| Literature DB >> 6424415 |
Abstract
As is apparent from the foregoing, there is only one etiologic variant of CAH for which specific therapy is available, namely, that associated with Wilson's disease. However, Wilson's disease is responsible for only a minute portion of the total cases of CAH. Drug-induced CAH also seems highly responsive to appropriate management--in this instance, removal of an offending agent rather than administration of a therapeutic drug. However, as stated, it too is an infrequent contributor to the CAH pool. Among the remaining forms of CAH, a reasonably consistent response to treatment can be expected only from patients with autoimmune CAH. This entity is a serious disorder with unequivocally high morbidity and mortality and thus clearly warrants treatment. Despite the considerable side effects that invariably result from the long-term use of corticosteroids--the only available, although nonspecific, form of treatment--corticosteroid use is justified and indeed recommended. Current evidence, derived from the Mayo Clinic data, suggests that the best therapeutic approach is to use both corticosteroids and azathioprine, a combination that offers the highest therapeutic index with the lowest rate of side effects. Using this regimen, complete remission is reported to result in 65% of cases within 2-3 years, although a considerable proportion of these individuals relapse and require retreatment. Much publicity has surrounded the Mayo Clinic and Royal Free Hospital treatment trials; however, it is probable that autoimmune CAH represents far less than 20% of all cases, and that severe disease requiring corticosteroid therapy comprises but a minor fraction of these. Thus, the bulk of cases of CAH in the United States occur in patients with either established or inferred viral-related disease, the group for which clearly effective therapy is not yet available. Most of these persons are asymptomatic, their disease having been detected through routine screening programs or at the time of evaluation of other disorders. Much interest is evoked, at present, by the new experimental forms of treatment, but none has proved to be consistently effective, and for some, toxicity is high. All appear to reduce levels of replicating virus, but none clearly affects HBsAg or disease activity. Research in this area continues, with highest expectations of success for the use of combination therapy.(ABSTRACT TRUNCATED AT 400 WORDS)Entities:
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Year: 1984 PMID: 6424415
Source DB: PubMed Journal: Adv Intern Med ISSN: 0065-2822