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Biomedical subjects

A G Pandya

Publications and source records attributed to A G Pandya.

24 records · Page 2Linked to original sources

Papulonodular mucinosis associated with systemic lupus erythematosus: possible mechanisms of increased glycosaminoglycan accumulation.

BACKGROUND: The mechanism for the production of papulonodular mucinosis in patients with lupus erythematosus (LE) is not known. OBJECTIVE: Our purpose was to determine whether fibroblasts in a patient with LE and papulonodular mucinosis produced more mucin than normal fibroblasts and whether this mucin production could be stimulated by the patient's serum. METHODS: Skin fibroblasts from a patient with systemic LE and massive papulonodular mucin deposition, as well as normal fibroblasts, were incubated in the presence of serum from the patient or from a healthy volunteer. The production of glycosaminoglycan by fibroblasts was analyzed. RESULTS: Fibroblasts from the patient produced more glycosaminoglycan than did normal fibroblasts. Glycosaminoglycan production was increased in all cells when incubated in the presence of the patient's serum. CONCLUSION: Cutaneous mucin deposition in patients with papulonodular LE skin lesions is associated with increased glycosaminoglycan production by dermal fibroblasts. Our preliminary observations suggest glycosaminoglycan production by these fibroblasts appears to be stimulated by a factor, (or factors) in the patient's serum that is yet to be identified.

Adult↗

Treatment of dermatomyositis with methotrexate.

BACKGROUND: No published data exist on the incidence of liver fibrosis in patients with dermatomyositis treated with methotrexate. OBJECTIVE: Our purpose was to examine the efficacy, steroid-sparing potential, and side effects of methotrexate in patients with dermatomyositis and to report liver biopsy results in four patients. METHODS: A retrospective review of all cases of dermatomyositis treated with methotrexate in a dermatology and rheumatology referral practice was conducted. RESULTS: Of the 10 cases reviewed, seven were of dermatomyositis whereas three were of amyopathic dermatomyositis (ADM). Nine patients received oral methotrexate. One patient received intravenous methotrexate. Improvement of cutaneous disease occurred in seven (100%) of the patients with dermatomyositis and in two (66%) of those with ADM; myositis improved in four (57%) of the patients with dermatomyositis. The initial prednisone dose was halved after an average of 18 weeks of methotrexate therapy in the patients with dermatomyositis and 13 weeks in the patients with ADM. Methotrexate-related side effects occurred in six (86%) of the patients with dermatomyositis and in one (33%) of the patients with ADM. Of the four patients who had liver biopsies, two (50%) showed mild hepatic fibrosis, resulting in discontinuation of the drug. Both patients in whom fibrosis developed had preexisting steroid-induced diabetes mellitus. CONCLUSION: Although methotrexate is an effective treatment for dermatomyositis, side effects are common. Patients with diabetes mellitus should be closely monitored for toxic effects on the liver.

Adult↗

Treatment of pemphigus vulgaris with pulse intravenous cyclophosphamide.

BACKGROUND: Although corticosteroids have dramatically altered the prognosis in pemphigus vulgaris, morbidity and mortality from systemic corticosteroid side effects remains high. While immunosuppressive agents have been successfully used in pemphigus vulgaris, there is a high incidence of side effects with these agents as well. Particularly bothersome are reports of increased risk of malignancy with long-term use of immunosuppressive agents. For these reasons, we used a protocol that includes low-dose oral cyclophosphamide coupled with pulse intravenous cyclophosphamide in two patients with recalcitrant pemphigus vulgaris. OBSERVATIONS: Both patients responded well to monthly doses of intravenous cyclophosphamide with rapid decrease in the frequency and severity of blistering, resulting in resolution of their disease after 7 and 10 months, respectively. CONCLUSIONS: Pulse doses of immunosuppressive agents appear to be successful in the treatment of pemphigus vulgaris. High-dose steroid therapy can be tapered with the use of this treatment. Because monthly intravenous doses of cyclophosphamide lead to a substantially reduced cumulative dose, when compared with standard oral regimens, the risk of developing malignancy may also be reduced. Further studies using larger groups of patients are needed to evaluate the efficacy of pulse intravenous cyclophosphamide in pemphigus vulgaris. Long-term follow up will be necessary to compare the incidence of malignancy in patients receiving pulse doses of immunosuppressive agents with that in patients receiving continuous oral treatment.

Adult↗