Publications by authors named "Stephanie Amarger"

Article Synopsis
  • MGUS-associated angioedema due to acquired C1 inhibitor deficiency (AAE-C1-INH) hasn't been specifically characterized before, prompting this study to explore its biological and clinical features over 30 years in France.
  • In a study of 41 patients, most had anti-C1INH antibodies, and treatments included acute management and long-term prophylaxis, with a significant number developing malignant blood disorders like lymphoma or myeloma.
  • The study found a link between the remission of angioedema and the underlying hematological malignancy, emphasizing the need for regular hematological evaluations in patients with MGUS-AE-C1-INH.
View Article and Find Full Text PDF
Article Synopsis
  • Angioedema due to acquired C1-inhibitor deficiency (AAE-C1-INH) can result from excess consumption of C1-INH or the presence of anti-C1-INH antibodies, commonly linked to lymphoid cancers or monoclonal gammopathies.
  • A study in France assessed the effectiveness of rituximab for treating 55 patients with AAE-C1-INH, revealing that 34 patients achieved remission after an average of nearly 4 years of follow-up.
  • The results indicated that patients without anti-C1-INH antibodies had a better chance of remission; those with lymphoma and those treated with rituximab and chemotherapy experienced lower rates of relapse, suggesting that rituximab
View Article and Find Full Text PDF

Angio-oedema is a transitory, localized, noninflammatory oedema of subcutaneous tissue or mucous. When the oedema affects the mouth, lips, tongue or larynx, it can result in fatal asphyxiation in the absence of specific treatment. Oedema secondary to plasma extravasation is usually mediated by either histamine or bradykinin.

View Article and Find Full Text PDF

Background: Currently used antimalarial drugs (AM) are hydroxychloroquine and chloroquine, which are prescribed for many autoimmune disorders. The value of skin tests on cutaneous adverse drug reactions (CADR) with AM remains unknown.

Objective: The main objective of this retrospective study is to know whether skin tests for AM are useful and how to manage the recovery of AM therapy in these patients.

View Article and Find Full Text PDF

Objective: Bradykinin-mediated angioedema is characterized by transient attacks of localized edema of subcutaneous or submucosal tissues and can be life-threatening when involving the upper airways. The aim of this study was to determine the features of acute attacks that might be associated with admission to an ICU.

Patients And Methods: We carried out a retrospective, multicenter, observational study in consecutive patients attending one of six reference centers in France for acute bradykinin-mediated angioedema attacks.

View Article and Find Full Text PDF
Article Synopsis
  • Acquired C1-inhibitor deficiency can be caused by types I and II, with type II associated with anti-C1-inhibitor autoantibodies.
  • Efficacy of treatments like danazol and corticosteroids varies, but rituximab shows some promise in type II angioedema.
  • In a study of 7 patients treated with rituximab, type II showed mixed results, with only a subset achieving significant improvement and indicating that multiple treatments may be necessary.
View Article and Find Full Text PDF

Fixed drug eruption (FDE) is one of the most typical cutaneous drug adverse reactions. This localized drug-induced reaction is characterized by its relapse at the same sites. Few large series of FDE are reported.

View Article and Find Full Text PDF

Rituximab has been documented to be an effective treatment for autoimmune diseases with contribution of B cells. We report a case of antisynthetase syndrome with a history of EBV-induced lymphoma which developed a pemphigus vulgaris. Rituximab was effective both on polymyositis and on pemphigus.

View Article and Find Full Text PDF