1/66. adrenal insufficiency as the first clinical manifestation of the primary antiphospholipid antibody syndrome.We describe a 60-year-old man who developed clinical symptoms and signs of Addison's disease, which was subsequently confirmed biochemically; no cause was apparent. Several months later the patient represented with a fit, followed by a large and extensive venous thrombosis in the right iliac vein and in the veins of the right leg. He had strongly positive antibodies to cardiolipin, strongly suggesting a diagnosis of primary antiphospholipid syndrome. While Addison's disease is a well-recognized, albeit rare, manifestation of the antiphospholipid syndrome, the Addison's disease preceded other clinical evidence of the syndrome by several months, in our patient, at variance with previous cases described in the literature. The antiphospholipid syndrome should be considered as a possible pathogenetic process in patients presenting with Addison's disease where the aetiology is not obvious.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
2/66. Brittle Addison's disease: a new variation on a familiar theme.Unstable and unpredictable disease control in diabetes or asthma, with frequent hospitalisations, is frequently referred to as 'brittle'. We describe two cases of Addison's disease with recurrent hospitalisations in hypo-adrenal crises. Both patients had significant psychosocial disruption, and failure to take hydrocortisone replacement therapy was admitted in one and biochemically proven in the other. We propose that 'brittle' Addison's disease in these cases was due to poor treatment compliance related to psychosocial factors. These features have particular similarities with the syndrome of brittle diabetes.- - - - - - - - - - ranking = 0.125keywords = syndrome (Clic here for more details about this article) |
3/66. Systemic lupus erythematosus with polyglandular autoimmune syndrome type II: report of an unusual case.A patient with systemic lupus erythematosus (SLE) is described who had associated autoimmune thyroiditis with subclinical hypothyroidism and idiopathic Addison's disease. The presence of autoimmune thyroid disease and Addison's disease could classify her as having polyglandular autoimmune syndrome (PGAS) type II, which is the commonest of the three types of this syndrome. However, the additional presence of SLE in PGAS type II has not been described earlier. This patient appears to be the first such case.- - - - - - - - - - ranking = 0.75keywords = syndrome (Clic here for more details about this article) |
4/66. Schmidt's syndrome in a Saudi family.We present 3 patients from a Saudi family who are presented with polyglandular autoimmune syndrome type 2. They have Addison's disease with either autoimmune thyroid disease or insulin dependent diabetic mellitus. Although this syndrome is rare, the incidence among saudi arabia or the Arab population is not known.- - - - - - - - - - ranking = 0.75keywords = syndrome (Clic here for more details about this article) |
5/66. Myelodysplastic syndrome accompanied by Addison's disease and multiple autoimmune phenomena: steroid therapy resolved cytopenias and all immune disorders.We report here a patient with myelodysplastic syndromes (MDS), which was complicated with several autoimmune disorders and asymptomatic immunologic abnormalities. An 82-year-old woman with refractory anemia (RA) rapidly developed thrombocytopenia with the appearance of symptoms such as purpura, fatigue, anorexia, and weight loss. Furthermore, clinical examinations revealed that she also had Addison's disease, rheumatoid arthritis, and autoimmune hematological diseases such as thrombocytopenia and hemolytic anemia. However, the cytopenia and all autoimmune disorders were remarkably improved after she received steroid therapy.- - - - - - - - - - ranking = 0.625keywords = syndrome (Clic here for more details about this article) |
6/66. Distinct clinical phenotype and immunoreactivity in Japanese siblings with autoimmune polyglandular syndrome type 1 (APS-1) associated with compound heterozygous novel AIRE gene mutations.We herein report on two Japanese siblings with autoimmune polyglandular syndrome type 1 (APS-1). The brother, who expressed a characteristic phenotype of APS-1, had developed severe mucocutaneous candidiasis in early infancy and thereafter developed hypoparathyroidism and Addison's disease, along with a severe deterioration of his immunologic function. In contrast, the 44-year-old sister, who showed a noncharacteristic phenotype of APS-1, developed insulin-dependent diabetes with high anti-glutamic acid decarboxylase antibody, mild nail candidiasis, and autoimmune hepatitis with intact immunoreactivity. She had three susceptible human leukocyte antigen (HLA) loci for type 1 autoimmune diabetes. The expression of T cell receptor (TCR)V beta 5.1 increased in both patients, while the brother showed a widely suppressed expression of many TCRV beta families. Both individuals possessed compound heterozygous novel autoimmune regulator (AIRE) gene mutations (L29P and IVS9-1G > C). The same AIRE gene mutations can thus be associated with characteristic and noncharacteristic phenotypes of APS-1, and HLA may possibly influence the phenotype of APS-1.- - - - - - - - - - ranking = 0.625keywords = syndrome (Clic here for more details about this article) |
7/66. hyperpigmentation mimicking Laugier syndrome, levodopa therapy and Addison's disease.The Laugier-Hunziker syndrome is an acquired, idiopathic, benign mucocutaneous hypermelanosis that usually occurs on the lips and oral mucosa, although it may appear at other sites. nails are frequently involved, mainly forming longitudinal hyperpigmented bands. We report the case of a patient that presented a typical picture of this entity, nearly 1 year after the beginning of treatment with levodopa. Two years after the first lesions occurred, she developed Addison's disease. The patient suffered from a diffuse discrete hyperpigmentation (it was more remarkable on exposed areas) and an intensification of the melanotic macules that were previously noticeable before in oral and genital mucosa, fingers, toes and nails. Hormonal replacement treatment enabled the control of laboratory and general manifestations and to decrease the degree of mucocutaneous hyperpigmentation considerably, despite initial hyperpigmented lesions persisting in described areas.- - - - - - - - - - ranking = 0.625keywords = syndrome (Clic here for more details about this article) |
8/66. Unusual presentation of Addison's disease in Schmidt's syndrome.A patient with Schmidt's syndrome and atypical symptoms of Addison's disease is presented. Autoimmune thyroiditis was diagnosed at the age of 12 years and then at 15 years; a diagnosis of migraine was made following 10 days of headache, visual field defects and dysarthria. One week later the patient was admitted to hospital in critical condition. Addison's disease was diagnosed and replacement therapy was introduced which brought about a rapid clinical improvement. Positive adrenal autoantibodies confirmed the diagnosis. We suggest that patients with organ-specific autoimmune disease undergo annual screening for adrenal activity, and in the event of abnormal results, adrenal autoantibody evaluation should be carried out, to avoid a life-threatening crisis caused by Addison's disease, which is often difficult to recognize due to atypical clinical presentation, as in the patient reported here.- - - - - - - - - - ranking = 0.625keywords = syndrome (Clic here for more details about this article) |
9/66. Unusual skin pigmentation in a patient with human immunodeficiency virus (hiv) infection.Diffuse addisonian hyperpigmentation in a male patient with acquired immunodeficiency syndrome related complex (ARC) is described. The etiology of pigmentation in this patient remains obscure but is most probably related to the H.I.V. infection. Other causes of addisonian hyperpigmentation are considered less likely.- - - - - - - - - - ranking = 0.125keywords = syndrome (Clic here for more details about this article) |
10/66. Addisonian crisis precipitated by thyroxine therapy: a complication of type 2 autoimmune polyglandular syndrome.hypothyroidism is a common condition. Rarely, it may occur in combination with autoimmune failure of other endocrine glands (autoimmune polyendocrinopathy syndrome type 2, previously known as Schmidt's syndrome). In such cases, restoring normal thyroid function may precipitate adrenal failure. Clinicians should have a high index of suspicion for this condition in patients with Addison's disease, those with a family history of autoimmune endocrine gland failure, patients with one autoimmune endocrine disease who develop nonspecific or serious illness, and patients with type 1 diabetes mellitus whose insulin requirements drop without obvious explanation.- - - - - - - - - - ranking = 0.75keywords = syndrome (Clic here for more details about this article) |
| | Next -> |