ACTA ENDOCRINOLOGICA (BUC)

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Introduction. The polyglandular autoimmune syndrome (PAS) type III is a rare condition defined as the coexistence of autoimmune thyroid disorder with other endocrine autoimmune diseases, including type 1 diabetes, without adrenal dysfunction. PAS may associate with other non-endocrine autoimmune diseases, overlapping with the multiple autoimmune syndromes (MAS). We present a case of PAS III/ MAS type 3, including autoimmune thyroiditis, autoimmune diabetes, vitiligo, lupus erythematosus, associated with adult-onset atopic dermatitis, a combination not reported previously. Case report. A 40 years old woman, registered as nurse working in dialysis unit, previously diagnosed with vitiligo, euthyroid autoimmune thyroiditis and disseminated granuloma annulare, with personal and familial history of atopic disorders, presented in our clinic for disseminated eczematous and lichenoid cutaneous rashes. She was tested positive for antinuclear, anti-double stranded DNA and anti-histone antibodies, with inflammatory syndrome and marginal lymphopenia and she was diagnosed with systemic lupus erythematosus (SLE). Subsequently, moderate hyperglycemia, positive anti-glutamic acid decarboxylase antibodies and low C-peptide level prompted the diagnosis of autoimmune diabetes. Recurrent flexural eczematous rashes, with negative epicutaneous tests but positive specific IgE tests for common allergens fulfilled the clinical criteria for the diagnosis of atopic dermatitis. The clinical, immunological and glycemic status were controlled with low doses of oral prednisone (<0.5 mg/kg), methotrexate (10mg/week), antimalarials, metformin, emollients and photoprotection. After changing her workplace, the immunosuppressive treatment could be discontinued, and the patient maintained normal immunological and biochemical profile at 6 months follow-up. This case brings a unique perspective on the evolution, associations spectrum and the management challenges of endocrine polyautoimmunity associated with atopic diathesis.

At the end of 2019, a novel coronavirus was identified as the cause of a cluster of pneumonia in China and it spread quickly to other countries. Although Covid-19 causes severe pneumonia, it is known that it can be associated with different diseases and prognosis of disease can be due to many of disorders such as hyponatremia. A 65-year-old female patient with sarcoidosis, cronic obstructive lung disease, hypertension and congestive heart failure presented to the emergency department with shortness of breath and fever. Oropharyngeal swab for Covid-19 PCR test was positive. After the initiation of treatment, the patient developed hyponatremia. This case is remarkable because there is no reported case of Covid-19 and inappropriate ADH syndrome coexistence and it demonstrates that there may be a correlation between Covid-19 infection prognosis and hyponatremia.

Paget’s disease is a disorder of aging bone which occurs in the setting of accelarated bone remodelling. In the presented case we discuss the difficulties in the diagnosis of Paget’s disease in a 77 year old patient with coexisting endometrium carcinoma. The patient was initially diagnosed with metastatic bone disease due to endometrium adenocarcinoma when she was admitted to oncology clinic with pelvic pain. Bone scintigraphy with Tc99 and (18) F fluorodeoxyglucose positron emission tomography/CT revealed an increased uptake on the bone lesions which were reported as metastatic bone involvement. Although the (18) F-FDG uptake was much higher than the levels that would generally be anticipated in a case with Paget’s disease, high levels of bone turnover markers indicated further evaluation in the differential diagnosis and the definitive diagnosis of Paget’s disease was established with the pathological evaluation of bone biopsy.

The study comments unusual associations between thyroid and cutaneous autoimmunity: Graves-Basedow disease (GBD), vitiligo and alopecia areata (AA) starting from two cases. In the first case, a woman with systemic lupus erythematosus (SLE), data were recorded from 38 to 49 years as follows: vitiligo (at 38 ys), alopecia areata (4-6 months afterwards), SLE (after 2 ys) and then GBD (after 8 ys). After 3 years, hyperthyroidism has spontaneously vanished, but vitiligo, AA, leucothrichia, SLE, goiter and ophthalmopathy persisted. In the second case, a man, data were recorded from 26 to 70 years and the disease was associated with psoriasis. The sequence of diseases was: vitiligo (at 26 ys), AA and GBD (after 8 ys), followed by iatrogenic 131I hypothyroidism, and psoriasis (after 33 ys). Vitiligo and AA have spontaneously vanished before GBD began. These multiple immune syndrome associations bring up the question: ?Are these diseases multiple associations or a unique immune disease?? A possible point of view, related to immune network, suggests that these multiple associations represent in fact only one process, therefore they represent not many diseases, but different expressions in time (sequence) and space (organ-lesion) of the disease of the immune network.

Thyroid isthmus agenesia (TIA) which is characterized by the absence of isthmus is one of these anomalies and its etiology is unclear. Marine Lenhart Syndrome (MLS), on the other hand, is defined as the coexistence of Graves disease and hyperactive nodules,although the diagnostic criteria are not clearly defined. Fifty five years old male patient with no concomitant disease had been diagnosed with MLS and accompanying subclinical hyperthyroidy for two years. He had dysphagia for a year. Neck ultrasound revealead retrosternal goiter with multiple hypoechoic nodules with the largest size of 33x30 mm. He had normal FT3 and FT4 levels with a decreased level of TSH. Scintigraphy revealed diffuse thyroid uptake with a hyperactive nodule of 16x11 mm on right upper lobe. Due to compressive sypmtoms and MLS he underwent surgery and diagnosed with thyroid isthmus agenesia peroperatively. MLS and TIA are rare thyroid pathologies and their etiopathogenesis has not been clarified yet. This case is thought to be the first case in which MLS and TIA coexistence was reported. Even though it is a rare disorder,having a basic knowledge about TIA can be an important step in establishing a treatment plan and avoiding possible complications. Especially in management of MLS, when surgery is considered the physician should be careful for presence of a TIA.

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Objective: Thyroid development was studied mainly on animal models and data in humans are scarce. Knowing that there are interspecies differences and a specific timing of thyroid development we aimed to reveal intimate aspects of the human foetal morphology and function.\r\nMaterial and method: Thyroids from 20 aborted fetuses of different gestational ages (8-16 weeks) were embedded in paraffin, sectioned, coloured and immunohistochemically processed using the Avidin-Biotin Complex&#8211;Peroxidase (ABC) method with a pannel of antibodies aimed to reveal the secretory activity (antithyroglobulin monoclonal and polyclonal and anti TITF1 antibodies), the differentiation of intermediate filaments (anti AE1/AE3, anti CK7 and antivimentin monoclonal antibodies), of C cells (anti CEA monoclonal antibodies) and of the thyroid vascular net (anti CD34 monoclonal antibodies).\r\nResults: Thyroglobulin expression was present in thyrocytes cytoplasm even before follicles are formed (8-10 weeks); after 12 weeks appeared also within the colloid and expression increased after 14 weeks showing a luminal pattern of distribution similar to the mature thyroid. TITF1 was present in the thyrocytes nuclei of all groups, weak till 14 weeks and intense thereafter and in the C cells nuclei. C cells appeared after 10 weeks and expressed CEA, vimentin and CK7. Immunostaining for keratins (AE1/AE3, CK7) was rarely positive in cordonal thyrocytes, but was present in follicular thyrocytes and increased with gestational age. Some thyrocytes of all groups were vimentin positive and showed coexpression with cytokeratins. CD 34 expression indicated an early vascular differentiation being present in isolated endothelial cells before 10 weeks and structured capillaries after 10 weeks of gestational age.\r\nConclusions: Immunohistochemistry proved to be a useful tool in our attempt to shed light on human thyroid development which would permit a better pathogenic understanding of thyroid dysgenesias and thyroid neoplasms.

Objective. In our study, we aimed to investigate the levels of irisin, nesfatin-1 and the relationship between levels of these relatively new molecules with cardiometabolic risk markers; carotid intima-media thickness (CIMT), epicardial adipose tissue (EAT) thickness in patients with nonfunctional adrenal incidentaloma (NFAI). Materials and Methods. Patients with NFAI (n=59) and age, sex and body mass index-matched healthy control subjects (n=59) were enrolled in this study. Serum glucose, insulin, C-reactive protein (CRP), lipid, irisin and nesfatin-1 levels and echocardiographic CIMT and EAT thickness measurements were performed in patients and controls. Results. The irisin level was 17.58 ± 4.38 pg/mL in the NFAI group, significantly higher (p<0.001) than 14.03 ± 4.03 pg/mL in the control group. Nesfatin-1 level was significantly lower in the NFAI group 194.98 ± 119.15 pg/ mL ((p < 0.001)) versus 303.48 ± 200.78 pg/mL in the control group. A positive correlation was found between irisin and nesfatin-1 levels and CIMT and EAT thickness in the NFAI group. Conclusions. In our study, we found that irisin level was higher and nesfatin-1 level was lower in patients with NFAI, and both irisin and nesfatin-1 levels were associated with CIMT and EAT thickness in NFAI patients.