ABSTRACT
Generalized edema is commonly due to cardiac failure, renal changes, hepatic and metabolic disturbances, or it can be idiopathic, i.e. primitive lymphedema. Here we describe a patient with several episodes of fluid extravasation characterized by hypotension, hemoconcentration and functional renal insufficiency. These findings, associated to a monoclonal gammopathy, lead to the diagnosis of systemic capillary leak syndrome or Clarkson Syndrome. This rare and perplexing disorder, characterized by a typical three-phase clinical feature, is due to an endothelium permeability alteration, rather responsible of these paroxysmal manifestations. Interleukin-2-pathway is considered as one of the underlying mechanisms. During acute phase the patient underwent therapy with plasma-expanders and glucocorticoids, although in quiescent phase we administered aminophylline, salbutamol and prednisone. After three months, the patient is asymptomatic.
Subject(s)
Angioedema/diagnosis , Capillary Leak Syndrome/diagnosis , Monoclonal Gammopathy of Undetermined Significance/diagnosis , Adrenergic beta-Agonists/therapeutic use , Albuterol/therapeutic use , Aminophylline/therapeutic use , Angioedema/immunology , Angioedema/physiopathology , Angioedema/therapy , Anti-Inflammatory Agents/therapeutic use , Blood Transfusion , Capillary Leak Syndrome/immunology , Capillary Leak Syndrome/physiopathology , Capillary Leak Syndrome/therapy , Capillary Permeability , Cardiotonic Agents/therapeutic use , Drug Therapy, Combination , Humans , Interleukin-2/metabolism , Male , Middle Aged , Monoclonal Gammopathy of Undetermined Significance/immunology , Monoclonal Gammopathy of Undetermined Significance/physiopathology , Monoclonal Gammopathy of Undetermined Significance/therapy , Prednisone/therapeutic use , Treatment OutcomeABSTRACT
Interferon (IFN)-induced sarcoidosis is well documented. Herein, we report the case of a patient with chronic hepatitis C (CHC) who developed IFN-alpha-induced sarcoidosis. The clinical features of this case make it unique among all cases so far described. The patient was, in fact, asymptomatic for sarcoidosis, and the disease, characterized by liver and lung granulomatosis, was discovered by chance during the CHC follow-up. The diagnosis was made 5 years after IFN-alpha discontinuation. A pathogenetic role for IFN-alpha in our patient is supported by a liver biopsy performed before the therapy with IFN-alpha was started, showing no evidence of granulomatous localizations. This case suggests that the incidence of sarcoidosis during IFN-alpha treatment is underestimated. A search for clinical and laboratory findings typical of the disease, as well as a liver biopsy, should always be included in the follow-up of CHC patients undergoing therapy with IFN-alpha.
