Chronic systemic capillary leak syndrome. Report of a case and review of the literature.
Systemic capillary leak syndrome (SCLS) is a rare condition characterized by recurrent episodes of generalized oedema and severe hypotension, associated with paraproteinaemia. In addition to the acute form, a few cases of chronic SCLS have been reported. We describe a 64-year-old woman who was hospitalized because of a 6-month history of progressive generalized oedema with pericardial and pleural effusions, associated with a serum paraprotein. Clinical and laboratory findings were consistent with a chronic form of SCLS. Treatment with prednisone, furosemide and theophylline was started, which led to a gradual improvement in 2 weeks and a persistent remission after 9 months. This report indicates that SCLS may occur in a chronic form, which seems to be responsive to a therapeutic regimen with prednisone, furosemide, and theophylline.
Airaghi
L
L
Third Division of Internal Medicine and the Division of Thoracic Surgery, IRCCS Ospedale Maggiore Policlinico, Milan, Italy. lairagh@tin.it
Montori
D
D
Santambrogio
L
L
Miadonna
A
A
Tedeschi
A
A
D019559:Capillary Leak Syndrome / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*; D002908:Chronic Disease; D003937:Diagnosis, Differential; D004232:Diuretics / Q000627:therapeutic use; D004487:Edema / Q000209:etiology; D005260:Female; D005665:Furosemide / Q000627:therapeutic use; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D008875:Middle Aged; D010265:Paraproteinemias / Q000209:etiology; D010490:Pericardial Effusion / Q000209:etiology; D010996:Pleural Effusion / Q000209:etiology; D011241:Prednisone / Q000627:therapeutic use; D013806:Theophylline / Q000627:therapeutic use; D014665:Vasodilator Agents / Q000627:therapeutic use
D004232:Diuretics; D005938:Glucocorticoids; D014665:Vasodilator Agents; D005665:Furosemide; D013806:Theophylline; D011241:Prednisone