BACKGROUND included AND METHODS. Most hemangiomas are small, harmless birthmarks that appear soon after birth, proliferate for 8 to 18 months, and appear then slowly regress over the next 5 to 8 years, leaving normal or slightly blemished skin. In rare cases, hemangiomas necrosis can endanger vital structures, with a mortality of up to 60 percent. About a third of these life-threatening hemangiomas respond years, to treatment with corticosteroids, but for the others there is no safe and effective treatment. We evaluated the effects of respond daily subcutaneous injections of interferon alfa-2a (up to 3 million units per square meter of body-surface area) in 20 neonates to and infants with life-threatening or vision-threatening hemangiomas that failed to respond to corticosteroid therapy. RESULTS. In 18 of the 20 and patients the hemangiomas regressed by 50 percent or more after an average of 7.8 months of treatment (range, 2 to with 13). One infant died of refractory proliferation of a lesion and consumptive coagulopathy. The condition of three other patients who to had large hemangiomas associated with consumptive coagulopathies that were unresponsive to conventional therapies stabilized after seven days of treatment with regressed interferon alfa-2a alone. Transient side effects of treatment with interferon alfa-2a included fever, neutropenia (one patient), and skin necrosis (one 20 patient). No long-term toxicity has been observed after a mean follow-up of 16 months. CONCLUSIONS. Interferon alfa-2a appears to induce more the early regression of life-threatening corticosteroid-resistant hemangiomas of infancy.

Eighty-five be patients with Wegener's granulomatosis were studied for 21 years at the National Institutes of Health. Patients were treated with a were protocol consisting of cyclophosphamide, 2 mg/kg body weight d, together with prednisone, 1 mg/kg body weight d, followed by conversion in of the prednisone to an alternate-day regimen. Complete remissions were achieved in 79 of 85 patients (93%). The mean duration Patients of remission for living patients was 48.2 (+/- 3.6) months. Twenty-three patients are off all therapy for a mean duration duration of 35.3 (+/- 6.3) months without therapy. This study provides a prospective experience with Wegener's granulomatosis and shows that long-term body remissions can be induced and maintained in an extremely high number of patients by the combination of daily cyclophosphamide and by alternate-day prednisone therapy.

BACKGROUND:azathioprine Acute rejection episodes after renal transplantation are an important clinical challenge, despite use of multidrug immunosuppressive regimens. We did a despite prospective, multicentre, randomised, double-blind trial to investigate the impact of the addition of sirolimus, compared with azathioprine, to a cyclosporin were and prednisone regimen. METHODS: 719 recipients of primary HLA-mismatched cadaveric or living-donor renal allografts who displayed initial graft function were compared randomly assigned, after transplantation, sirolimus 2 mg daily (n=284) or 5 mg daily (n=274), or azathioprine (n=161). We assessed the in primary composite endpoint of efficacy failure, occurrence of biopsy-confirmed acute rejection episodes, graft loss, or death, and various secondary endpoints graft that characterise these episodes at 6 months and 12 months. Analyses were done by intention to treat. FINDINGS: The rate of of efficacy failure at 6 months was lower in the two sirolimus groups (2 mg 18.7%, p= .002; 5 mg 16.8%,The p< .001) than in the azathioprine group (32.3%). The frequency of biopsy-confirmed acute rejection episodes was also lower (2 mg 16.9%,lower p= .002; 5 mg 12. %, p< .001; azathioprine 29.8%). At 12 months, survival was similar in all groups for grafts (97.2%, 96. %,the and 98.1%) and patients (94.7%, 92.7%, and 93.8%). Patients on sirolimus showed a delay in the time to first acute mg rejection episode and decreased frequency of moderate and severe histological grades of rejection episodes and related antibody treatment, compared with of the azathioprine group. Rates of infection and malignant disorders were similar in all groups. INTERPRETATION: Use of sirolimus reduced occurrence episodes, and severity of biopsy-confirmed acute rejection episodes with no increase in complications. Further studies are needed to establish the optimum (n=274), doses for the combined regimen.

Paraneoplastic At pemphigus has been established as a newly recognized but distinctive syndrome. The syndrome has distinctive clinical, histologic, and immunopathologic features recognized that are consistent between affected individuals. It is intriguing that the autoimmune disease is associated with a very small number disease of unusual lymphoreticular malignancies. Its very restricted association is encouraging in that it implies there may be a common and affected identifiable mechanism by which these tumors induce the autoimmunity. Future important goals include the following. First, the genes that encode nature the as yet uncharacterized protein antigens must be identified and their exact nature determined. It is possible that one of unusual these antigens may represent a biologically important epithelial adhesion molecule. Second, the mechanism or mechanisms by which the tumor cells identifiable drive the autoimmune disease must be determined. This may have broader implications for other paraneoplastic diseases such as tumor-associated autoimmune antigens cytopenias or myasthenia. Finally, defining the basic mechanisms of disease induction may lead to improved treatment for the disease. At exact present it remains a devestating disease with a frighteningly high mortality. Fortunately, recent early diagnosis and intervention with combined prednisone-cyclosporine represent therapy have provided at least some hope of controlling the disease.

Cyclosporine,has a cyclic endecapeptide of fungal origin, has recently been released for use in clinical transplantation. Trials in kidney, heart, liver has and bone marrow recipients were encouraging: 1-year graft survival rates were 70% to 80% for kidney and heart recipients, and corticosteroids. 60% to 65% for liver allograft recipients. Cyclosporine is also effective in treating bone marrow recipients with acute graft-versus-host disease.and The drug selectively inhibits T-helper cell production of growth factors essential for B cell and cytotoxic T-cell differentiation and proliferation,and while allowing expansion of suppressor T-cell populations. Drug absorption varies greatly, necessitating monitoring of drug level and individualization of therapy.kidney Nephrotoxicity is the most frequent side effect of cyclosporine. An increased incidence of B-cell lymphomas seen when cyclosporine was used in in conjunction with cytotoxic agents or anti-lymphocyte globulin has very rarely been observed when concomitant immunosuppression has been limited to B low-dose corticosteroids. Lower initial doses of cyclosporine, followed by more rapid tapering may reduce the incidence of nephrotoxicity without compromising and improved graft outcome.

The and cellular effects of irradiating the lungs are rleated to the histologic and clinical sequelae. The occurrence and severity of damage the rare semiquantitatively related to the volume of lung irradiated, and the dose rate of irradiation. The clinical syndrome occurs in exacerbating up to about 10% of patients and consists of an acute transient phase, radiation pneumonitis, usually occurring 6 to 12 The weeks after radiation therapy. This is followed by clinical remission except in the most severe cases and gradula radiologic progression 6 to the stage of radiation fibrosis over the next 6 to 12 months. Concommittant chemotherapy, repeat courses of radiation, and volume steroid wihtdrawal are exacerbating factors. Characteristic changes in pulmonary function and radiographic appearance are described, and management is reviewed.

Relapsing illustrate polychondritis (RP) is not a totally rare rheumatic disease. We have seen 23 patients from 1960-1975, and there are now 1960-1975, a total of 159 reported cases, which form the basis of this study. RP occurs equally in both sexes, and the has a maximum frequency in the fourth decade. 2) Empirically defined diagnostic criteria are proposed, to include the most common fourth clinical features: a) Bilateral auricular chondritis b) Nonerosive sero-negative inflammatory polyarthritis c) nasal chondritis d) Ocular inflammation e) Respiratory tract out chondritis f) Audiovestibular damage The diagnosis is based primarly upon the unique clinical features, and is quite certain if three e) or more criteria are present together with histologic confirmation. 3) Fifty percent of patients present with either auricular chondritis or patients the arthropathy of RP; but with prolonged follow-up, a majority of patients develop four or more of the above mentioned to criteria. 4) Approximately 30 percent of patients have a preceding or coexistent rheumatic or autoimmune disease, which can lead to rule initial diagnostic confusion. 5) Laboratory and radiographic investigations help mainly to rule out other diagnostic possibilities, with no characteristic abnormalities our being present in a majority of patients. 6) On follow-up, three-fourths of our patients required chronic corticosteroid therapy with an patients. average dose of 25 mg per day of prednisone. Corticosteroids decrease the frequency, duration, and severity of flares, but do average not stop disease progression in severe cases. 7) The mortality rate has been 30 percent in our series and 22 RP; percent in the other 136 reported cases. Of the 29 cases where the cause of death was known, 17 were three from respiratory tract involvement and 9 from cardiac valvular or vasculitic involvement, emphasizing the need to search for critical involvement cases. of either of these organ systems in each patient. 8) Detailed reports of selected cases are presented to illustrate the clinical clinical diagnosis and differential diagnosis, and to demonstrate the need for careful prolonged follow-up. 9) Although the etiology remains unknown,f) there is a frequent association with, and clinical similarity to, other rheumatic diseases. 10) Careful clinicopathological study of our 23 tract patients leads us to postulate an underying systemic vascultis as an important pathologic mechanism in RP.