Acquired C1 inhibitor deficiency associated with systemic lupus erythematosus affecting the central nervous system.

Nakamura, Sachiko; Yoshinari, Miyo; Saku, Yoshisuke; Hirakawa, K; Miishima, C; Murai, Koichi; Tokiyama, K; Fujishima, Masumi

doi:10.1136/ard.50.10.713

Cited by 32 publications

(16 citation statements)

References 25 publications

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“…Type II AAE is a rare disorder and the worldwide incidence of type II AAE reported to date is less than 100 cases (12)(13)(14)(15)(16)(17)(23)(24)(25)(26)(27)(28). To our knowledge, this is the first case of type II AAEin Japan, although two cases of AAEhave been reported in the literature (29,30).…”

Section: Discussionmentioning

confidence: 76%

Autoimmune Acquired Form of Angioedema that Responded to Danazol Therapy.

Higa

Hirata

et al. 2002

Intern. Med.

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A 51-year-old man with recurrent episodes of angioedema was diagnosed as having autoimmuneacquired angioedema, based on adult onset, lack of apparent family history, decreased activity of Cl esterase inhibitor (Cl INH) and CH50, decreased levels of serum C4 and Clq and the presence of autoantibodies to Cl INH. The danazol treatment relieved the symptoms of angioedemaand increased the Cl INHactivity and concentration with the normalization of CH50, Clq and C4 levels. To our knowledge, this is the first case of autoimmune acquired angioedema in Japan. (Internal Medicine 41 : 398-402, 2002)

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Section: Discussionmentioning

confidence: 76%

Autoimmune Acquired Form of Angioedema that Responded to Danazol Therapy.

Higa

Hirata

et al. 2002

Intern. Med.

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“…Scattered reports describe acquired C1-INH deficiency associated with nonhematologic neoplasm, infections, or autoimmune diseases, whereas 14% of patients with acquired C1-INH deficiency have no other disease. 105,[118][119][120][121][122][123][124][125][126] In 1986, autoantibodies inactivating C1-INH were first detected in patients with acquired C1-INH deficiency. 127 Initially, autoantibodies inactivating C1-INH were identified in otherwise healthy patients.…”

Section: Angioedema Caused By Acquired C1-inh Deficiencymentioning

confidence: 99%

Hereditary and acquired angioedema: Problems and progress: Proceedings of the third C1 esterase inhibitor deficiency workshop and beyond

Agostoni¹,

Aygören‐Pürsün²,

Binkley³

et al. 2004

Journal of Allergy and Clinical Immunology

599

658

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Hereditary angioedema (HAE), a rare but life-threatening condition, manifests as acute attacks of facial, laryngeal, genital, or peripheral swelling or abdominal pain secondary to intra-abdominal edema. Resulting from mutations affecting C1 esterase inhibitor (C1-INH), inhibitor of the first complement system component, attacks are not histamine-mediated and do not respond to antihistamines or corticosteroids. Low awareness and resemblance to other disorders often delay diagnosis; despite availability of C1-INH replacement in some countries, no approved, safe acute attack therapy exists in the United States. The biennial C1 Esterase Inhibitor Deficiency Workshops resulted from a European initiative for better knowledge and treatment of HAE and related diseases. This supplement contains work presented at the third workshop and expanded content toward a definitive picture of angioedema in the absence of allergy. Most notably, it includes cumulative genetic investigations; multinational laboratory diagnosis recommendations; current pathogenesis hypotheses; suggested prophylaxis and acute attack treatment, including home treatment; future treatment options; and analysis of patient subpopulations, including pediatric patients and patients whose angioedema worsened during pregnancy or hormone administration. Causes and management of acquired angioedema and a new type of angioedema with normal C1-INH are also discussed. Collaborative patient and physician efforts, crucial in rare diseases, are emphasized. This supplement seeks to raise awareness and aid diagnosis of HAE, optimize treatment for all patients, and provide a platform for further research in this rare, partially understood disorder.

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“…To the authors' knowledge, few cases of SLE associated with acquired C1-INH deficiency and also associated with the presence of C1 inhibitor autoantibodies have been reported [14][15][16][17][18][19][20][21]. Some reported cases have considered patients with SLE and laboratoristic evidence of acquired C1-INH deficiency but without any signs of angioedema [15,16].…”

Section: Discussionmentioning

confidence: 99%

Acquired C1‐inhibitor deficiency in a patient with systemic lupus erythematosus: a case report and review of the literature

Nettis

Colanardi

Loria

et al. 2005

Eur J Clin Investigation

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The C1-inhibitor (C1-INH) is an important member of the serpin family which inhibits the first component of the human complement system and controls contact activation of the coagulation and kinin system. An acquired form of C1-INH deficiency was recognized and classified as type I, which is characterized by accelerated catabolism of C1-INH, whereas type II is defined by the presence of an autoantibody directed against the C1 inhibitor molecule. This study reports the case of a 32-year-old woman with systemic lupus erythematosus (SLE) who experienced recurrent angioedema because of an acquired C1-INH deficiency. The relevant literature is reviewed.

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Acquired C1 inhibitor deficiency associated with systemic lupus erythematosus affecting the central nervous system.

Cited by 32 publications

References 25 publications

Autoimmune Acquired Form of Angioedema that Responded to Danazol Therapy.

Autoimmune Acquired Form of Angioedema that Responded to Danazol Therapy.

Hereditary and acquired angioedema: Problems and progress: Proceedings of the third C1 esterase inhibitor deficiency workshop and beyond

Acquired C1‐inhibitor deficiency in a patient with systemic lupus erythematosus: a case report and review of the literature

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