Autoimmune Polyendocrinopathies (Schmidt Syndrome)

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77 relevant articles (4 outcomes, 3 trials/studies) found for this Disease

Description: Autoimmune diseases affecting multiple endocrine organs. Type I is characterized by childhood onset and chronic mucocutaneous candidiasis (CANDIDIASIS, CHRONIC MUCOCUTANEOUS), while type II exhibits any combination of adrenal insufficiency (ADDISON'S DISEASE), lymphocytic thyroiditis (THYROIDITIS, AUTOIMMUNE;), HYPOPARATHYROIDISM; and gonadal failure. In both types organ-specific ANTIBODIES against a variety of ENDOCRINE GLANDS have been detected. The type II syndrome differs from type I in that it is associated with HLA-A1 and B8 haplotypes, onset is usually in adulthood, and candidiasis is not present.

Also Known As:

Schmidt Syndrome; Schmidt's Syndrome; Autoimmune Polyendocrinopathy; Polyendocrinopathies, Autoimmune; Autoimmune Polyglandular Syndrome Type I; Autoimmune Polyglandular Syndrome Type II; Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy; Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal-Dystrophies; Polyendocrinopathy, Autoimmune; Polyendocrinopathy-Candidiasis-Ectodermal-Dystrophies, Autoimmune; Polyendocrinopathy-Candidiasis-Ectodermal-Dystrophy, Autoimmune; Schmidts Syndrome; Syndrome, Schmidt's; Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal-Dystrophy; Autoimmune Syndrome Type I, Polyglandular; Autoimmune Syndrome Type II, Polyglandular; Polyglandular Type I Autoimmune Syndrome; Polyglandular Type II Autoimmune Syndrome

Relationship Network

Disease Context: Research Results

Endocrine System Diseases: 398

Autoimmune Polyendocrinopathies: 77

Immune System Diseases: 1319

Autoimmune Diseases: 12462

Autoimmune Polyendocrinopathies: 77

Related Diseases

1.	Autoimmune Diseases (Autoimmune Disease)
2.	Hypothyroidism
3.	Thyroiditis
4.	Syndrome
5.	Addison Disease (Addison's Disease)

Experts

1.	Perheentupa, Jaakko: 4 articles (01/2007 - 06/2002)
2.	Perheentupa, J: 3 articles (09/2001 - 03/2000)
3.	Peterson, Pärt: 2 articles (01/2007 - 07/2006)
4.	Eskelin, Petra: 2 articles (07/2006 - 06/2002)
5.	Partanen, J: 2 articles (12/2000 - 03/2000)
6.	Björses, P: 2 articles (12/2000 - 03/2000)
7.	Tuomi, T: 2 articles (12/2000 - 03/2000)
8.	Skarpa, Vasiliki: 1 article (07/2007)
9.	Kousta, Eleni: 1 article (07/2007)
10.	Papathanasiou, Asteroula: 1 article (07/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Autoimmune Polyendocrinopathies:

1.	AntibodiesIBA 05/11/1991 - "Organ-specific cardiac antibodies were detected in significantly more of the patients with autoimmune polyendocrinopathy (28 [17%]) than of those with autoimmunity confined to one gland (1 [1%]) or of normal subjects (7 [3.5%]; p = 0.0001). " 05/11/1991 - "Organ-specific cardiac antibodies: serological markers for systemic hypertension in autoimmune polyendocrinopathy." 01/01/2000 - "The significance of the antibodies observed outside the context of paraneoplastic syndromes is not well understood, but the anti-GAD antibodies associated with progressive cerebellar disorders and autoimmune polyendocrinopathies could be an expression of the autoimmune nature of certain neurological degenerative processes affecting the central nervous system." 05/11/1991 - "Patients with autoimmune polyendocrinopathy can have organ-specific cardiac antibodies, which may represent novel serological markers for an autoimmune form of systemic hypertension in the absence of overt cardiac disease." 05/01/1997 - "With this assay, 21-OH Abs were detected in 43 of 60 (72%) sera from patients with isolated Addison's disease, 11 of 12 (92%) autoimmune polyglandular syndrome type I sera, 27 of 27 (100%) autoimmune polyglandular syndrome type II sera, and 24 of 30 (80%) sera from patients who were positive for adrenal cortex antibodies by immunofluorescence but had no overt Addison's disease. " Order ALL the reference details at left...
2.	Thyroxine (Levothyroxine)FDA LinkGeneric 06/01/2000 - "[Manifestation of adrenal insufficiency after administration of levothyroxine in a patient with polyglangular autoimmune syndrome type II (Schmidt-syndrome)]" 10/01/2001 - "This confirmed the diagnosis of Schmidt syndrome as part of polyglandular autoimmune disease (PGAS) type II. After another 18 months of treatment with hormone substitution of L-thyroxine and hydrocortisone, the hyperpigmentation resolved and the hyperpigmented nevi decreased in number." 10/01/1987 - "Anti-thyroid antibodies were present in high titer, supporting the diagnosis of Hashimoto's thyroiditis, and hence of autoimmune polyglandular syndrome type II. The patient was not submitted to thyroxine therapy, and during cortisol replacement thyroid function improved, as judged by normalization of basal and stimulated thyrotropin and elevation of thyroid hormone levels. " Order ALL the reference details at left...
3.	Ketoconazole (Nizoral)FDA LinkGeneric 01/01/1986 - "Ketoconazole is effective against the chronic mucocutaneous candidosis of autoimmune polyendocrinopathy-candidosis-ectodermal dystrophy (APECED)." 01/01/1986 - "Ketoconazole was administered as a single daily oral dose of 200 mg to 12 patients with chronic mucocutaneous candidosis (CMC) of autoimmune polyendocrinopathy-candidosis-ectodermal dystrophy (APECED). " 07/01/1982 - "Ketoconazole in the treatment of chronic mucocutaneous candidiasis secondary to autoimmune polyendocrinopathy-candidiasis syndrome." Order ALL the reference details at left...
4.	Hydrocortisone (Cortisol)FDA LinkGeneric 10/01/2001 - "This confirmed the diagnosis of Schmidt syndrome as part of polyglandular autoimmune disease (PGAS) type II. After another 18 months of treatment with hormone substitution of L-thyroxine and hydrocortisone, the hyperpigmentation resolved and the hyperpigmented nevi decreased in number." 10/01/1987 - "Anti-thyroid antibodies were present in high titer, supporting the diagnosis of Hashimoto's thyroiditis, and hence of autoimmune polyglandular syndrome type II. The patient was not submitted to thyroxine therapy, and during cortisol replacement thyroid function improved, as judged by normalization of basal and stimulated thyrotropin and elevation of thyroid hormone levels. " Order ALL the reference details at left...
5.	HormonesIBA 10/01/2001 - "This confirmed the diagnosis of Schmidt syndrome as part of polyglandular autoimmune disease (PGAS) type II. After another 18 months of treatment with hormone substitution of L-thyroxine and hydrocortisone, the hyperpigmentation resolved and the hyperpigmented nevi decreased in number." 10/01/1987 - "Anti-thyroid antibodies were present in high titer, supporting the diagnosis of Hashimoto's thyroiditis, and hence of autoimmune polyglandular syndrome type II. The patient was not submitted to thyroxine therapy, and during cortisol replacement thyroid function improved, as judged by normalization of basal and stimulated thyrotropin and elevation of thyroid hormone levels. " Order ALL the reference details at left...
6.	Thyrotropin (Thyroid-Stimulating Hormone)FDA Link 10/01/1987 - "Anti-thyroid antibodies were present in high titer, supporting the diagnosis of Hashimoto's thyroiditis, and hence of autoimmune polyglandular syndrome type II. The patient was not submitted to thyroxine therapy, and during cortisol replacement thyroid function improved, as judged by normalization of basal and stimulated thyrotropin and elevation of thyroid hormone levels. " Order ALL the reference details at left...
7.	CortisoneIBA 01/01/1980 - "Cortisone-induced remission of hypothyroidism in Schmidt's syndrome." Order ALL the reference details at left...
8.	rituximab (Mabthera)FDA Link 09/02/2008 - "A therapeutic reagent directed against B cells, Rituximab, induced remission of the autoimmune disease in Aire-deficient mice, raising the hope of applying it to human patients with autoimmune-polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED)." Order ALL the reference details at left...
9.	AutoantibodiesIBA 10/01/2000 - "OBJECTIVE: The aim of the study was to assess the complex of autoantibodies which can be detected in patients with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), a rare autosomal recessive disease in which the extent of autoimmunity is still unknown. " 01/01/2007 - "Pituitary autoantibodies in autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED)." 10/01/2000 - "Organ-specific and non-organ-specific autoantibodies in children and young adults with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED)." 03/01/2000 - "Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a rare monogenic autoimmune disease with endocrine components including type 1 diabetes, adrenal failure, and thyroid dysfunction, with major autoantibodies directed against adrenal, pancreas, and thyroid tissue. " 07/01/1996 - "There have been only four previous reports of Schmidt's syndrome with sarcoidosis, and a possible fifth with negative autoantibodies. " Order ALL the reference details at left...
10.	AutoantigensIBA 09/01/2001 - "Hepatic autoantigens in patients with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy." 02/01/1997 - "Immunoprecipitation of steroidogenic enzyme autoantigens with autoimmune polyglandular syndrome type I (APS I) sera; further evidence for independent humoral immunity to P450c17 and P450c21." 05/01/2008 - "On the other hand and very interestingly, there are PIDs systematically associated with several autoimmune manifestations in which SLE has not been described, such as autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED), immunodysregulation polyendocrinopathy enteropathy X-linked (IPEX), and autoimmune lymphoproliferative syndrome (ALPS), suggesting that mechanisms considered as critical players for induction and maintenance of tolerance to autoantigens, such as (1) AIRE-mediated thymic negative selection of lymphocytes, (2) Foxp3+ regulatory T cell-mediated peripheral tolerance, and (3) deletion of auto-reactive lymphocytes by Fas-mediated apoptosis, could not be relevant in SLE physiopathology. " 10/01/1994 - "Earlier studies have shown that 17 alpha-hydroxylase (P450c17), steroid 21-hydroxylase (P450c21) and side-chain cleavage enzyme (P450scc) are the main autoantigens recognized by sera from patients with Addison's disease associated with autoimmune polyglandular syndrome type I (APS I). " 06/01/2000 - "Here we studied the presence of steroid cell antibodies (SCA), autoantibodies to 3betaHSD and to two other known autoantigens in ovarian failure, steroidogenic enzymes 17alpha-hydroxylase (P450c17), and side-chain cleavage enzyme (P450scc) in POF patients and patient groups with autoimmune polyendocrinopathy syndromes type 1 and 2 (APS1 and -2), isolated Addison's disease, type 1 diabetes, and healthy controls. " Order ALL the reference details at left...

Therapies and Procedures

1.	Corneal Transplantation (Keratoplasty) 04/01/2001 - "Corneal pathology and outcome of keratoplasty in autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED)." Order ALL the reference details at left...
2.	Plasmapheresis 01/01/1997 - "[A case of the successful use of plasmapheresis in the combined treatment of a female patient with autoimmune agranulocytosis against a background of Schmidt's syndrome]" Order ALL the reference details at left...
3.	Laminectomy 11/01/2004 - "Schmidt's syndrome presenting during laminectomy." Order ALL the reference details at left...
4.	Electrocoagulation (Electrocautery) 11/07/1997 - "DIAGNOSIS, TREATMENT AND COURSE: The constellation of test results suggested autoimmune polyglandular syndrome type I. The condylomata were treated by electrocautery and the intestinal candidiasis with amphotericin B suspension. " Order ALL the reference details at left...
5.	Denervation 06/01/1995 - "We report on a patient with an interstitial myositis in type II polyendocrine autoimmune disease (Schmidt's syndrome) showing an immunohistochemical demonstrated perifascicular expression of Leu 19 indicating perifascicular denervation, which could not be detected with classical histological methods. " Order ALL the reference details at left...

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