scholarly journals An Case of Poems Syndrome With Primary Adrenal Insufficiency

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A114-A115
Author(s):  
Anne Sillars ◽  
Stephen Cleland ◽  
Jillian Tough

Abstract POEMS syndrome is rare multi-system disorder characterised by a paraneoplastic plasma cell disorder. The acronym POEMS stands for Polyneuropathy, Organomegaly, Endocrinopathy, M protein, and Skin changes. The underlying mechanisms are poorly understood, but chronic overproduction of pro-inflammatory cytokines have an integral role in the disorder. Diagnosis of POEMS is difficult owing to the variety of clinical manifestations. Mandatory diagnostic criteria are (a) one major criterion of either polyneuropathy or monoclonal gammopathy, in association with (b) one minor criterion including, but not limited to, organomegaly, endocrinopathy or skin changes. Endocrinopathies have been identified in 67–84% of patients with POEMS [1]. While hypogonadism and hypothyroidism are relatively common, primary adrenal failure is rarely reported. We present a 54-year-old woman who was found to have a raised hemoglobin, hematocrit and thrombocytosis on routine blood testing. She was concomitantly investigated for a rapidly-ascending, bilateral peripheral motor and sensory neuropathy. Diagnosis of POEMS was made and she underwent chemotherapy with Lenalidomide and high dose dexamethasone in 2015. Since then, she has not received endogenous glucocorticoids. She had an unplanned admission in April 2020 with a likely viral illness and was found to be glucocorticoid deplete. A 250-mcg short synacthen test demonstrated an inadequate response in cortisol, from 4.31 µg/dl to 6.13 µg/dl, with a raised adrenocorticotrophic hormone (ACTH) level of 66 mU/L. Adrenal antibodies were not detected. CT scan of the abdomen reported no adrenal gland abnormalities. The patient denied symptoms of mineralocorticoid deficiency, with no postural blood pressure (BP) drop - sitting BP 123/69 mmHg and standing BP 131/74 mmHg. Serum electrolytes were normal (renin and aldosterone levels are pending) and androgen screen was within normal limits. Thyroid stimulating hormone (TSH) was 8.07 mIU/L, free thyroxine (fT4) levels 0.93 ng/dL, and thyroid receptor and thyroid peroxidase antibodies were both negative. The patient is now established on Hydrocortisone therapy: 10mg (morning), 5mg (lunchtime). In summary, we present a 54-year-old woman with POEMS syndrome with subacute primary adrenal failure, characterised by glucocorticoid deficiency and ACTH excess. Although rare, it is important for all clinicians to be aware of POEMS syndrome as a potential diagnosis if the diagnostic criteria described above are filled, and for Endocrinologists to be aware that POEMS endocrinopathies can occur in any gland, including the adrenal glands. 1.Gandhi, G.Y., et al., Endocrinopathy in POEMS syndrome: the Mayo Clinic experience. Mayo Clin Proc, 2007. 82(7): p. 836–42.

2018 ◽  
Vol 90 (2) ◽  
pp. 133-137 ◽  
Author(s):  
Tomoki Suichi ◽  
Sonoko Misawa ◽  
Yasunori Sato ◽  
Minako Beppu ◽  
Emiko Sakaida ◽  
...  

ObjectiveTo propose the optimal diagnostic criteria for polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS) syndrome using appropriate statistical methods and disease controls.MethodsThis retrospective cohort study included 104 consecutive patients with suspected POEMS syndrome, among whom a gold standard group of 60 patients with definitive POEMS syndrome diagnosis were followed for at least 12 months to strictly exclude other disorders mimicking POEMS syndrome and to confirm response to POEMS syndrome-specific treatment. Thirty patients with chronic inflammatory demyelinating polyradiculoneuropathy (demyelinating polyradiculoneuropathy controls) and 30 with multiple myeloma or immunoglobulin light chain amyloidosis (monoclonal plasma cell proliferation controls) were also included. Logistic regression analyses were performed to determine optimal combination of clinical and laboratory abnormalities, characteristic of POEMS syndrome.ResultsThe diagnostic criteria were statistically defined as the presence of the three major criteria (polyneuropathy (typically demyelinating), monoclonal plasma cell proliferative disorder and elevated vascular endothelial growth factor) and at least two of the four minor criteria (oedema/effusion, skin changes, organomegaly and sclerotic bone lesions), based on best performance by area under the receiver operating characteristic curve analyses. The sensitivity and specificity were 100% and 100%, respectively; the diagnostic accuracy of the proposed criteria was equivalent to somewhat complicated previous criteria.ConclusionsThe statistically defined, simple diagnostic criteria for POEMS syndrome could accelerate early diagnosis and treatment, thereby contribute to better outcome in patients with this serious disease. Prospective larger studies are required to confirm the validity.


2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Zong Fei Ji ◽  
Dan Ying Zhang ◽  
Shu Qiang Weng ◽  
Xi Zhong Shen ◽  
Hou Yu Liu ◽  
...  

POEMS syndrome is a rare paraneoplastic disorder associated with an underlying plasma cell dyscrasia presenting polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. This study reviewed the clinical characteristics of 14 POEMS patients in Zhongshan hospital. The ratio of male to female was 9 : 5, and the average age was 47.1 years. The clinical manifestations were various, including motorial symptoms (weakness), sensory symptoms (numbness), lymphadenopathy, edema, abdominal distention, and skin hyperpigmentation. Imaging studies and laboratory tests also exhibited hepatomegaly, splenomegaly, thrombocytosis, endocrinopathy, and positive serum immunofixation in most patients. In addition, increased plasma cells in bone marrow and Castleman Disease were found in bone marrow and lymph nodes biopsies. All the eight follow-up patients were treated with alkylator-based combination chemotherapy or corticosteroids and thalidomide, with or without autologous stem cell transplantation. Unfortunately, two patients died three or four years after diagnosis of POEMS syndrome. The others showed response to therapy to some extent, but not completely remission. Currently, treatments for POEMS include radiation to the plasmacytoma, and systemic therapy is indicated. Low-dose alkylators with or without corticosteroids are effective in some patients. However, high-dose chemotherapy with auto-SCT dramatically improved symptoms and outcomes for POEMS patients.


2020 ◽  
Vol 11 (4) ◽  
pp. 55-68
Author(s):  
Polina A. Sobolevskaia ◽  
Boris V. Andreev ◽  
Anton N. Gvozdetckii ◽  
Anastasia A. Dolina ◽  
Anna M. Stepochkina ◽  
...  

Hashimoto thyroiditis is the most common thyroid disease. This form of pathology has a diverse clinical picture, including neuropsychiatric disorders. There are frequent cases of comorbidity of autoimmune thyroiditis and psychiatric forms of pathology, along with such a nosological entity as Hashimotos encephalopathy (aka: Steroid-responsive encephalopathy of autoimmune thyroiditis), characterized by an increased level of antithyroid autoantibodies and various mental disorders, with still unclear pathogenesis. The question arises, how to regard patients with psychiatric disorders and Hashimoto thyroiditis either as patients having autoimmune thyroiditis, comorbid with psychiatric forms of pathology, or as patients with Hashimotos encephalopathy? We studied groups of patients with autoimmune thyroiditis free from any psychiatric disorders, autoimmune thyroiditis comorbid with psychiatric forms of pathology, and a group of healthy donors similar as regards to their age and sex. We also studied medical history, clinical manifestations of the disease, instrumental data and the serum levels of thyrotropin, thyroid hormones, various antithyroid autoantibodies, and prolactin. We analyzed the correlation of laboratory and instrumental parameters and clinical data in all groups of patients. Therewas a significant relationship (p 0,05) between various psychiatric symptoms and a decreased level of free thyroxine, an increased level of thyroid stimulating hormone (TSH), an increased level of prolactin and an increased volume of a thyroid gland. Asignificant relationship (p 0,05) was also found between various symptoms of hypothyroidism and a decreased level of free triiodothyronine (FT3), an increased level of antibodies to thyroglobulin (anti-TG Ab), and an increased level of antibodies to thyroid peroxidase (anti-TPO Ab).


2020 ◽  
pp. 206-212
Author(s):  
A. F. Verbovoy ◽  
Yu. A. Dolgikh

Hypothyroidism is the most common endocrine disease after diabetes mellitus. Its frequency depends on age, sex and iodine intake. The highest prevalence of hypothyroidism is observed in older women. Chronic autoimmune thyroiditis is the most common cause of this condition. The peculiarity of hypothyroidism is an erased clinical picture, diversity and nonspecific symptoms. This makes it difficult to diagnose the disease, leads to an erroneous diagnosis and later detection of thyroid insufficiency. This article discusses the various «masks» of hypothyroidism and peculiarities of clinical manifestations. The main «masks» are: cardiological, dermatological, urological, gastroenterological, endocrine and reproductive system disorders, neurological, psychiatric, hematological, rheumatological. Free thyroxine and thyroid-stimulating hormone are used to diagnose hypothyroidism, as well as antibody titer to thyroid peroxidase and thyroglobulin to detect chronic autoimmune thyroiditis. Levothyroxine preparations are used as a substitution therapy. The dose of the drug depends on the age of the patient and the presence of cardiovascular disease. Patients under 50 years of age without a severe concomitant cardiovascular disease are given 1.6 µg of levothyroxine per kg of body weight. In persons over 50 years of age with cardiovascular diseases, the drug dose is prescribed at the rate of 0.9 µg per kg of body weight. The therapy starts with small doses, slowly increasing it under the control of electrocardiography. At occurrence or strengthening of symptoms of angina a dose of levothyroxine is reduced to the previous one and the cardiovascular therapy is corrected. Evaluation of the effectiveness of the treatment is carried out on the level of thyroid hormone.


Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4612-4612
Author(s):  
Andrea Nozza ◽  
Fabrizia Terenghi ◽  
Rita Mazza ◽  
Eduardo Nobile Orazio ◽  
Fausto Adami ◽  
...  

Abstract Abstract 4612 Introduction: POEMS syndrome is a rare multisystemic disease. Vascular endothelial growth factor (VEGF) correlates with the activity of the disease and it could account for clinical manifestations. Actually no controlled trials are available and there is no clear evidence for a standard therapeutic approach. Lenalidomide has anti-angiogenic activity through inhibition of VEGF and TNF alpha The aim of this study is to evaluate efficacy and safety of Lenalidomide in POEMS syndrome. Patients & method: From 10/09, we started a pilot study with Lenalidomide plus dexamethasone (RD) in pretreated or newly diagnosed POEMS patients not eligible for transplant procedure. Lenalidomide 25 mg/day was given for 21 days in association with weekly dexamethasone 40 mg until toxicity or progression occurred. After 6 cycles, pts were evaluated for response. Utonow 10pts have been enrolled, 9 men and 1 woman; median age was 51 years (range 45–76). All pts were pretreated and 2 pts had previously received high-dose melphalan with peripheral blood stem cell transplant. Monoclonal component (MC) was detected in all pts: IgAl in 3 pts, IgG l in 6 pts, l light chain in 1; all pts had sensory/motor peripheral neuropathy. Sclerotic bone lesions were detected in 5 pts, endocrinopathies in 8 pts, skin changes in 8 pts, peripheral edema in 8 pts, organomegaly in 7 pts, lymphoadenophaty in 3 pts, papilledema in 7 pts, thrombocytosis in 3 pts. VEGF serum level was elevated in all patient with a median value of 3544 pg/ml (range 1430–9788). Results: Seven pts are still on treatment with a median of 10 RD cycles (range 5–18). Six pts are evaluable for response after 6 cycles. A clinical response, with improvement of all disease manifestations, was observed in all pts. Neurological improvement was observed after 3 RD cycles, and was confirmed by nerve conduction studies after 6 cycles. One patient with tetraparesis is currently able to walk and his upper limb strength is normal with improved sensory neuropathy. MC disappeared in 2 patients. VEGF levels decreased in all pts: from a median of 3544 pg/ml (range 1430–9788) before treatment to 1539 pg/ml (467-3579) after 6 cycles. Three pts discontinued treatment: 1 withdrew consensus, 1 dropped out after 4 cycles for progression and 1 died for pulmonary infection on cycle 1. Dexamethasone dose adjustment was necessary in 5 pts and lenalidomide in 2 pts, (1 extraematological toxicity and 11 thrombocytopenia). Conclusions: This preliminary analysis,although in a limited series, highlights efficacy and safety of Lenalidomide in POEMS. Noteworthy the neurological improvement has been rapid and continuous, also in heavily pre-treated pts. At this time no patients experienced disease progression. A clear relationship between response and VEGF level was confirmed. The study accrual is ongoing. Disclosures: Off Label Use: Bortezomib and Thalidomide as induction therapy prior to and consolidation therapy after double autologous stem-cell transplantation in newly diagnosed multiple myeloma.


2019 ◽  
Vol 7 ◽  
pp. 2050313X1983953 ◽  
Author(s):  
Shanna Ariane Tucker ◽  
Justin Choi ◽  
Dhruv Khullar

In this clinical vignette, we present a case of a 59-year-old woman with catastrophic antiphospholipid syndrome likely triggered by polymicrobial sepsis. The diagnostic criteria and clinical manifestations of catastrophic antiphospholipid syndrome are reviewed. We also compare diagnostic criteria and clinical manifestations with other clinical entities in the differential diagnosis, including thrombotic thrombocytopenic purpura–hemolytic-uremic syndrome, disseminated intravascular coagulation, sepsis, and inflammatory bowel disease. Catastrophic antiphospholipid syndrome is a rare, but lethal condition, and treatment recommendations are based on expert consensus and analyses of the international Catastrophic Antiphospholipid Syndrome Registry. Current management guidelines recommend triple therapy, with anticoagulation, glucocorticoids, and plasma exchange or intravenous immunoglobulins. This case brings this rare clinical entity to the attention of clinicians and emphasizes the need for more research to understand the best management. It also raises the question of whether high-dose steroids should be continued for treatment of catastrophic antiphospholipid syndrome in the setting of a severe sepsis.


2020 ◽  
Vol 11 (1) ◽  
Author(s):  
Mouzarllem B. Reis ◽  
Fernanda L. Rodrigues ◽  
Natalia Lautherbach ◽  
Alexandre Kanashiro ◽  
Carlos A. Sorgi ◽  
...  

Abstract Scorpion envenomation is a leading cause of morbidity and mortality among accidents caused by venomous animals. Major clinical manifestations that precede death after scorpion envenomation include heart failure and pulmonary edema. Here, we demonstrate that cardiac dysfunction and fatal outcomes caused by lethal scorpion envenomation in mice are mediated by a neuro-immune interaction linking IL-1 receptor signaling, prostaglandin E2, and acetylcholine release. IL-1R deficiency, the treatment with a high dose of dexamethasone or blockage of parasympathetic signaling using atropine or vagotomy, abolished heart failure and mortality of envenomed mice. Therefore, we propose the use of dexamethasone administration very early after envenomation, even before antiserum, to inhibit the production of inflammatory mediators and acetylcholine release, and to reduce the risk of death.


Author(s):  
Olha Kasiyan ◽  
Halyna Tkachenko ◽  
Natalia Kurhaluk ◽  
Svitlana Yurchenko ◽  
Alek Manenko

AbstractThe current study aimed to identify correlative and regressive dependencies between the water iodine concentration and the levels of TSH (thyroid-stimulating hormone), thyroglobulin antibodies (TgAbs), and thyroid peroxidase (TPOAb) in the serum of 168 in patients (34 men and 134 women) with a hypothyroid form of Hashimoto’s thyroiditis who use water from the supply network and individual wells. Based on the water iodine concentration, low and moderate degrees of iodine endemia in the location of the patients were determined. In the groups of men and women using water from different water supply sources, there were direct correlations between the water iodine concentrations and the TgAbs and TPOAb titers as well as an inverse dependence between iodine and TSH levels. Multivariate regressive analysis indicated that TgAb and TSH in the group of women using water from a supply network and TPOAb titers in the group of women using well water were independent factors associated with water iodine concentrations. Statistically significant correlations and regressive dependencies between the water iodine concentrations and the biomarkers of the thyroid status of the patients indicate the risk of Hashimoto’s thyroiditis progression, especially among women with additional iodine intake.


2020 ◽  
Author(s):  
Xiao-an Pang ◽  
Zhi-xiao Wei ◽  
Jun-hong Li ◽  
Xiao-qi Pang

Abstract Background Hashimoto’s thyroiditis (HT) may cause salivary dysfunction in patients resulting in xerostomia, but little is known about changes in salivary function in patients with no obvious dry mouth symptoms. In this study we assessed salivary function in women with HT, who had not experienced xerostomia and, for the first time, evaluated the effects of thyroid auto-antibodies on this function. Methods Sixty consecutive subjects were included, comprising 32 women (mean age, 36 ± 12 years) diagnosed with HT accompanied by differentiated thyroid cancer (DTC) in the study group (HT group), along with a control group (DTC group) of 28 women (mean age, 40 ± 12 years) diagnosed with DTC only. Salivary gland scintigraphy was used to assess salivary function with the semi-quantitative parameters of maximum absorption ratio and maximum secretion ratio, the decrease of which indicate impaired salivary function. Moreover, the HT and DTC groups were divided into four subgroups (Anti– HT, Anti+ HT, Anti– DTC, and Anti+ DTC), based on the presence of anti-thyroid peroxidase antibody (TPOAb) and anti-thyroglobulin antibody (TgAb). Finally, salivary gland semi-quantitative parameters were correlated with levels of thyroid-stimulating hormone (TSH), TGAb, and TPOAb in the HT and DTC groups. Results None of the semi-quantitative parameters examined in parotid or submandibular glands differed significantly between the HT and DTC groups. However, the maximum secretion ratio for the parotid and submandibular glands were significantly different in the subgroup comparison (p < 0.05). Furthermore, the TgAb, TPOAb, and TSH values correlated significantly with salivary excretive function (p ≤ 0.05). Conclusion Women with HT without xerostomia may not have salivary functional impairment during hypothyroidism. Serum thyroid autoantibody and TSH levels may mainly influence salivary excretive function but not uptake function.


Author(s):  
Jing Song ◽  
Rongxin Sun ◽  
Yuanyuan Zhang ◽  
Ying Fu ◽  
Dong Zhao

Abstract Objective Resolvins are produced by the catabolism of polyunsaturated fatty acids (PUFAs) and play vital roles in inflammation resolution. Resolvins have been associated with autoimmune disorders. This study aimed to measure the level of Resolvin D1 (RVD1) in the serum of Hashimoto's thyroiditis (HT) patients and healthy controls (HCs) and to further analyse its correlation with thyroid autoantibodies and inflammatory factors. Methods Sixty-three participants were recruited, namely, 30 untreated HT patients and 33 sex- and age-matched HCs. Serum RVD1 and inflammatory chemokine (MCP-1 and IP-10) levels were measured by ELISA according to the manufacturer’s protocol. Serum total T3 (TT3), TT4, free T3 (FT3), FT4, thyroglobulin antibody (TgAb), thyroid peroxidase antibody (TPOAb) and thyroid-stimulating hormone (TSH) levels were measured using an electrochemiluminescence immunoassay. Thyroid homeostasis parameters, including the thyroid secretory capacity (SPINA-GT), the total deiodinase activity (SPINA-GD), Jostel’s TSH index (TSHI) and the thyrotroph thyroid hormone sensitivity index (TTSI), were calculated. Results Serum RVD1 levels in HT patients (134.76, 85.35–201.36 pg/mL) were significantly lower than those in HCs (187.64, 131.01–326.85 pg/mL) (P=0.004). As the TPOAb level increased, the RVD1 level showed a decreasing trend (P for trend=0.002). Both multinomial and ordinal logistics analyses revealed that serum RVD1 levels were negatively correlated with TPOAb levels in the adjusted models. Moreover, RVD1 showed a negative correlation with the inflammatory chemokine IP-1 0 (r=–0.276, P=0.034), TSHI (r=–0.269, P=0.036) and TTSI (r=–0.277, P=0.031). Conclusions Thyroid autoimmunity may be associated with low levels of RVD1. Decreased RVD1 levels indicate impaired resolution of inflammation in HT patients.


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