Neoplasm and systemic vasculitis: a case report.
Explore the source record for details and available documents.
Adult↗
Biomedical subjects
T R Cupps
Publications and source records attributed to T R Cupps.
Corticosteroid-mediated immunoregulation in man.
Glucocorticoids have profound and complex effects on the human immune response. However, the precise mechanisms of the corticosteroid-induced immunoregulation in man have not been precisely defined. Intracytoplasmic corticosteroid-specific receptors appear to be an important common pathway for steroid-induced changes, but variations of receptor parameters do not account for the multifaceted effects on the immune system. Human circulating mononuclear cells redistribute out of the intravascular compartment following treatment with corticosteroids. Although certain components at this redistribution phenomenon have been well-characterized, the importance of this compartmental cellular shift with respect to the mechanisms of corticosteroid-induced immunoregulation are less well-defined. Recent observations that activated lymphocytes may be sensitive to the lytic effects of glucocorticoids suggest that under certain situations the elimination of selected subsets of cells may be a relevant mechanism of corticosteroid-mediated immunoregulation in man. Corticosteroid-mediated effects on monocyte function may be an important mechanism of drug-induced immunoregulation in monocyte-dependent responses. In some experimental conditions, corticosteroids inhibit Interleukin 1 production by monocytes. The immunoregulatory effects of corticosteroids on lymphocyte immune responses are complex. In vitro corticosteroids appear to selectively affect early immunoregulatory events as opposed to altering an established response. Multiple sites of steroid-induced modulations of human B cell responses have been defined.
Animals↗
Suppression of human B lymphocyte function by cyclophosphamide.
The immune responses of 16 patients with nonneoplastic immune mediated diseases including Wegener's granulomatosis, systemic necrotizing vasculitis, cutaneous vasculitis, and relapsing nodular panniculitis were evaluated before and during therapy with chronic low-dose (2 mg/kg/day) cyclophosphamide. A striking selective suppression of B cell function was noted as measured by PWM-induced immunoglobulin secretion. This suppression was a direct effect on the B cells themselves because T cell function, measured by blastogenic responses to the mitogens PHA, Con A, and PWM, was not significantly suppressed. Furthermore, the ability of T cells from cyclophosphamide-treated patients to provide helper function in T cell-dependent B cell assays remained intact. Treated patients manifested a total lymphocytopenia without a selective depletion of relative proportions of B cells or T cell subsets. However, the spontaneous secretion of immunoglobulin by peripheral blood B cells that is elevated in untreated patients was suppressed back to normal levels during cyclophosphamide therapy. This selective effect on spontaneous and induced secretion of immunoglobulin by human B cells may help explain the efficacy of cyclophosphamide therapy in certain antibody and immune complex-mediated diseases.
Adult↗
Successful treatment with acyclovir of an immunodeficient patient infected simultaneously with multiple herpesviruses.
A patient with recurrent simultaneous chronic infections, including cytomegalovirus pneumonia, disseminated zoster and perineal herpes simplex infection, whose immune responses were deficient (immunodeficient), is presented. Following treatment with acyclovir (19-(2-hydroxyethoxymethyl)guanine), this patient had a rapid remission of these viral infections. The patient's clinical improvement is remarkable considering the duration of the viral infections and the continued immune deficiency. Acyclovir appears to act by a highly selective activation by and inhibition of viral enzymes. Prospective trials of this agent in immunosuppressed patients with herpes virus infections seem warranted.
Acyclovir↗
Facial erysipelas in the immunocompromised host. Report of two cases.
Two cases of facial erysipelas in immunologically altered hosts are reported herein. The unusual presentation with absence of erythema in the skin lesion is emphasized. Atypical fever patterns were also noted. In one patient, the facial lesion followed the onset of fever by 48 hours, and, in the other, the facial swelling preceded the fever. Various aspects of the patient's altered host status are discussed in light of the atypical clinical presentation. Recognition of facial erysipelas as a potential source of group A beta-hemolytic streptococcemia in immune-altered hosts is important to ensure rapid and appropriate therapeutic intervention.
Adult↗
Herpes zoster in patients with treated Wegener's granulomatosis. A possible role for cyclophosphamide.
In review of the ongoing protocol for the treatment of Wegener's granulomatosis with cyclophosphamide at te National Institutes of Health, an increased incidence of herpes zoster infection was noted. There were a total of nine episodes in seven of a total of 65 patients with a 255 patient year follow-up. The infections occurred while the patients were in complete clinical remission during immunosuppressive therapy. Cutaneous dissemination was noted in two episodes, but no visceral or central nervous system involvement was noted despite continuation of immunosuppressive therapy. The major causal factor of the increased incidence of herpes zoster appeared to be the cyclophosphamide therapy.
Cyclophosphamide↗
Immunoglobulin replacement therapy by slow subcutaneous infusion.
Explore the source record for details and available documents.
Adult↗
Primary empty sella syndrome with panhypopituitarism, diabetes insipidus, and visual field defects.
A 58 year old woman with a history of hypothyroidism was evaluated for marked visual impairment and found to have the primary empty sella syndrome with multiple endocrine abnormalities. Visual field determination revealed preservation of vision only in the left inferior quadrants bilaterally. Failure of growth hormone (hGH), cortisol and prolactin to respond to insulin induced hypoglycaemia (0.1 U/kg), of luteinizing hormone (LH) and follicle stimulating hormone (FSH) to respond to gonadotrophin releasing hormone (GnRH, 100 microgram) and of thyrotrophin (TSH) and prolactin to increase after thyrotrophin releasing hormone (TRH, 500 microgram), confirmed the diagnosis of panhypopituitarism. Following water deprivation with a 9% loss in body weight, her urine osmolality remained at 204 mOsm./kg H2O), indicating that she had posterior pituitary deficiency as well. During surgical exploration, which was performed in an effort to improve her markedly impaired vision, a compromised vascular supply to the left optic nerve and chronic arachnoiditis was demonstrated. This case represents one extreme of functional impairment in a syndrome which is generally considered benign and which rarely requires therapeutic intervention. Our patient is compared to 29 reported cases of the primary empty sella syndrome with visual field defects. The operative findings in eight of these cases are reviewed. The need for a multidisciplinary approach and close follow-up of patients with an empty sella and functional deficits is emphasized. Surgical intervention including lysis of adhesions and chiasmapexy has been effective in selected cases in reversing or stabilizing visual field abnormalities.
Adolescent↗
Orbital involvement in Wegener granulomatosis: MR findings in 12 patients.
PURPOSE: Our goal was to characterize the MR features of orbital Wegener granulomatosis (WG). METHOD: Twelve patients with pathologically proven WG were studied with MRI. Enhanced and unenhanced T1-weighted sequences with conventional and fat-suppressed techniques were used. T2-weighted images were also obtained. Signal intensity measurements were made in each orbital lesion on the T2-weighted images and compared with that of normal fat. The degree of enhancement was also evaluated by measuring the change in signal intensity of each lesion between the unenhanced and enhanced studies. RESULTS: Seventeen orbital lesions were identified in the 12 patients. Fifteen of the lesions examined by T2-weighted sequences demonstrated a marked decrease in signal intensity. The unenhanced, non-fat-suppressed T1-weighted sequences provided the best contrast between lesion and normal structures. All of the WG lesions enhanced, but the degree of enhancement varied. The enhancement characteristics were best appreciated on the fat-suppressed T1-weighted technique. CONCLUSION: MRI is an excellent imaging modality to evaluate orbital involvement in WG. A marked decrease in the T2 signal is a characteristic feature of this entity. The unenhanced, non-fat-suppressed T1-weighted sequence is the preferred method for lesion detection and for definition of the pattern of anatomic involvement when utilizing MRI.
Adult↗
Corticosteroid-induced modulation of immunoglobulin secretion by human B lymphocytes: potentiation of background mitogenic signals.
The modulation of immunoglobulin (Ig) secretion of human peripheral blood mononuclear cells by in vitro hydrocortisone (HC) was evaluated. A marked enhancement of Ig secretion was observed in unstimulated cultures in the presence of HC as compared to cultures without HC. The augmented response was not due to a direct induction of Ig secretion by HC, but resulted from an enhancement or unveiling of the background mitogenic signal provided by the supplemental serum used in culture. HC-induced augmentation of Ig secretion was only seen in unstimulated cultures performed in fetal calf serum (FCS) which is known to possess mitogenic properties. In contrast, HC had no significant effect on Ig secretion of cultures performed in human serum, which provides little or no background mitogenic signal. On the other hand, no enhancement of Ig secretion by HC was seen in cultures maximally stimulated with pokeweed mitogen regardless of whether FCS or human A serum was used. The mechanisms of this modulation of Ig secretion are unclear at present and may include a synergy between corticosteroids and background mitogenic signals (such as FCS) indirectly triggering B cells and/or the dampening of a negative immunoregulatory effect of an accessory cell.
Adrenal Cortex Hormones↗