hg. The creatine kinase concentration was 1200 U per liter, with an MB isoenzyme fraction of 3 to 4 percent. On the 4th hospital day (during the 24th week of gestation), the patient was transferred to this hospital, after she had received prednisone, aspirin, ranitidine, labetalol, a calcium supplement, and multivitamins.There was a questionable history of blood clots in both legs at the age of 16 years, without known recurrence.The temperature was 37.1°C, the pulse was 108, and the respirations were 26. The blood pressure was 110/70 mm Hg. Physical examination revealed only distant breath sounds, a few crackles at both bases, and a grade 2 systolic murmur. An electrocardiogram showed sinus tachycardia at a rate of 102, with left-axis deviation and minor, nonspecific STsegment and T-wave abnormalities. The results of an echocardiographic examination were normal; the estimated right ventricular systolic pressure was 45 mm Hg.Examination by fiberoptic upper gastrointestinal endoscopy disclosed a nonbleeding gastric ulcer, 6 mm in diameter, located at the gastroesophageal junction and related to a sliding hernia. Pulmonary-function studies were performed. The findings on an obstetrical ultrasonographic evaluation were normal. Microscopical examination of a biopsy specimen of the right quadriceps muscle showed slight myositis.

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Section: Pathological Discussionmentioning

confidence: 80%

Case 4-1999

Landzberg¹,

Roberts²,

Mark³

1999

N Engl J Med

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“…Satoh et al (1988) found that in SLE, only blood pressure correlates well with intimal thickening. Raynaud's phenomenon has been thought to be closely related to pulmonary hypertension (Winters et al 1964); it also decreases the pulmonary compliance (Ploysongsang and Foad 1984). However, all our patients with MCTD had severe Raynaud's phenomenon even in those in whom advanced pulmonary vascular disease did not develop, so it is not reasonable to ascribe the vascular disease to Raynaud's phenomenon alone, even though it may be one of the most important predisposing factors.…”

Section: Interstitiummentioning

confidence: 80%

Morphometric Analysis of the Kidney Lesions in Mixed Connective Tissue Disease(MCTD).

Sawai

Murakami

Kurasono

1994

Tohoku J. Exp. Med.

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“…Systemic hypertension due to the "scleroderma kidney" and PAH in later stages of the disease may effect the cardiac function and lead to heart failure and cor pulmonale [9]. The typical vascular manifestation in PSS is Raynaud's phenomenon, which may persist in peripheral vasculature and pulmonary vascular beds [10][11][12]. Among the leading factors in the formation of impaired pulmonary circulation such as direct vascular wall damage by antibodies mad immune complexes, enhanced sympathetic activity and hypoxia have been shown to play the main role [ 11,[13][14][15].…”

Section: Introductionmentioning

confidence: 99%