Both autosomal dominant and recessive polycystic kidney disease are conditions with severe associated morbidity and mortality. Recent advances in the understanding of the genetic and molecular pathogenesis of both ADPKD and ARPKD have resulted in new, targeted therapies designed to disrupt cell signaling pathways responsible for the abnormal cell proliferation, dedifferentiation, apoptosis, and fluid secretion characteristic of the disease. Herein we review the current understanding of the pathophysiology of these conditions, as well as the current treatments derived from our understanding of the mechanisms of these diseases.
IntroductionSarcoidosis is a systemic granulomatous disease of unknown cause. The management of sarcoidosis remains problematic. Systemic and topical corticosteroids are the mainstay of therapy but may cause unacceptable side effects. Biologic therapies, such as infliximab, have recently been proposed as another treatment option for cutaneous sarcoidosis.Case ReportThe authors describe three patients who were diagnosed with cutaneous sarcoidosis with systemic involvement. All of the patients were refractory to conventional therapies but responded to infliximab therapy.ConclusionInfliximab is an alternative medication for refractory sarcoidosis that has a relatively benign side-effect profile. However, definite indications, dosage, interval, and duration of treatment for cutaneous sarcoidosis are not firmly established.
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