Browse

You are looking at 1 - 10 of 10 items

Agnieszka Łebkowska Department of Internal Medicine and Metabolic Diseases, Diabetology and Internal Medicine

Search for other papers by Agnieszka Łebkowska in
Google Scholar
PubMed
Close
,
Anna Krentowska Department of Internal Medicine and Metabolic Diseases, Diabetology and Internal Medicine

Search for other papers by Anna Krentowska in
Google Scholar
PubMed
Close
,
Agnieszka Adamska Department of Endocrinology, Diabetology and Internal Medicine

Search for other papers by Agnieszka Adamska in
Google Scholar
PubMed
Close
,
Danuta Lipińska Department of Endocrinology, Diabetology and Internal Medicine

Search for other papers by Danuta Lipińska in
Google Scholar
PubMed
Close
,
Beata Piasecka Department of Endocrinology, Diabetology and Internal Medicine

Search for other papers by Beata Piasecka in
Google Scholar
PubMed
Close
,
Otylia Kowal-Bielecka Department of Rheumatology and Internal Diseases, Medical University of Bialystok, Bialystok, Poland

Search for other papers by Otylia Kowal-Bielecka in
Google Scholar
PubMed
Close
,
Maria Górska Department of Endocrinology, Diabetology and Internal Medicine

Search for other papers by Maria Górska in
Google Scholar
PubMed
Close
,
Robert K Semple Centre for Cardiovascular Science, Queen’s Medical Research Institute, University of Edinburgh, Edinburgh, UK

Search for other papers by Robert K Semple in
Google Scholar
PubMed
Close
, and
Irina Kowalska Department of Internal Medicine and Metabolic Diseases, Diabetology and Internal Medicine

Search for other papers by Irina Kowalska in
Google Scholar
PubMed
Close

Summary

Type B insulin resistance syndrome (TBIR) is characterised by the rapid onset of severe insulin resistance due to circulating anti-insulin receptor antibodies (AIRAs). Widespread acanthosis nigricans is normally seen, and co-occurrence with other autoimmune diseases is common. We report a 27-year-old Caucasian man with psoriasis and connective tissue disease who presented with unexplained rapid weight loss, severe acanthosis nigricans, and hyperglycaemia punctuated by fasting hypoglycaemia. Severe insulin resistance was confirmed by hyperinsulinaemic euglycaemic clamping, and immunoprecipitation assay demonstrated AIRAs, confirming TBIR. Treatment with corticosteroids, metformin and hydroxychloroquine allowed withdrawal of insulin therapy, with stabilisation of glycaemia and diminished signs of insulin resistance; however, morning fasting hypoglycaemic episodes persisted. Over three years of follow-up, metabolic control remained satisfactory on a regimen of metformin, hydroxychloroquine and methotrexate; however, psoriatic arthritis developed. This case illustrates TBIR as a rare but severe form of acquired insulin resistance and describes an effective multidisciplinary approach to treatment.

Learning points:

  • We describe an unusual case of type B insulin resistance syndrome (TBIR) in association with mixed connective tissue disease and psoriasis.

  • Clinical evidence of severe insulin resistance was corroborated by euglycaemic hyperinsulinaemic clamp, and anti-insulin receptor autoantibodies were confirmed by immunoprecipitation assay.

  • Treatment with metformin, hydroxychloroquine and methotrexate ameliorated extreme insulin resistance.

Open access
Baris Akinci Brehm Center for Diabetes Research and Division of Metabolism, Endocrinology & Diabetes, University of Michigan, Ann Arbor, Michigan, USA
Division of Endocrinology and Metabolism, Dokuz Eylul University, Izmir, Turkey

Search for other papers by Baris Akinci in
Google Scholar
PubMed
Close
,
Rasimcan Meral Brehm Center for Diabetes Research and Division of Metabolism, Endocrinology & Diabetes, University of Michigan, Ann Arbor, Michigan, USA

Search for other papers by Rasimcan Meral in
Google Scholar
PubMed
Close
,
Diana Rus Brehm Center for Diabetes Research and Division of Metabolism, Endocrinology & Diabetes, University of Michigan, Ann Arbor, Michigan, USA

Search for other papers by Diana Rus in
Google Scholar
PubMed
Close
,
Rita Hench Brehm Center for Diabetes Research and Division of Metabolism, Endocrinology & Diabetes, University of Michigan, Ann Arbor, Michigan, USA

Search for other papers by Rita Hench in
Google Scholar
PubMed
Close
,
Adam H Neidert Brehm Center for Diabetes Research and Division of Metabolism, Endocrinology & Diabetes, University of Michigan, Ann Arbor, Michigan, USA

Search for other papers by Adam H Neidert in
Google Scholar
PubMed
Close
,
Frank DiPaola Division of Pediatric Gastroenterology, University of Michigan, Ann Arbor, Michigan, USA

Search for other papers by Frank DiPaola in
Google Scholar
PubMed
Close
,
Maria Westerhoff Department of Pathology, University of Michigan, Ann Arbor, Michigan, USA

Search for other papers by Maria Westerhoff in
Google Scholar
PubMed
Close
,
Simeon I Taylor Division of Endocrinology, Diabetes, and Nutrition, University of Maryland School of Medicine, Baltimore, Maryland, USA

Search for other papers by Simeon I Taylor in
Google Scholar
PubMed
Close
, and
Elif A Oral Brehm Center for Diabetes Research and Division of Metabolism, Endocrinology & Diabetes, University of Michigan, Ann Arbor, Michigan, USA

Search for other papers by Elif A Oral in
Google Scholar
PubMed
Close

Summary

A patient with atypical partial lipodystrophy who had a transient initial response to metreleptin experienced acute worsening of her metabolic state when neutralizing antibodies against metreleptin appeared. Because her metabolic status continued to deteriorate, a therapeutic trial with melanocortin-4 receptor agonist setmelanotide, that is believed to function downstream from leptin receptor in the leptin signaling system, was undertaken in an effort to improve her metabolic status for the first time in a patient with lipodystrophy. To achieve this, a compassionate use (investigational new drug application; IND) was initiated (NCT03262610). Glucose control, body fat by dual-energy X-ray absorptiometry and MRI, and liver fat by proton density fat fraction were monitored. Daily hunger scores were assessed by patient filled questionnaires. Although there was a slight decrease in hunger scales and visceral fat, stimulating melanocortin-4 receptor by setmelanotide did not result in any other metabolic benefit such as improvement of hypertriglyceridemia or diabetes control as desired. Targeting melanocortin-4 receptor to regulate energy metabolism in this setting was not sufficient to obtain a significant metabolic benefit. However, complex features of our case make it difficult to generalize these observations to all cases of lipodystrophy. It is still possible that melanocortin-4 receptor agonistic action may offer some therapeutic benefits in leptin-deficient patients.

Learning points:

  • A patient with atypical lipodystrophy with an initial benefit with metreleptin therapy developed neutralizing antibodies to metreleptin (Nab-leptin), which led to substantial worsening in metabolic control. The neutralizing activity in her serum persisted for longer than 3 years.

  • Whether the worsening in her metabolic state was truly caused by the development of Nab-leptin cannot be fully ascertained, but there was a temporal relationship. The experience noted in our patient at least raises the possibility for concern for substantial metabolic worsening upon emergence and persistence of Nab-leptin. Further studies of cases where Nab-leptin is detected and better assay systems to detect and characterize Nab-leptin are needed.

  • The use of setmelanotide, a selective MC4R agonist targeting specific neurons downstream from the leptin receptor activation, was not effective in restoring metabolic control in this complex patient with presumed diminished leptin action due to Nab-leptin.

  • Although stimulating the MC4R pathway was not sufficient to obtain a significant metabolic benefit in lowering triglycerides and helping with her insulin resistance as was noted with metreleptin earlier, there was a mild reduction in reported food intake and appetite.

  • Complex features of our case make it difficult to generalize our observation to all leptin-deficient patients. It is possible that some leptin-deficient patients (especially those who need primarily control of food intake) may still theoretically benefit from MC4R agonistic action, and further studies in carefully selected patients may help to tease out the differential pathways of metabolic regulation by the complex network of leptin signaling system.

Open access
Aishah Ekhzaimy Department of Medicine and College of Medicine, King Saud University, Riyadh, Saudi Arabia

Search for other papers by Aishah Ekhzaimy in
Google Scholar
PubMed
Close
,
Afshan Masood Obesity Research Center, and College of Medicine, King Saud University, Riyadh, Saudi Arabia

Search for other papers by Afshan Masood in
Google Scholar
PubMed
Close
,
Seham Alzahrani Department of Medicine and College of Medicine, King Saud University, Riyadh, Saudi Arabia

Search for other papers by Seham Alzahrani in
Google Scholar
PubMed
Close
,
Waleed Al-Ghamdi Department of Medicine and College of Medicine, King Saud University, Riyadh, Saudi Arabia

Search for other papers by Waleed Al-Ghamdi in
Google Scholar
PubMed
Close
,
Daad Alotaibi Department of Medicine and College of Medicine, King Saud University, Riyadh, Saudi Arabia

Search for other papers by Daad Alotaibi in
Google Scholar
PubMed
Close
, and
Muhammad Mujammami Department of Medicine and College of Medicine, King Saud University, Riyadh, Saudi Arabia

Search for other papers by Muhammad Mujammami in
Google Scholar
PubMed
Close

Summary

Central diabetes insipidus (CDI) and several endocrine disorders previously classified as idiopathic are now considered to be of an autoimmune etiology. Dermatomyositis (DM), a rare autoimmune condition characterized by inflammatory myopathy and skin rashes, is also known to affect the gastrointestinal, pulmonary, and rarely the cardiac systems and the joints. The association of CDI and DM is extremely rare. After an extensive literature search and to the best of our knowledge this is the first reported case in literature, we report the case of a 36-year-old male with a history of CDI, who presented to the hospital’s endocrine outpatient clinic for evaluation of a 3-week history of progressive facial rash accompanied by weakness and aching of the muscles.

Learning points:

  • Accurate biochemical diagnosis should always be followed by etiological investigation.

  • This clinical entity usually constitutes a therapeutic challenge, often requiring a multidisciplinary approach for optimal outcome.

  • Dermatomyositis is an important differential diagnosis in patients presenting with proximal muscle weakness.

  • Associated autoimmune conditions should be considered while evaluating patients with dermatomyositis.

  • Dermatomyositis can relapse at any stage, even following a very long period of remission.

  • Maintenance immunosuppressive therapy should be carefully considered in these patients.

Open access
Maria Tomkins Department of Endocrinology and Diabetes, Beaumont Hospital Dublin, Dublin, Ireland

Search for other papers by Maria Tomkins in
Google Scholar
PubMed
Close
,
Roxana Maria Tudor Department of Endocrinology and Diabetes, Beaumont Hospital Dublin, Dublin, Ireland

Search for other papers by Roxana Maria Tudor in
Google Scholar
PubMed
Close
,
Diarmuid Smith Department of Endocrinology and Diabetes, Beaumont Hospital Dublin, Dublin, Ireland

Search for other papers by Diarmuid Smith in
Google Scholar
PubMed
Close
, and
Amar Agha Department of Endocrinology and Diabetes, Beaumont Hospital Dublin, Dublin, Ireland

Search for other papers by Amar Agha in
Google Scholar
PubMed
Close

Summary

This case is the first to describe a patient who experienced concomitant agranulocytosis and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis as an adverse effect of propylthiouracil treatment for Graves’ disease. A 42-year-old female with Graves’ disease presented to the emergency department (ED) with a 2-week history of fevers, night sweats, transient lower limb rash, arthralgia, myalgia and fatigue. She had been taking propylthiouracil for 18 months prior to presentation. On admission, agranulocytosis was evident with a neutrophil count of 0.36 × 109/L and immediately propylthiouracil was stopped. There was no evidence of active infection and the patient was treated with broad-spectrum antibodies and one dose of granulocyte colony-stimulation factor, resulting in a satisfactory response. On further investigation, ANCAs were positive with dual positivity for proteinase 3 and myeloperoxidase. There was no evidence of end-organ damage secondary to vasculitis, and the patient’s constitutional symptoms resolved completely on discontinuation of the drug precluding the need for immunosuppressive therapy.

Learning points:

  • Continued vigilance and patient education regarding the risk of antithyroid drug-induced agranulocytosis is vital throughout the course of treatment.

  • ANCA-associated vasculitis is a rare adverse effect of antithyroid drug use.

  • Timely discontinuation of the offending drug is vital in reducing end-organ damage and the need for immunosuppressive therapy in drug-induced ANCA-associated vasculitis.

  • Similarities in the pathogenesis of agranulocytosis and drug-induced ANCA-associated vasculitis may offer insight into an improved understanding of vasculitis and agranulocytosis.

Open access
Masato Kotani Center for Diabetes, Endocrinology and Metabolism
Research Support Center, Shizuoka General Hospital, Shizuoka, Shizuoka, Japan
Asahina Shinryoujo, Fujieda, Shizuoka, Japan

Search for other papers by Masato Kotani in
Google Scholar
PubMed
Close
,
Naohisa Tamura Center for Diabetes, Endocrinology and Metabolism
Research Support Center, Shizuoka General Hospital, Shizuoka, Shizuoka, Japan

Search for other papers by Naohisa Tamura in
Google Scholar
PubMed
Close
,
Tatsuhide Inoue Center for Diabetes, Endocrinology and Metabolism

Search for other papers by Tatsuhide Inoue in
Google Scholar
PubMed
Close
, and
Issei Tanaka Center for Diabetes, Endocrinology and Metabolism

Search for other papers by Issei Tanaka in
Google Scholar
PubMed
Close

Summary

Type B insulin resistance syndrome is characterized by the presence of autoantibodies to the insulin receptor. We present a 57-year-old male admitted to a hospital due to body weight loss of 16 kg and hyperglycemia of 13.6 mmol/L. He was diagnosed with type B insulin resistance syndrome because the anti-insulin receptor antibodies were positive. We informed him that some hyperglycemic cases of this syndrome had been reported to be spontaneously remitted in 5 years, and he did not agree to be treated with high-dose glucocorticoids and/or immunosuppressive agents due to his concern for their adverse effects such as hyperglycemia and immunosuppression. He chose to be treated with insulin and voglibose, but fair glucose control could not be obtained. Six years later, he agreed to be treated with low-dose glucocorticoids practicable in outpatient settings. One milligram per day of betamethasone was tried orally and reduced gradually according to the values of glycated hemoglobin. After 30 months of glucocorticoid treatment, the anti-insulin receptor antibodies became undetectable and his fasting plasma glucose and glycated hemoglobin were normalized. This case suggests that low-dose glucocorticoids could be a choice to treat type B insulin resistance syndrome in outpatient settings.

Learning points:

  • Type B insulin resistance syndrome is an acquired autoimmune disease for insulin receptors.

  • This case suggested the possibility of long-lasting, low-dose glucocorticoid therapy for the syndrome as an alternative for high-dose glucocorticoids or immunosuppressive agents.

  • Since the prevalence of autoimmune nephritis is high in the syndrome, a delay of immunosuppressive therapy initiation might result in an exacerbation of nephropathy.

Open access
Shunsuke Funazaki Division of Endocrinology and Metabolism Department of Medicine, Jichi Medical University Saitama Medical Center, Saitama, Japan

Search for other papers by Shunsuke Funazaki in
Google Scholar
PubMed
Close
,
Hodaka Yamada Division of Endocrinology and Metabolism Department of Medicine, Jichi Medical University Saitama Medical Center, Saitama, Japan

Search for other papers by Hodaka Yamada in
Google Scholar
PubMed
Close
,
Kazuo Hara Division of Endocrinology and Metabolism Department of Medicine, Jichi Medical University Saitama Medical Center, Saitama, Japan

Search for other papers by Kazuo Hara in
Google Scholar
PubMed
Close
, and
San-e Ishikawa Division of Endocrinology and Metabolism Department of Medicine, Jichi Medical University Saitama Medical Center, Saitama, Japan
Division of Endocrinology and Metabolism Division of Endocrinology and Metabolism, International University of Health and Welfare Hospital, Tochigi, Japan

Search for other papers by San-e Ishikawa in
Google Scholar
PubMed
Close

Summary

Lymphocytic hypophysitis (LyH) has been known to be associated with pregnancy. We herein report the case of a 33-year-old woman who underwent vaginal delivery without massive bleeding at 40 weeks of gestation. Because of the presence of headache and terrible fatigue after childbirth, she visited our hospital. Severe hyponatremia (Na, 118 mEq/L) and visual field abnormality was noted upon examination. MRI revealed pituitary enlargement with a swollen pituitary stalk, albeit at low signal intensity. Basal pituitary hormone levels were all reduced and remained low after exogenous administration of hypothalamic-releasing hormones. She was diagnosed with LyH and was started on prednisolone 60 mg/day. A month later, her pituitary function had gradually improved together with a decrease in pituitary enlargement and recovery of her visual field. The dose of prednisolone was gradually reduced and finally withdrawn 27 months later. After prednisolone withdrawal, her pituitary function remained normal despite the absence of any hormonal replacement. A year later, she became pregnant without medication and delivered a second baby without LyH recurrence. Thereafter, her pituitary function has been normal for more than 5 years. Two valuable observations can be highlighted from the case. First, the patient completely recovered from LyH through prompt prednisolone therapy during its initial phase and had almost normal pituitary function. Second, after recovery from LyH, she was able to undergo spontaneous pregnancy and deliver a baby. We believe that reporting incidences of spontaneous pregnancy after complete normalization of pituitary function in patients with LyH is of great significance.

Learning points:

  • Females are more affected by LyH than males given its strong association with pregnancy.

  • LyH possesses characteristic findings on pituitary MRI.

  • Glucocorticoid therapy for LyH has been recommended as an effective treatment.

  • A history of previous pregnancies does not increase the risk of developing AH in subsequent pregnancies.

  • Early induction of high-dose prednisolone was therapeutically effective in treating LyH.

Open access
S Hussain Department of Diabetes and Endocrinology, Newham University Hospital, Barts Health NHS Trust, London, UK

Search for other papers by S Hussain in
Google Scholar
PubMed
Close
,
S Keat Department of Diabetes and Endocrinology, Newham University Hospital, Barts Health NHS Trust, London, UK

Search for other papers by S Keat in
Google Scholar
PubMed
Close
, and
S V Gelding Department of Diabetes and Endocrinology, Newham University Hospital, Barts Health NHS Trust, London, UK

Search for other papers by S V Gelding in
Google Scholar
PubMed
Close

Summary

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.

Open access
Laila Ennazk Department of Endocrinology, Diabetology and Metabolic Diseases, Mohammed VI University Hospital of Marrakech, Caddi Ayyad University, Marrakech, Morocco

Search for other papers by Laila Ennazk in
Google Scholar
PubMed
Close
,
Ghizlane El Mghari Department of Endocrinology, Diabetology and Metabolic Diseases, Mohammed VI University Hospital of Marrakech, Caddi Ayyad University, Marrakech, Morocco

Search for other papers by Ghizlane El Mghari in
Google Scholar
PubMed
Close
, and
Nawal El Ansari Department of Endocrinology, Diabetology and Metabolic Diseases, Mohammed VI University Hospital of Marrakech, Caddi Ayyad University, Marrakech, Morocco

Search for other papers by Nawal El Ansari in
Google Scholar
PubMed
Close

Summary

Autoimmune pancreatitis is a new nosological entity in which a lymphocytic infiltration of the exocrine pancreas is involved. The concomitant onset of autoimmune pancreatitis and type 1 diabetes has been recently described suggesting a unique immune disturbance that compromises the pancreatic endocrine and exocrine functions. We report a case of type1 diabetes onset associated with an autoimmune pancreatitis in a young patient who seemed to present a type 2 autoimmune polyglandular syndrome. This rare association offers the opportunity to better understand pancreatic autoimmune disorders in type 1 diabetes.

Learning points:

  • The case makes it possible to understand the possibility of a simultaneous disturbance of the endocrine and exocrine function of the same organ by one autoimmune process.

  • The diagnosis of type 1 diabetes should make practitioner seek other autoimmune diseases. It is recommended to screen for autoimmune thyroiditis and celiac diseases. We draw attention to consider the autoimmune origin of a pancreatitis associated to type1 diabetes.

  • Autoimmune pancreatitis is a novel rare entity that should be known as it is part of the IgG4-related disease spectrum.

Open access
Hiroto Minamino The First Department of Medicine, Wakayama Medical University, 811-1, Kimiidera, Wakayama, 641-8509, Japan
Department of Diabetes and Endocrinology, Wakayama Red Cross Hospital, Wakayama, Japan

Search for other papers by Hiroto Minamino in
Google Scholar
PubMed
Close
,
Hidefumi Inaba The First Department of Medicine, Wakayama Medical University, 811-1, Kimiidera, Wakayama, 641-8509, Japan

Search for other papers by Hidefumi Inaba in
Google Scholar
PubMed
Close
,
Hiroyuki Ariyasu The First Department of Medicine, Wakayama Medical University, 811-1, Kimiidera, Wakayama, 641-8509, Japan

Search for other papers by Hiroyuki Ariyasu in
Google Scholar
PubMed
Close
,
Hiroto Furuta The First Department of Medicine, Wakayama Medical University, 811-1, Kimiidera, Wakayama, 641-8509, Japan

Search for other papers by Hiroto Furuta in
Google Scholar
PubMed
Close
,
Masahiro Nishi The First Department of Medicine, Wakayama Medical University, 811-1, Kimiidera, Wakayama, 641-8509, Japan

Search for other papers by Masahiro Nishi in
Google Scholar
PubMed
Close
,
Takashi Yoshimasu Department of Dermatology, Wakayama Medical University, Wakayama, Japan

Search for other papers by Takashi Yoshimasu in
Google Scholar
PubMed
Close
,
Akinori Nishikawa Department of Hematology, Wakayama Medical University, Wakayama, Japan

Search for other papers by Akinori Nishikawa in
Google Scholar
PubMed
Close
,
Masanori Nakanishi Department of Respiratory Medicine & Medical Oncology, Wakayama Medical University, Wakayama, Japan

Search for other papers by Masanori Nakanishi in
Google Scholar
PubMed
Close
,
Shigeki Tsuchihashi Department of Otolaryngology, Wakayama Medical University, Wakayama, Japan

Search for other papers by Shigeki Tsuchihashi in
Google Scholar
PubMed
Close
,
Fumiyoshi Kojima Department of Human Pathology, Wakayama Medical University, Wakayama, Japan

Search for other papers by Fumiyoshi Kojima in
Google Scholar
PubMed
Close
,
Shin-ichi Murata Department of Human Pathology, Wakayama Medical University, Wakayama, Japan

Search for other papers by Shin-ichi Murata in
Google Scholar
PubMed
Close
,
Gen Inoue Department of Diabetes and Endocrinology, Wakayama Red Cross Hospital, Wakayama, Japan

Search for other papers by Gen Inoue in
Google Scholar
PubMed
Close
, and
Takashi Akamizu The First Department of Medicine, Wakayama Medical University, 811-1, Kimiidera, Wakayama, 641-8509, Japan

Search for other papers by Takashi Akamizu in
Google Scholar
PubMed
Close

Summary

A 73-year-old man with Hashimoto's thyroiditis (HT) suffered from purpura on the lower legs. He was diagnosed with IgG4-related disease (IgG4-RD) with serum IgG4 elevation and dacryo-sialadenitis confirmed histologically. Serum Th2 and Treg cytokines, interleukin 7 (IL7), IL8 and Th2 chemokine levels were elevated, while skewed Th1 balance was seen in fluorescence-activated cell sorting (FACS). Therefore, preferential Th1 balance in HT appeared to be followed by IgG4-RD characterized with Th2 and Treg polarization. The commencement of steroid therapy dramatically exacerbated clinical manifestations including IgG4-RD-associated HT. The measurement of cytokine and chemokine levels as well as FACS analysis in the development of IgG4-RD seemed to be beneficial. In conclusion, an innovative association of HT, IgG4-RD and vasculitis was observed. This report also offers novel diagnostic and therapeutic approaches for IgG4-RD.

Learning points

  • Recently, a subtype of HT has been considered to be a thyroid manifestation of IgG4-RD, although the etiology of IgG4-RD is not established yet.

  • Immunologically a close association between HT and vasculitis was reported.

  • Leukocytoclastic vasculitis is a rare skin presentation of IgG4-RD.

  • In the current case, during the course of HT, IgG4-RD and leukocytoclastic vasculitis occurred; thus, innate immunity and acquired immunity seem to be involved in the development of IgG4-RD.

  • The measurement of cytokine and chemokines appeared to be beneficial in the development of IgG4-RD.

  • Remarkably, effectiveness of steroid therapy for HT suggested presence of IgG4-RD-associated HT. Therefore, this report highlights the pathogenesis of IgG4-RD and proposes novel therapeutic mechanisms. Clinicians should pay attention to the development of IgG4-RD and vasculitis during long course of HT.

Open access
Jingjing Jiang Department of Endocrinology and Metabolism, Zhongshan Hospital, Fudan University, Shanghai, 200032, People's Republic of China

Search for other papers by Jingjing Jiang in
Google Scholar
PubMed
Close
,
Mei Zhang Department of Endocrinology, The First Affiliated Hospital of Nanjing Medical University, 300 Guangzhou Road, Nanjing, 210029, People's Republic of China

Search for other papers by Mei Zhang in
Google Scholar
PubMed
Close
,
Ronghua He Department of Endocrinology, The First Affiliated Hospital of Nanjing Medical University, 300 Guangzhou Road, Nanjing, 210029, People's Republic of China

Search for other papers by Ronghua He in
Google Scholar
PubMed
Close
,
Meiping Shen Department of General Surgery, The First Affiliated Hospital of Nanjing Medical University, Nanjing, 210029, People's Republic of China

Search for other papers by Meiping Shen in
Google Scholar
PubMed
Close
, and
Wei Liu Department of Nuclear Medicine, The First Affiliated Hospital of Nanjing Medical University, Nanjing, 210029, People's Republic of China

Search for other papers by Wei Liu in
Google Scholar
PubMed
Close

Summary

Functional parathyroid cysts are a rare cause of primary hyperparathyroidism and are often mistaken for thyroid cysts. Systemic lupus erythematosus (SLE) is also a very rare cause of hypercalcemia. We report the case of a 62-year-old woman, who was diagnosed with SLE 30 years ago, presenting with clinical and biochemical features of primary hyperparathyroidism. Laboratory investigation revealed increased serum calcium and parathyroid hormone (PTH) levels; neck ultrasonography (USG) revealed 40×34×26 mm cystic mass in the left lobe of thyroid gland. PTH level in the cysts was >2500 pg/ml, determined by USG-guided fine-needle aspiration (FNA). In this case, no evidence for potential pathogenic association between parathyroid cyst and SLE was uncovered. However, the recognition of this association is very important because the therapeutical strategy is completely different. Operative management is usually straightforward and alleviates symptoms and any biochemical abnormalities caused by the cyst.

Learning points

  • Functional parathyroid cysts are the rare cause of primary hyperparathyroidism and are often mistaken for thyroid cysts.

  • SLE is also a very rare cause of hypercalcemia.

  • Ultrasound-guided FNA of cystic fluid with assay for PTH level is an accurate method of differentiating parathyroid cyst from thyroid cyst.

  • Appropriate management of functional parathyroid cysts is surgical excision.

Open access