Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The widespread use of corticosteroids in clinical practice emphasises the need for a thorough understanding of their metabolic effects. In general, the actions of corticosteroids on carbohydrate, protein, and lipid metabolism result in increased hepatic capacity for gluconeogenesis and enhanced catabolic actions upon muscle, skin, lymphoid, adipose and connective tissues. Because of the morbidity associated with steroid therapy, the clinician must carefully consider in each case the gains that can reasonably be expected from corticosteroid therapy versus the inevitable undesirable side effects of prolonged therapy. Thus, it is important to remember that the enhanced anti-inflammatory activity of the various synthetic analogues of cortisol is not dissociated from the expected catabolic actions of glucocorticoid hormones. Replacement therapy with physiological doses of cortisol in primary or secondary adrenal insufficiency is intended to simulate the normal daily secretion of cortisol. Short term, high dose suppressive glucocorticoid therapy is indicated in the treatment of medical emergencies such as necrotising vasculitis, status asthmaticus and anaphylactic shock. With improvement of the underlying disorder, the steroid dosage can be rapidly tapered and then discontinued over a 2 to 3 day period. Long term, high dose suppressive therapy is often commonly used to treat certain diseases (see sections 4.7.2 and 4.7.3). In this setting, suppression of the hypothalamic-pituitary-adrenal axis may persist for as long as 9 to 12 months following steroid withdrawal if steroid doses are administered in the supraphysiological range for longer than 2 weeks. In general, higher doses, longer duration of usage, and frequent daily administration are all correlated with the severity of pituitary ACTH suppression. When steroid therapy is to be withdrawn, gradual tapering of the dosage is necessary; the steroid dosage should also be given as a single morning dose if possible. Rapid or total withdrawal of the steroid therapy may be associated with exacerbation of the underlying disease or with a steroid withdrawal syndrome. An additional important point to remember in any withdrawal programme is that the steroid dosage should be appropriately increased for an exacerbation of the underlying disease or for intercurrent major stress. Alternate day therapy is recommended as a steroid maintenance programme for patients requiring high dose glucocorticoid therapy over a prolonged period of time. Thus, it is usually employed to maintain a therapeutic benefit which had previously been extablished by daily steroid treatment. Complications resulting from corticosteroid therapy include: (1) proximal muscle weakness; (2) osteopenia; (3) unmasking of latent diabetes mellitus; (4) sodium retention and/or elevation of mean arterial blood pressure; (5) adverse psychiatric reactions; (6) development of glaucoma; and (7) reactivation of latent infections (such as tuberculosis).
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PMID:Corticosteroids: clinical pharmacology and therapeutic use. 20 58

A 71-year-old male complaining of chest pain was admitted to our hospital. A single cavitary mass shadow was observed on chest X-ray films. Urinalysis revealed microscopic hematuria. CT examination demonstrated a tumorous shadow in the maxillary sinus. The diagnosis of Wegener's granulomatosis was histologically established by biopsy specimens from the nasal mucosa which showed necrotizing vasculitis and granuloma with fibrinoid degeneration. He was treated with combination therapy of prednisolone and cyclophosphamide. The abnormal shadows on chest X-ray and in the maxillary sinus on CT improved rapidly, but the patient developed progressive weight loss and complained of cold intolerance, weakness and dysphagia. Serum T3, T4 and TSH were found to be reduced. Anterior pituitary function tests showed reduction of TSH, GH and ACTH responses, which was probably due to irreversible vasculitis.
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PMID:[A case of Wegener's granulomatosis complicated by hypopituitarism]. 148 37

A radioimmunoassay for circulating alpha-interferon (IFN alpha) has been developed using lymphoblastoid IFN alpha. The assay was specific for IFN alpha, and did not cross-react with IFN beta, IFN gamma, or ACTH, while it was specifically inhibited by recombinant IFN alpha. The radioimmunoassay (y) correlated linearly with the virus inhibition assay (x), with a regression line of y on x of y = 0.659x + 245 (u) (P less than 0.01). alpha-Interferon-like substance (IFN alpha-LS) was extracted and concentrated from plasma either by silicic acid or by antibody immunoadsorption. Serial dilutions of plasma and extracted samples of plasma showed dilution curves identical to those of standard IFN alpha, suggesting the presence of endogenous IFN alpha in human plasma. The circulating IFN alpha-LS of healthy individuals aged 20 to 45 was 0.207 +/- 0.055 ng/ml in males (n = 48) and 0.172 +/- 0.076 ng/ml in females (n = 34). Gel filtration studies on a Sephadex G-75 column suggested that circulating IFN alpha-LS exists in a fragmented form, inactive in virus inhibition assays, in the plasma of healthy individuals. The finding may help explain why biological IFN alpha is often undetectable in the plasma of healthy donors, yet is detectable by radioimmunoassay. Circulating IFN alpha-LS in the plasma of healthy individuals declined gradually with age. IFN alpha-LS was significantly decreased in the plasma of rheumatoid arthritis patients, when compared with the value found in the age and sex-matched healthy controls and in osteoarthritis patients (P less than 0.0001). The decrease was related neither to treatment nor to disease activity. IFN alpha-LS was, however, not decreased in the plasma of vasculitis patients. Decreased IFN alpha-LS in rheumatoid arthritis may be important from pathogenetic and therapeutic standpoints.
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PMID:A sensitive radioimmunoassay for alpha-interferon: circulating alpha-interferon-like substance in the plasma of healthy individuals and rheumatoid arthritis patients. 380 76

Ectopic ACTH syndrome is rarely caused by pheochromocytoma. We report a case of a 28-year-old woman with Cushing's syndrome due to ACTH-producing adrenal pheochromocytoma. She had delivered preterm baby at 32nd week of gestation with 'severe preeclampsia'. After delivery, persistent hypertension accompanied by severe headache led her to being misdiagnosed as Cushing's syndrome due to right adrenal adenoma (normal plasma ACTH level) and cerebral vasculitis of unknown etiology. She was referred to our hospital for surgical treatment. Repeated biochemical studies suggested coexistence of ectopic ACTH syndrome and pheochromocytoma. To reverse her clinical deterioration, right total and left subtotal adrenalectomy was performed with presumptive diagnosis of 1) right adrenal pheochromocytoma causing ectopic ACTH syndrome or 2) coexistence of ACTH-dependent Cushing's syndrome and right adrenal pheochromocytoma. Pathologic examination of right adrenal mass revealed pheochromocytoma which showed strong immunostaining for ACTH. Plasma ACTH and urinary cortisol excretion normalized after surgery, but she succumbed to multiple cerebral infarcts and disseminated intravascular coagulation. Pregnancy and inappropriately low plasma ACTH at initial evaluation might have hampered early diagnosis. To our knowledge, this is the first description of a case with ectopic ACTH syndrome due to pheochromocytoma associated with pregnancy.
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PMID:A case of ACTH-producing pheochromocytoma associated with pregnancy. 1470 46