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At least 757 records · Page 42Linked to original sources
PubMed1999

[Pulmonary involvement in systemic scleroderma. Part I. Chronic fibrosing interstitial lung disease].

INTRODUCTION: Chronic pulmonary interstitial fibrosis is the most frequent respiratory manifestation in systemic sclerosis, occurring in 80% of cases. It remains a severe complication of the disease and is the primary cause of mortality related to respiratory insufficiency in 20 to 60% of cases. CURRENT KNOWLEDGE AND KEY POINTS: The date of onset of interstitial lung disease remains undetermined, and only in rare cases does it reveal the presence of systemic sclerosis. The clinical signs are only observable at a later stage, when at least 50% of the lung parenchyma is affected. The methods of choice adopted for early diagnosis of this disease are high resolution computed tomography and pulmonary functional investigations; they should be carried out during the preliminary investigation and at follow-up once a year. Moreover, high resolution computed tomography also provides prognostic data, for there is a correlation between the type of lesion and its severity as determined by high resolution computed tomography and by histological findings. The value of other methods of investigation, in particular bronchoalveolar lavage, has not yet been clearly established. The association of cyclophosphamide and corticoids is currently being evaluated (indications, administration modalities, duration), and this combination may be the most effective treatment. FUTURE PROSPECTS AND PROJECTS: Interstitial lung disease is one of the major causes of morbidity and mortality in systemic sclerosis. Early diagnosis and management of this disease is therefore of utmost importance.

Adrenal Cortex Hormones↗
PubMed2002

Primary anti-phospholipid antibody syndrome caused by isolated anti-phosphatidylethanolamine antibodies presenting as cryptogenic fibrosing alveolitis with recurrent pulmonary hemorrhage after single-lung transplantation.

A 28-year-old man underwent a single-lung transplantation for rapidly progressive usual interstitial pneumonitis. Following transplantation, primary anti-phospholipid antibody syndrome was diagnosed after a thromboembolic event and continued dyspnea. Review of his native lung and allograft tissue showed diffuse hemorrhage secondary to capillary injury, probably reflecting anti-phospholipid antibody effects on endothelia.

Adult↗
PubMed2001

Follow-up of a case of retroperitoneal fibroses treated sequencially via right side autotransplant and left uterolysis with wrapper of posterior preperitoneal fat.

We present a case of retroperitoneal fibrosis treated with a renal autotransplantation in a patient inicially treated with endourologic measures and corticotherapy without success. The extense ureteral affectation was the indication to carry out an autotransplant. The follow-up is of nine years, with the function of the renal unity still preserved. We checked the literature for the therapeutic possibilities, medical as much as surgical, of the retroperitoneal fibrosis with special attention to the usage of the refractory IRF to other treatments.

Follow-Up Studies↗
PubMed2001

A phase II trial of pirfenidone (5-methyl-1-phenyl-2-[1H]-pyridone), a novel anti-fibrosing agent, in myelofibrosis with myeloid metaplasia.

The anti-fibrotic and cytokine modulatory properties of pirfenidone suggest its usefulness in the treatment of myelofibrosis with myeloid metaplasia (MMM). In a prospective study, 28 patients with MMM were treated with oral pirfenidone. Twelve patients completed 1 year of therapy; 13 were withdrawn because of disease progression and three because of drug intolerance. Only one patient experienced a clinically relevant benefit with respect to anaemia and splenomegaly. The overall lack of clinical benefit correlated with no significant improvement in the bone marrow morphological features of the disease. We conclude that pirfenidone has no significant clinical or biological activity in MMM.

Administration, Oral↗