CASE REPORT
A rare case of autoimmune dysglycemia syndrome associated with systemic lupus erythematosus and dermatomyositis
 
More details
Hide details
1
Division of Endocrinology, Hospital Nacional Edgardo Rebagliati Martins, Universidad Nacional Mayor de San Marcos, Lima, PERU
2
Division of Endocrinology, Norbert Wiener University, Voto Bernales Hospital, Lima, PERU
3
Division of Medicine, Hospital de Apoyo Chepén, Chepén, PERU
4
Department of Medicine, Universidad Privada Antenor Orrego, Trujillo, PERU
5
Universidad Nacional Mayor de San Marcos, Lima, PERU
Online publication date: 2023-05-21
Publication date: 2023-09-01
 
Electron J Gen Med 2023;20(5):em512
 
KEYWORDS
ABSTRACT
Autoimmune dysglycemia syndrome (ADS) is a rare condition that presents as episodes of hypoglycemia as well as hyperglycemia and is classified as insulin autoimmune syndrome (IAS) and type B insulin resistance (TBIR). Autoimmunity plays a key role in the pathogenesis of this disorder, as evidenced by the presence of autoantibodies against endogenous insulin or the insulin receptor, and by its association with rheumatologic disorders. Treatment usually includes glycemic control and immunomodulatory agents. We report a case of a 31-year-old woman who was admitted for severe hypoglycemia. Further workup revealed underlying systemic lupus erythematosus (SLE) with renal involvement. During hospitalization, she continued to experience episodes of fasting hypoglycemia, as well as episodes of postprandial hyperglycemia. Hypoglycemia associated with a high serum insulin concentration and positive anti-insulin antibodies were consistent with IAS. Likewise, hyperglycemia and hypoglycemia in association with weight loss, acanthosis nigricans, polycystic ovarian syndrome, and normotriglyceridemia strongly suggested TBIR, although testing for antibodies against the insulin receptor was not available in Peru. Immunosuppressive therapy and metformin were indicated, resulting in remission of SLE and ADS. Years later, the patient exhibited features of dermatomyositis, such as Raynaud’s phenomenon, muscular weakness, heliotrope exanthema, and elevated muscle enzymes. Once again, the patient received immunosuppressive therapy. ADS is an infrequent cause of hypoglycemia, and the coexistence of its two pathophysiological mechanisms in a patient with SLE and subsequent development of dermatomyositis is even more rare. Our case is the first one reported describing this association.
 
REFERENCES (19)
1.
Yu S, Yang G, Dou J, et al. Comparison of two autoimmune dysglycemia syndromes: Insulin autoimmune syndrome (IAS) and type B insulin resistance syndrome (B-IRS). Horm Metab Res. 2019;51(11):723-8. https://doi.org/10.1055/a-1005... PMid:31683342.
 
2.
Yukina MY, Davtyan DA, Troshina EA, Nuralieva NF. Autoimmune hypoglycemia. Obes Metabol. 201823;15(3):9-13. https://doi.org/10.14341/omet9....
 
3.
Lupsa BC, Chong AY, Cochran EK, Soos MA, Semple RK, Gorden P. Autoimmune forms of hypoglycemia. Medicine (Baltimore). 2009;88(3):141-53. https://doi.org/10.1097/MD.0b0... PMid:19440117.
 
4.
Łebkowska A, Krentowska A, Adamska A, et al. Type B insulin resistance syndrome associated with connective tissue disease and psoriasis. Endocrinol Diabetes Metab Case Rep. 2020;2020:20-0027. https://doi.org/10.1530/EDM-20... PMid:32755965 PMCid:PMC7424346.
 
5.
Maazoun F, Frikha F, Snoussi M, Kaddour N, Masmoudi H, Bahloul Z. Systemic lupus erythematosusmyositis overlap syndrome: Report of 6 cases. Clin Pract. 2011;1(4):e89. https://doi.org/10.4081/cp.201... PMid:24765389 PMCid:PMC3981442.
 
6.
Greidinger E. Overlap syndromes. In: Tsakos GC, editor. Systemic lupus erythematosus: Basic, applied and clinical aspects. Boston, MI: Academic Press; 2016. p. 425-30. https://doi.org/10.1016/B978-0....
 
7.
Lin M, Chen Y, Ning J. Insulin autoimmune syndrome: A systematic review. Int J Endocrinol. 2023;2023:e1225676. https://doi.org/10.1155/2023/1... PMid:36844104 PMCid:PMC9946739.
 
8.
Patel M, Shah R, Ramteke-Jadhav S, et al. Management of insulin autoimmune hypoglycaemia: Single-centre experience from western India with systematic review of world literature. Clin Endocrinol. 2020;92(5):409-20. https://doi.org/10.1111/cen.14... PMid:32064669.
 
9.
Martins LM, Fernandes VO, Carvalho MMDD, Gadelha DD, Queiroz PCD, Montenegro RM. Type B insulin resistance syndrome: A systematic review. Arch Endocrinol Metab. 2020;64(4):337-48. https://doi.org/10.20945/2359-... PMid:32813762.
 
10.
Zhang S, Wang G, Wang J. Type B insulin resistance syndrome induced by systemic lupus erythematosus and successfully treated with intravenous immunoglobulin: Case report and systematic review. Clin Rheumatol. 2013;32(2):181-8. https://doi.org/10.1007/s10067... PMid:23053690.
 
11.
Qing Y, Zhou JG, Yuan G. Systemic lupus erythematosus presenting as hypoglycaemia with insulin receptor antibodies and insulin autoantibodies. Lupus. 2009; 18(5):457-9. https://doi.org/10.1177/096120... PMid:19318401.
 
12.
Hughes LE, Fenn JS, Ford C, Gama R. Autoimmune hypoglycaemia: A narrative review from the laboratory perspective. J Lab Precis Med. 2021;6:1-8. https://doi.org/10.21037/jlpm-....
 
13.
Willard DL, Stevenson M, Steenkamp D. Type B insulin resistance syndrome. Curr Opin Endocrinol Diabetes Obes. 2016;23(4):318-23. https://doi.org/10.1097/MED.00... PMid:27254267.
 
14.
Ernste FC, Reed AM. Idiopathic inflammatory myopathies: Current trends in pathogenesis, clinical features, and up-to-date treatment recommendations. Mayo Clin Proc. 2013;88(1):83-105. https://doi.org/10.1016/j.mayo... PMid:23274022.
 
15.
Song GG, Lee YH. Circulating prolactin level in systemic lupus erythematosus and its correlation with disease activity: A meta-analysis. Lupus. 2017;26(12):1260-8. https://doi.org/10.1177/096120... PMid:28420051.
 
16.
Censi S, Mian C, Betterle C. Insulin autoimmune syndrome: from diagnosis to clinical management. Ann Transl Med. 2018;6(17):335. https://doi.org/10.21037/atm.2... PMid:30306074 PMCid:PMC6174196.
 
17.
Willard D, Upadhyay J, Kim C, Steenkamp D. Diabetic ketoacidosis without diabetes. J Clin Endocrinol Metab. 2016;101(11):3870-3. https://doi.org/10.1210/jc.201... PMid:27636019.
 
18.
Ismail AAA. The insulin autoimmune syndrome (IAS) as a cause of hypoglycaemia: An update on the pathophysiology, biochemical investigations and diagnosis. Clin Chem Lab Med. 2016;54(11):1715-24. https://doi.org/10.1515/cclm-2... PMid:27071154.
 
19.
Dalakas MC. Inflammatory muscle diseases. N Engl J Med. 2015;372(18):1734-47. https://doi.org/10.1056/NEJMra... PMid:25923553.
 
eISSN:2516-3507