PubMed · 3675293
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R M Feldman, R L Gross, R P Wilson, G L Spaeth, R Varma, R C Eagle. 1987. Encapsulated filtering blebs.. https://doi.org/10.1001/archopht.1987.01060110135047
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In pathological conditions, the negative intrapleural pressures (Ppl) may increase unequally. At some sites they may remain increased. We maintain that at these sites the increased pressures may lead to the production of oedema in the adjacent pleural walls and in adjacent pre-existing connective tissue, which may remain as oedema or may become connective tissue. Occasionally the increased negative Ppl may contribute to the production of pleural effusion. We term all these processes the suction mechanism (SM). This mechanism affords an interpretation of a series of images observed in chest X-rays. According to this view, lung shrinkage, narrowing of the pulmonary vessels and impairment of the lung function are not caused by the diffuse pleural fibrosis, but result from damage to the lung parenchyma. We also interpret the apical cap, the blunting of the costophrenic sulci, the broadening of the mediastinum and the retrosternal stripe in terms of the SM.
The intensivist should be aware of the upper airway manifestations of the common rheumatologic disorders which may lead to ICU admission or which may potentially pose a problem during airway management. Information should be obtained from the patient, the patient's family, and the patient's primary physician, if possible. One should be fully prepared with various options in case a problem arises with an airway. Equipment for managing a difficult airway should be available. Alternate methods of managing the airway (e.g., the laryngeal mask airway, fiberoptic scopes, and the WU Scope) (Achi Corporation, Fremont, CA) are of great help in dealing with airway problems. The potential for cervical spine instability exists in patients with rheumatologic disorders. Intubating with care and avoiding spinal movement both seem to be more important than any particular mode of intubation in preserving neurologic function. One should make a concentrated and serious effort to be as gentle as possible and to avoid even minimal trauma to the mucosa in these patients, because they are at risk for mucosal edema and subsequent postextubation stridor. In cases of stridor, helium-oxygen mixtures may be of help and may eliminate the need for reintubation. When difficulty in establishing an airway is anticipated, it is prudent to attempt airway control in the operating room with surgical assistance standing by should cervical tracheotomy is required.
Connective tissue diseases such as systemic lupus erythematosus (SLE), rheumatoid arthritis, Sjögren's syndrome, and scleroderma are systemic disorders that may have an autoimmune basis. The system manifestations vary, and there is frequent overlap among the syndromes. Liver involvement in patients with connective tissue diseases has been well documented but is generally considered rare. Although advanced liver disease with cirrhosis and liver failure is rare in patients with connective tissue diseases, clinical and biochemical evidence of associated liver abnormalities is common. Previous treatment with potentially hepatotoxic drugs or coincident viral hepatitis has usually been implicated as the main causes of liver disease in patients with connective tissue diseases. However, even after careful exclusion of these etiologies, the question remains whether to classify the patient as having a primary liver disease with associated autoimmune, clinical, and laboratory features or as having liver disease as a manifestation of generalized connective tissue disease. The main example of this pathogenetic dilemma is autoimmune hepatitis and SLE-associated hepatitis, which have been regarded as two different entities, although they have features in common of autoimmune syndromes. Several clinical and histopathologic features have been used to discriminate autoimmune hepatitis from SLE, a relevant diagnostic exercise because complications and therapy are quite different. Although hepatic steatosis and abnormal results on biochemical liver function tests are the most common hepatic abnormalities associated with connective tissue diseases, other less frequent abnormalities have been noted, such as nodular regenerative hyperplasia, portal vein obliteration and portal hypertension, features of primary biliary cirrhosis, and rarely portal fibrosis with abnormal lobular architecture. Vascular disorders of the liver also have been described, such as Budd-Chiari syndrome. Histologic assessment may reveal a variety of subclinical liver diseases. The aim of this contribution is to review the current published data regarding liver involvement in connective tissue diseases.