Delphine Gobert

A nationwide study of acquired C1-inhibitor deficiency in France: Characteristics and treatment responses in 92 patients.

Abstract Acquired angioedema (AAE) due to C1-inhibitor (C1INH) deficiency is rare. Treatment options for acute attacks are variable and used off-label. Successful treatment of the associated lymphoma with rituximab seems to prevent acute attacks in subjects with AAE. The aim of this study was to describe AAE manifestations, its associated diseases, and patients’ responses to treatments in a representative cohort. A retrospective nationwide study was conducted in France. The inclusion criteria were recurrent angioedema attacks and an acquired decrease in functional C1INH <50% of the reference value. A total of 92 cases were included, with a median age at onset of 62 years. Facial edema and abdominal pain were the most frequent symptoms. Fifteen patients were hospitalized in the intensive care unit because of laryngeal edema, and 1 patient died. Anti-C1INH antibodies were present in 43 patients. The associated diseases were primarily non-Hodgkin lymphoma (n = 44, with 24 splenic marginal zone lymphomas) and monoclonal gammopathy of undetermined significance (n = 24). Three patients had myeloma, 1 had amyloid light-chain (of immunoglobulin) (AL) amyloidosis, 1 patient had a bronchial adenocarcinoma, and 19 patients had no associated disease. Icatibant relieved the symptoms in all treated patients (n = 26), and plasma-derived C1INH concentrate in 19 of 21 treated patients. Six patients experienced thromboembolic events under tranexamic acid prophylaxis. Rituximab prevented angioedema in 27 of 34 patients as a monotherapy or in association with chemotherapy. Splenectomy controlled AAE in 7 patients treated for splenic marginal zone lymphoma. After a median follow-up of 4.2 years, angioedema was on remission in 52 patients. AAE cases are primarily associated with indolent lymphoma—especially splenic marginal zone lymphoma—and monoclonal gammopathy of undetermined significance but not with autoimmune diseases or other conditions. Icatibant and plasma-derived C1INH concentrate control attacks; splenectomy and immunochemotherapy prevent angioedema in lymphoma setting.

Angiœdèmes bradykiniques médicamenteux (inhibiteurs de l’enzyme de conversion et autres traitements)

Angiotensin Converting Enzyme inhibitors (ACE-I) use is a frequent cause of AE and must be suspected systematically in all patients with AE. The risk of AE is increased in: black people, transplanted patients, those who take gliptins or immunosuppressants (mTOR i). Angiotensin converting enzyme inhibitors induced angioedema (ACE-I AE) can occur a few days to several years after the beginning of this treatment and persist a few months after stopping it. ACE-I AE affect mainly the face and particularly the tongue but also the abdomen. ACE-I AE can be life threatening, due to the involvement of the tongue and the larynx. ACE-I AE must be treated as in the hereditary form when life threatening signs are present, with icatibant or C1 inhibitor concentrate. AE can occur in 10% of angiotensin receptor blockers (ARBs) treated patients who had developed ACE-I AE.

Œdème insulinique au cours d’une glycogénose hépatique

INTRODUCTIONnHepatic glycogenosis is a rare syndrome, which includes poorly controlled diabetes mellitus, hepatomegaly, delayed puberty, and growth delay. Insulin edema is sometimes associated.nnnCASE REPORTnAn 18-year-old woman presented with diffuse edema, hepatomegaly, amenorrhea, uncontrolled diabetes, and elevated transaminases and cholestasis. Hepatic ultrasonography and abdominal computed tomographic scan confirmed the hepatomegaly. The liver biopsy showed a massive glycogenosis and the diagnosis of hepatic glycogenosis was confirmed. Too large doses of insulin were responsible of diffuse edema. Diabetes equilibration and diminution of insulin intakes allow correction of this disorder.nnnCONCLUSIONnExcess of insulin can lead to excessive hepatic glycogen storage by activation of glycogenosis enzymes. Biological manifestations consist on elevated liver enzymes and hyperlactatemia. There is a link between administration of high dose of insulin and edema. Hepatic glycogenosis should be suspected when diabetes is uncontrolled and be considered as a differential diagnosis of steatosis. It may be associated and revealed by insulin edema directly related to excessive insulin intakes.

Aseptic muscular abscesses associated with myelodysplastic syndrome

Abstract