Utility of myxedema score as a predictor of mortality in myxedema coma.

OBJECTIVE: Myxedema crisis (MC) is a rare condition. There is a dearth of data regarding the predictors of mortality in MC. Predictive scores for mortality specific to the clinical and biochemical profile of MC are still lacking. DESIGN AND METHODS: All consecutive patients presenting with MC from September 2006 to December 2020 comprised the new cohort. Patients managed between January 1999 and August 2006 comprised the old cohort. Both cohorts were compared for the determination of secular trends. Combined analysis of both the cohorts was done for clinico-demographic profile and predictors of mortality. Myxedema score (MS) and qSOFA (Quick Sequential Organ Failure Assessment) score were evaluated in all the patients. RESULTS: A total of forty-one patients (new cohort; n = 18 and old cohort; n = 23) were enrolled into the study. There was a female predominance (80.5%). Nearly half (51.2%) of the patients were newly diagnosed with hypothyroidism on admission. Overall mortality was 60.9%. On comparative analysis among survivors and non-survivors, female gender (OR 20.4, p value 0.018), need for mechanical ventilation (OR16.4, p value 0.009), in-hospital hypotension (OR 9.1, p value 0.020), and high qSOFA score (OR 7.1, p value 0.023) predicted mortality. MS of > 90 had significantly higher mortality (OR-11.8, p value - 0.026) while MS of > 110 had 100% mortality. There was no change in secular trends over last 20 years. There was no difference in outcome of patients receiving oral or IV levothyroxine. CONCLUSION: Myxedema crisis is associated with high mortality despite improvement in health care services. The current study is first to elucidate the role of the MS in predicting mortality in patients with MC.

Localized alopecic myxedema of the scalp.

Myxedema is a rare, cutaneous complication of autoimmune thyroid diseases that most often affects the anterior shins. Herein, we report a patient with a history of Graves disease and Hashimoto thyroiditis who presented with boggy, alopecic patches associated with scalp pruritus. Punch biopsies from these lesions showed increased interstitial mucin in the reticular dermis, consistent with localized myxedema. This report showcases a rare presentation of localized myxedema of the scalp, highlighting the diverse cutaneous manifestations of autoimmune thyroid diseases.

Novel teprotumumab treatment of severe thyroid dermopathy; ototoxicity as an adverse side effect.

Pretibial myxedema, more generally thyroid dermopathy, results from mucopolysaccharide accumulation in the dermis, typically between the knee and dorsal foot. Thyroid dermopathy presents in Graves disease, but can occur in Hashimoto thyroiditis, primary hypothyroidism, and euthyroid patients. Treatment of thyroid eye disease with teprotumumab is established in the literature, with few case reports also showing improvement in pretibial myxedema. Reported is a 76-year-old man with thyroid eye disease and pretibial myxedema treated with teprotumumab; improvement was demonstrated in both conditions. He developed "muffled" hearing as an adverse effect, a complication not widely published in the dermatology literature. At 18 months post-treatment, his symptoms are stable without recurrence, but hypoacusis persists. Given the long-term efficacy and side-effects, dermatologists should recognize the potential benefits and risks of using teprotumumab for thyroid dermopathy. A baseline audiogram may be considered prior to therapy. Additionally, longitudinal data is needed to document the benefits and risks of this novel therapy.

A case of fatal myxedema coma with electrocardiogram Osborne J-wave in a patient initially diagnosed with hypothyroidism.

Myxedema coma is a life-threatening endocrine emergency with a high mortality rate resulting from severe insufficiency of thyroid hormones. Intravenous levothyroxine replacement is considered the standard therapy for myxedema coma in many countries. In Japan, however, although there are diagnostic criteria highly suggestive or diagnostic for myxedema coma, no management strategy has been established, despite the availability of levothyroxine. Here we report a 75-year-old man with a history of Alzheimer's disease and schizophrenia who developed somnolence and generalized edema. Except for a pulse rate of 60 bpm, his vital signs and blood oxygen level were stable. Thyroid studies showed an elevated serum thyrotropin level of 219.2 µU/mL and a decreased serum free-thyroxine level of 0.15 ng/dL. On this basis he was diagnosed as having hypothyroidism rather than being highly suggestive for myxedema coma. Daily oral levothyroxine 25 µg was initiated and increased to 50 µg 3 days later. Seven days after being started on levothyroxine, the patient suddenly developed impaired consciousness, hypoxemia, hypotension, hypothermia, and hyponatremia. Electrocardiography revealed junctional bradycardia with Osborne J-wave. Myxedema coma was therefore diagnosed. He went into cardiac arrest in the emergency room but was resuscitated. Despite subsequent intravenous administration of hydrocortisone and levothyroxine, as well as intensive supportive care, he eventually died 12 hours after hospital admission. This case illustrates some of the challenges associated with the management of patients with signs highly suggestive/diagnostic of myxedema coma, including the optimal loading dosage and intervention timing of thyroid hormone replacement.

Improvement of Pretibial Myxedema Following Administration of Teprotumumab.

Pretibial myxedema (PTM) is a rare complication of Graves' disease. It is characterized by non-pitting edema with hyperpigmented hyperkeratotic papules and plaques on bilateral lower legs. Effective treatments for patients with PTM are lacking. The etiology of PTM is unknown; however, it may be similar to the mechanism of thyroid-associated ophthalmopathy (TAO). Activated fibroblasts produce inflammatory cytokines and synthesize excessive glycosaminoglycans (GAG) that accumulate in the dermis and subcutaneous tissue. A recent, novel pathway implicates IGF-1 receptor as a mediator in this process. We present two patients with refractory PTM that improved following treatment with teprotumumab, an IGF-1 receptor inhibitor approved for use in TAO. J Drugs Dermatol. 2022;21(11):1252-1254. doi:10.36849/JDD.6854.

Endocrine Emergencies in the Medical Intensive Care Unit.

Endocrine emergencies are underdiagnosed and often overlooked amid the management of severe multisystem pathologies in critically ill patients in the medical intensive care unit (ICU). In an appropriate clinical scenario, a low threshold of suspicion should be kept to investigate for various life-threatening, yet completely treatable, endocrinopathies. Prompt identification and treatment of endocrine emergencies such as diabetic ketoacidosis, myxedema coma, thyroid storm, and/or adrenal insufficiency leads to fewer complications, shorter ICU and hospital stay, and improved survival. This review article entails common endocrine emergencies encountered in the ICU and addresses their epidemiology, pathophysiology, clinical presentation and management.

Localized myxedema histologically mimicking spindle cell lipoma.

In this report, a 55-year-old woman with Graves disease and exophthalmos had a recurrent nodule on the foot. Her initial biopsy and excision specimens were believed to be consistent with spindle cell lipoma, which aligned with her early tumor-like clinical morphology. Her tumor recurred after excision, which is not consistent with spindle cell lipoma. As her condition progressed, her clinical morphology became more consistent with localized myxedema and her biopsies were congruent, securing clinicopathologic correlation. With standard treatment for localized myxedema, she improved significantly. This case emphasizes how clinicians need to have high suspicion for localized myxedema in patients with history of Graves disease and exophthalmos. It also emphasizes how localized myxedema should be included in the histologic differential diagnosis for spindle cell lipoma with prominent myxoid stroma, particularly in those not responding to treatment as anticipated.

A unique case of concurrent cutaneous lichen amyloidosis and myxedema.

Lichen amyloidosis is believed to be caused by damage to keratinocytes, often by chronic scratching. It has also been associated with autoimmune conditions, including thyroid disease. Dermatologic manifestations of poorly controlled thyroid disease are well described within the medical literature, within both hypothyroid and hyperthyroid states. Myxedema is a rare complication of Graves disease. We report a unique case of concurrent myxedema and lichen amyloidosis in a 63-year-old patient with uncontrolled hypothyroidism in the setting of post-ablative Graves disease.

Myxedema coma: Recognition and treatment.

ABSTRACT: Myxedema coma (MC) develops from a long-standing, unrecognized, or untreated hypothyroidism. This article discusses the pathophysiology, clinical manifestations, treatment, and nursing considerations for patients with MC.

Local immune microenvironment of skin may play an important role in the development of pretibial myxedema.

Pretibial myxedema (PTM), characterized by the accumulation of glycosaminoglycans in dermis is an autoimmune skin disorder, which is almost always associated with Graves' disease (GD). Although fibroblast stimulated by thyroid-stimulating hormone receptor (TSHR) antibody, cytokines and growth factors have been postulated as target of the autoimmune process in the dermopathy, the pathogenesis of PTM remains unclear. We hypothesize that the local immune microenvironment of the skin including the antigens and antibodies, T cells, B cells, plasma cells and fibroblasts may play an important role in the development of PTM. Results obtained on PTM patients indicate increased thyroid-stimulating hormone receptor antibodies (TRAb) in the blood positively correlate with the dermal thickness of the lesions. Further analysis shows that there were more CD3+ T cells and CD20+ B cells in the skin lesions. These T and B cells are in close contact, indicating that inducible skin-associated lymphoid tissue (iSALT) may be formed in the area. In addition, we found that the infiltrating plasma cells can secrete TRAb, proving that B cells in the skin other than the thyroid are an additional source of TSHR antibodies. Meanwhile, the T and B cells in the skin or skin homogenate of patients can promote the proliferation of pretibial fibroblasts. In conclusion, our results provide evidence that the local immune microenvironment of the skin may play an important role in the development of PTM.

Neurological Complications of Endocrine Emergencies.

PURPOSE OF REVIEW: Endocrine disorders are the result of insufficient or excessive hormonal production. The clinical course is long, and the manifestations are nonspecific due to the systemic effect of hormones across many organs and systems including the nervous system. This is a narrative review of the recent evidence of the diagnosis and treatment approach of these medical and neurological emergencies. RECENT FINDINGS: With the possible exception of diabetic ketoacidosis, hyperosmolar hyperglycemic state, and hypoglycemia, endocrinological emergencies are complex, uncommon yet life-threatening conditions with protean and often nonspecific early clinical signs. They frequently are the first manifestation of the endocrine derangement. The systemic effects of hormones extend to the nervous system and as such, these conditions can present with neurological complications manifested, in most cases, by a diffuse dysfunction of the brain in the form of encephalopathy, delirium, seizures, and coma; or specific and peculiar syndromes such as hemichorea, hemiballism, and epilepsia partialis continua. The severity of these conditions often necessitates management in the intensive care unit requiring substantial supportive care in addition to specific targeted therapy to correct the hormonal metabolic abnormalities while at the same time blocking hormonal activity, in cases of excessive function, or supplementing hormonal deficiencies. Endocrine emergencies and their neurological complications are infrequent. The major challenge for most is early recognition. Their morbidity and mortality are high and their diagnosis requires high index of suspicion. The neurological complication most often improves with the correction of the metabolic derangement and their acuity and severity require admission to the intensive care unit.

Sudden cardiac arrest associated with myxedema coma due to undiagnosed hypothyroidism: a case report.

BACKGROUND: Myxedema coma, which occurs due to hypothyroidism, is a rare and life-threatening condition. Some patients have hemodynamic dysfunction, which consequently leads to cardiac arrest. The rarity of this condition makes it difficult to determine the cause of cardiac arrest. It is important to diagnose myxedema coma based on clinical findings, including physical examination and laboratory parameters. We present a case of undiagnosed and untreated hypothyroidism that initially caused myxedema coma and then led to cardiac arrest. CASE PRESENTATION: A 56-year-old woman who had no medical history was transferred to our hospital for the management of return of spontaneous circulation due to sudden cardiac arrest. Findings of laboratory tests revealed that she had hypothyroidism. On physical examination, she was found to have a puffy face, thin eyebrows, and severe systemic non-pitting edema. Therefore, the patient was clinically diagnosed with myxedema coma, which was the cause of cardiac arrest. She was treated with thyroid hormone and hydrocortisone, resulting in improvement in her general condition, except for the neurological dysfunction. CONCLUSIONS: This case suggests that myxedema coma is caused by undiagnosed and untreated hypothyroidism, leading to sudden cardiac arrest. Our findings are useful in the differential diagnosis of hypothyroidism based on characteristic physical examination findings. Clinicians should be aware of the differential diagnosis of myxedema coma based on findings from physical examination and laboratory testing of thyroid function, and the treatment should be started immediately.

Congenital hypothyroidism presenting as myxedema coma in a teenager.

Congenital hypothyroidism is rare and is screened for in the United States during the newborn period in order to prevent a treatable cognitive delay. Myxedema coma is a complication due to severe hypothyroidism resulting from failure of homeostatic processes, causing altered mental status, generalized edema, and vital sign abnormalities. Treatment of myxedema coma consists of supportive care and hormone replacement. We describe a case of congenital hypothyroidism presenting as myxedema coma in an immigrant teenager.

Hypothyroidism.

Hypothyroidism is a common condition in which the thyroid gland provides insufficient amounts of thyroid hormone for the needs of peripheral tissues. The most common cause in adults is chronic lymphocytic thyroiditis (Hashimoto thyroiditis), but there are many other causes. Because most of the clinical features of hypothyroidism are nonspecific, the diagnosis requires laboratory testing. Serum thyroid-stimulating hormone (TSH) measurement is the best diagnostic test; an elevated TSH level almost always signals primary hypothyroidism. Serum free thyroxine levels may be below the reference range (overt hypothyroidism) or within the reference range (subclinical hypothyroidism). All patients with overt hypothyroidism should be treated, but those with subclinical hypothyroidism do not always benefit from treatment, especially elderly patients and those with baseline TSH levels below 10 mU/L. Oral L-thyroxine is the treatment of choice because of its well-demonstrated efficacy, safety, and ease of use. Therapy goals are symptom relief and maintenance of serum TSH levels within the reference range. Myxedema coma is a life-threatening form of decompensated hypothyroidism that must be treated with aggressive L-thyroxine replacement and other supportive measures in the inpatient setting.

[A case of elderly myxedema coma patient with end-stage renal failure].

CASE: 84-year-old woman had been diagnosed hypothyroidism before, but she did not take tablets of thyroid hormone prescribed. Anorexia and decreased consciousness level were discovered by workers of the facility. Her family doctor found thyroid function abnormality as a cause, and she was introduced to our hospital to treat because the whole body condition was bad. Her physical examination was observed that JCS was 3-10. Laboratory examination showed that TSH 562.81 µIU/mL, fT4 0.40 ng/mL, pH 7.33, and Na 124 mEq/L. It was not a contradictory finding with the diagnostic criteria of mucus edema coma. Blood and peritoneal dialysis is denied, although her data indicated eGFR 8-10 ml/min/1.73 m2. Her hospitalization was long-term, but exhibited finally good clinical course. After three months of hospitalization, she was discharged from the hospital. The problems were as follows. The clear guideline of myxedema coma with end-stage renal failure state and many complications was not found. She and her family did not hope dialysis under this condition.We treated a case of myxedema coma in a case of an elderly person with terminal renal failure who did not hope dialysis. In addition to myxedema coma as a rare disease, there are many basic diseases in the elderly. Although it was a difficult case, it is important to repeatedly confirm the explanation and the intention of the person and the family with regard to the selection of the treatment policy.

[Myxedema coma. Case report].

A rare case of hypothyroid coma developed in a patient with diagnosed primary hypothyroidism, complicated by the development of rhabdomyolysis and prolonged oppression of the respiratory center is presented. The predominance of previous cardiovascular pathology, marked oedema syndrome, and hypercholesterolemia in the clinical picture of the disease initially directed the diagnostics of cardiac pathology, which delayed the diagnosis of hypothyroidism. This publication aims to draw clinicians' attention to hypothyroid coma as a rare complication of hypothyroidism, which in this case was disguised as ischemic heart disease with the development of chronic heart failure.

Reversible bradycardia secondary to myxedema coma: case-report.

Myxedema coma occurs mostly in patients with long-standing untreated or undertreated hypothyroidism. Bradycardia is a well-known cardiac manifestation for myxedema coma; however, not all bradycardia with hypothyroidism are sinus bradycardia. Sick sinus syndrome is a group of arrhythmias caused by the malfunction of the natural pacemaker of the heart. Tachy-Brady syndrome is considered to be a type of sick sinus syndrome, where the heart alternates between tachycardia and bradycardia, and it is usually treated with pacemaker implantation along with rate slowing medical therapy. Here we report a case of an 83-year-old female who presented with myxedema coma and atrial fibrillation with tachycardia and intermittent slow ventricular response. We attempt to review the relationship between these two diseases and conclude that appropriate diagnosis of myxedema coma, may be beneficial in reducing the need for pacemaker implantation.

Myxedema ascites complicated by ischemic colitis.

Myxedema Ascites is a rare finding of primary hypothyroidism, thereby leading to delayed diagnosis. However, prompt treatment with levothyroxine leads to complete resolution of the condition. We present a rare case of myxedema ascites in an elderly female and highlight the importance of early diagnosis and management. We also present ischemic colitis in the same patient, which has not been reported thus far in literature as a complication of myxedema ascites.

Successful treatment of myxedema coma with a combination of levothyroxine and liothyronine.

Myxedema coma is a rare endocrine emergency resulting from the decompensation of severe hypothyroidism, which is associated with a high mortality rate. It is characterized by the deterioration of mental status, hypothermia, hypotension, hyponatremia, and hypoventilation. Early disease diagnosis and advancements in intensive supportive care have reduced the mortality rate. Besides intensive supportive care, appropriate management of the underlying thyroid hormone deficiency is essential. However, as the disease is rare and unrecognized, evidence-based treatment of myxedema has not yet been established in many countries. An 84-year-old Japanese man with a history of Hashimoto's thyroiditis was referred to our hospital. On arrival, conscious disturbance, hypothermia, hypotension, and hypoventilation were observed. He had discontinued thyroid hormone replacement therapy for a year. He was diagnosed with myxedema coma. Immediately, he received intensive supportive care and a combination therapy of 200 µg levothyroxine and 50 µg liothyronine until the fifth hospital day. Subsequently, monotherapy with levothyroxine was continued at a dose of 150 µg daily. The thyroid hormone level reached the normal range a few days later, and cardiovascular disease did not develop during hospitalization. This case demonstrated the efficacy of the combination of levothyroxine and liothyronine in treating myxedema coma.