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      Ketosis-prone diabetes and SLE co-presenting in an African lady with previous gestational diabetes

      research-article
      , ,
      Endocrinology, Diabetes & Metabolism Case Reports
      Bioscientifica Ltd
      Adult, Female, Black – African, United Kingdom, Pancreas, Diabetes, Insulin, Systemic lupus erythematosus, Diabetes mellitus type 2, Diabetic ketoacidosis, Gestational diabetes, Pleurisy, Chest pain (pleuritic), Dyspnoea, Pyrexia, Sweating, Tachycardia, Crackles, Heart murmur, Hypoxia, Metabolic acidosis, Hyperglycaemia, Diabetic ketoacidosis, Cardiomegaly, Haematuria, Proteinuria, Ketonuria, Glucosuria, Pericardial effusion, Lymphadenitis, Acid-base balance, Bicarbonate, Base excess, Lactate, Glucose (blood), Ketones (plasma), White blood cell count, Blood film, X-ray, Electrocardiogram, C-reactive protein, CTPA scan, Echocardiogram, MRI, antinuclear antibody, Haemoglobin, Methylprednisolone, Prednisolone, Radiology/Rheumatology, Unique/unexpected symptoms or presentations of a disease, October, 2017

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          Abstract

          We describe the case of an African woman who was diagnosed with ketosis-prone diabetes with diabetes-associated autoantibodies, after being admitted for diabetic ketoacidosis (DKA) precipitated by her first presentation of systemic lupus erythematosus (SLE). She had a seven-year history of recurrent gestational diabetes (GDM) not requiring insulin therapy, with return to normoglycaemia after each pregnancy. This might have suggested that she had now developed type 2 diabetes (T2D). However, the diagnosis of SLE prompted testing for an autoimmune aetiology for the diabetes, and she was found to have a very high titre of GAD antibodies. Typical type 1 diabetes (T1D) was thought unlikely due to the long preceding history of GDM. Latent autoimmune diabetes of adults (LADA) was considered, but ruled out as she required insulin therapy from diagnosis. The challenge of identifying the type of diabetes when clinical features overlap the various diabetes categories is discussed. This is the first report of autoimmune ketosis-prone diabetes (KPD) presenting with new onset of SLE.

          Learning points:
          • DKA may be the first presentation of a multi-system condition and a precipitating cause should always be sought, particularly in women with a history of GDM or suspected T2D.

          • All women with GDM should undergo repeat glucose tolerance testing postpartum to exclude frank diabetes, even when post-delivery capillary blood glucose (CBG) tests are normal. They should also be advised to continue CBG monitoring during acute illness in case of new onset diabetes.

          • KPD comprises a spectrum of diabetes syndromes that present with DKA, but subsequently have a variable course depending on the presence or absence of beta cell failure and/or diabetes autoantibodies.

          • KPD should be considered in a patient with presumed T2D presenting with DKA, especially if there is a personal or family history of autoimmune diabetes.

          • LADA should be suspected in adults presumed to have T2D, who do not require insulin therapy for at least six months after diagnosis and have anti-GAD antibodies.

          • Patients with autoimmune diabetes have an increased risk of other autoimmune diseases and screening for thyroid, parietal cell, coeliac and antinuclear antibodies should be considered.

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          Most cited references6

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          The many faces of diabetes: a disease with increasing heterogeneity.

          Diabetes is a much more heterogeneous disease than the present subdivision into types 1 and 2 assumes; type 1 and type 2 diabetes probably represent extremes on a range of diabetic disorders. Both type 1 and type 2 diabetes seem to result from a collision between genes and environment. Although genetic predisposition establishes susceptibility, rapid changes in the environment (ie, lifestyle factors) are the most probable explanation for the increase in incidence of both forms of diabetes. Many patients have genetic predispositions to both forms of diabetes, resulting in hybrid forms of diabetes (eg, latent autoimmune diabetes in adults). Obesity is a strong modifier of diabetes risk, and can account for not only a large proportion of the epidemic of type 2 diabetes in Asia but also the ever-increasing number of adolescents with type 2 diabetes. With improved characterisation of patients with diabetes, the range of diabetic subgroups will become even more diverse in the future. Copyright © 2014 Elsevier Ltd. All rights reserved.
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            Diabetes at the crossroads: relevance of disease classification to pathophysiology and treatment.

            Diabetes is not a single homogeneous disease but composed of many diseases with hyperglycaemia as a common feature. Four factors have, historically, been used to identify this diversity: the age at onset; the severity of the disease, i.e. degree of loss of beta cell function; the degree of insulin resistance and the presence of diabetes-associated autoantibodies. Our broad understanding of the distinction between the two major types, type 1 diabetes mellitus and type 2 diabetes mellitus, are based on these factors, but it has become apparent that they do not precisely capture the different disease forms. Indeed, both major types of diabetes have common features, encapsulated by adult-onset autoimmune diabetes and maturity-onset diabetes of the young. As a result, there has been a repositioning of our understanding of diabetes. In this review, drawing on recent literature, we discuss the evidence that autoimmune type 1 diabetes has a broad clinical phenotype with diverse therapeutic options, while the term non-autoimmune type 2 diabetes obscures the optimal management strategy because it encompasses substantial heterogeneity. Underlying these developments is a general progression towards precision medicine with the need for precise patient characterisation, currently based on clinical phenotypes but in future augmented by laboratory-based tests.
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              Accuracy and predictive value of classification schemes for ketosis-prone diabetes.

              Ketosis-prone diabetes (KPD) is an emerging, heterogeneous syndrome. A sound classification scheme for KPD is essential to guide clinical practice and pathophysiologic studies. Four schemes have been used and are based on immunologic criteria, immunologic criteria and insulin requirement, BMI, and immunologic criteria and beta-cell function (Abeta classification). The aim of the present study is to compare the four schemes for accuracy and predictive value in determining whether KPD patients have absent or preserved beta-cell function, which is a strong determinant of long-term insulin dependence and clinical phenotype. Consecutive patients (n = 294) presenting with diabetic ketoacidosis and followed for 12-60 months were classified according to all four schemes. They were evaluated longitudinally for beta-cell autoimmunity, clinical and biochemical features, beta-cell function, and insulin dependence. beta-Cell function was defined by peak plasma C-peptide response to glucagon >or=1.5 ng/ml. The accuracy of each scheme to predict absent or preserved beta-cell function after 12 months of follow-up was tested using multiple statistical analyses. The "Abeta" classification scheme was the most accurate overall, with a sensitivity and specificity of 99.4 and 95.9%, respectively, positive and negative likelihood ratios of 24.55 and 0.01, respectively, and an area under the receiver operator characteristic curve of 0.972. The Abeta scheme has the highest accuracy and predictive value in classifying KPD patients with regard to clinical outcomes and pathophysiologic subtypes.

                Author and article information

                Journal
                Endocrinol Diabetes Metab Case Rep
                Endocrinol Diabetes Metab Case Rep
                EDM
                Endocrinology, Diabetes & Metabolism Case Reports
                Bioscientifica Ltd (Bristol )
                2052-0573
                06 October 2017
                2017
                : 2017
                : 17-0086
                Affiliations
                [1]Department of Diabetes and Endocrinology , Newham University Hospital, Barts Health NHS Trust, London, UK
                Author notes
                Correspondence should be addressed to S Hussain Email: shaziahussain@ 123456doctors.org.uk
                Article
                EDM170086
                10.1530/EDM-17-0086
                5633049
                87ed1095-f45f-445d-a100-541c6d91d5b4
                © 2017 The authors

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.

                History
                : 16 August 2017
                : 13 September 2017
                Categories
                Unique/Unexpected Symptoms or Presentations of a Disease

                adult,female,black – african,united kingdom,pancreas,diabetes,insulin,systemic lupus erythematosus,diabetes mellitus type 2,diabetic ketoacidosis,gestational diabetes,pleurisy,chest pain (pleuritic),dyspnoea,pyrexia,sweating,tachycardia,crackles,heart murmur,hypoxia,metabolic acidosis,hyperglycaemia,cardiomegaly,haematuria,proteinuria,ketonuria,glucosuria,pericardial effusion,lymphadenitis,acid-base balance,bicarbonate,base excess,lactate,glucose (blood),ketones (plasma),white blood cell count,blood film,x-ray,electrocardiogram,c-reactive protein,ctpa scan,echocardiogram,mri,antinuclear antibody,haemoglobin,methylprednisolone,prednisolone,radiology/rheumatology,unique/unexpected symptoms or presentations of a disease,october,2017

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