Progressive systemic sclerosis complicated by diffuse pulmonary haemorrhage.

Kallenbach, J; Prinsloo, Isebrand; S, Zwi

doi:10.1136/thx.32.6.767

Cited by 35 publications

(9 citation statements)

References 16 publications

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“…Kallenbach et al (1977) have stressed the association of IPH with various connective tissue and allergic disorders, such as rheumatoid arthritis, systemic sclerosis, systemic lupus erythematus, immune complex nephritis, polyarteritis, and Wegener's granulomatosis. Vascular damage to the lung, in the form of haemorrhage, might be recognised more often if unexplained falls in haemoglobin concentrations or shadows on the chest radiograph were accompanied by measurements of pulmonary diffusing capacity.…”

Section: Discussionmentioning

confidence: 99%

Interpretation of increases in the transfer coefficient for carbon monoxide (TLCO/VA or KCO).

Lipscomb¹,

Patel²,

Hughes³

1978

Thorax

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Section: Discussionmentioning

confidence: 99%

Interpretation of increases in the transfer coefficient for carbon monoxide (TLCO/VA or KCO).

Lipscomb¹,

Patel²,

Hughes³

1978

Thorax

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“… First, patients with connective tissue diseases, such as systemic sclerosis [5–12] or lupus erythematosus [13–17], in whom the risk of gastrointestinal bleeding or pulmonary bleeding may be higher compared with IPAH patients [2]. Second, patients with PAH related to congenital heart disease (CHD) may be at some increased risk of haemoptysis [18,19].…”

Section: Introductionmentioning

confidence: 99%

Bleeding events in pulmonary arterial hypertension

Opitz

Kirch²,

Mueller³

et al. 2009

Eur J Clin Investigation

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Despite limited evidence from clinical studies, anticoagulant drugs such as vitamin K antagonists (VKA) (e.g., warfarin or phenprocoumon) are widely used in the background treatment of patients with pulmonary arterial hypertension (PAH). According to current guidelines, they are generally accepted as efficacious drugs, although their efficacy is neither supported by randomised controlled trials, nor formally approved by regulatory agencies for use in the specific PAH indication. The use of these drugs is not without problems, as a paradoxical situation has to be managed in the treatment of this condition. On one hand, thrombosis is one of the key pathophysiologic features of PAH (besides vasoconstriction, proliferation and inflammation). On the other hand, the incidence of bleeding events is increased in PAH patients. This applies particularly to PAH that is related to connective tissue diseases, congenital heart disease and chronic thromboembolic pulmonary hypertension. In patients receiving VKA, caution must be observed in particular when concomitantly using prostanoids or sildenafil. Similarly, VKA doses have to be adjusted according to the labelling when using sitaxentan concomitantly. Regular International Normalized Ratio monitoring contributes to the safety of PAH patients on VKA.

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“…8), both of which are very rare in scleroderma. 37 Other pulmonary changes in scleroderma include hemorrhage, 38 capillaritis, 39 and diffuse alveolar dam-age. 40 Damage to the capillaries leads to diffuse devascularization of the lungs and other organs.…”

Section: Pathological Discussionmentioning

confidence: 99%

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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Progressive systemic sclerosis complicated by diffuse pulmonary haemorrhage.

Cited by 35 publications

References 16 publications

Interpretation of increases in the transfer coefficient for carbon monoxide (TLCO/VA or KCO).

Interpretation of increases in the transfer coefficient for carbon monoxide (TLCO/VA or KCO).

Bleeding events in pulmonary arterial hypertension

Case 4-1999

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