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      An unusual cause of adrenal insufficiency and bilateral adrenal masses

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          Summary

          Primary adrenal insufficiency secondary to syphilis is extremely rare, with only five cases being reported in the literature. We report a case of adrenal insufficiency as a manifestation of Treponema pallidum infection (tertiary syphilis). A 69-year-old, previously fit and well Caucasian male was found to have adrenal insufficiency after being admitted with weight loss, anorexia and postural dizziness resulting in a fall. Biochemical testing showed hyponatraemia, hyperkalaemia, and an inadequate response to Synacthen testing, with a peak cortisol level of 302 nmol/L after administration of 250 µg Synacthen. Abdominal imaging revealed bilateral adrenal hyperplasia with inguinal and retroperitoneal lymphadenopathy. He was started on hydrocortisone replacement; however, it was not until he re-attended ophthalmology with a red eye and visual loss 1 month later, that further work-up revealed the diagnosis of tertiary syphilis. Following a course of penicillin, repeat imaging 5 months later showed resolution of the abnormal radiological appearances. However, adrenal function has not recovered and 3 years following initial presentation, the patient remains on both glucocorticoid and mineralocorticoid replacement. In conclusion, this case highlights the importance of considering syphilis as a potential differential diagnosis in patients presenting with adrenal insufficiency and bilateral adrenal masses, given the recent re-emergence of this condition. The relative ease of treating infectious causes of adrenal lesions makes accurate and timely diagnosis crucial.

          Learning points:
          • Infectious causes, including syphilis, should be excluded before considering adrenalectomy or biopsy for any patient presenting with an adrenal mass.

          • It is important to perform a full infection screen including tests for human immunodeficiency virus, other blood-borne viruses and concurrent sexually transmitted diseases in patients presenting with bilateral adrenal hyperplasia with primary adrenal insufficiency.

          • Awareness of syphilis as a potential differential diagnosis is important, as it not only has a wide range of clinical presentations, but its prevalence has been increasing in recent times.

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          Most cited references6

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          Syphilis: Re-emergence of an old foe

          Lola Stamm (2016)
          Syphilis is caused by infection with Treponema pallidum subsp. pallidum, a not-yet-cultivable spiral-shaped bacterium that is usually transmitted by sexual contact with an infected partner or by an infected pregnant woman to her fetus. There is no vaccine to prevent syphilis. Diagnosis and treatment of infected individuals and their contacts is key to syphilis control programs that also include sex education and promotion of condom use to prevent infection. Untreated syphilis can progress through four stages: primary (chancre, regional lymphadenopathy), secondary (disseminated skin eruptions, generalized lymphadenopathy), latent (decreased re-occurrence of secondary stage manifestations, absence of symptoms), and tertiary (gummas, cardiovascular syphilis and late neurological symptoms). The primary and secondary stages are the most infectious. WHO estimates that each year 11 million new cases of syphilis occur globally among adults aged 15-49 years. Syphilis has re-emerged in several regions including North America, Western Europe, China and Australia. Host-associated factors that drive the re-emergence and spread of syphilis include high-risk sexual activity, migration and travel, and economic and social changes that limit access to health care. Early, uncomplicated syphilis is curable with a single intramuscular injection of benzathine penicillin G (BPG), the first line drug for all stages of syphilis. Emergence of macrolide-resistant T. pallidum has essentially precluded the empirical use of azithromycin as a second-line drug for treatment of syphilis. Virulence attributes of T. pallidum are poorly understood. Genomic and proteomic studies have provided some new information concerning how this spirochete may evade host defense mechanisms to persist for long periods in the host.
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            Gummatous syphilis of the adrenal gland.

            A 55-year-old man with no relevant history was analyzed for weight loss, night sweats, and left upper quadrant pain. An abdominal ultrasound and CT scan were performed, revealing a lobulated rim-enhancing mass in the left adrenal gland. Further analysis by an F-FDG PET/CT scan demonstrated high uptake in the periphery of the adrenal lesion with central photopenia. Because a primary malignancy was suspected, an adrenalectomy was performed. Histopathology, however, revealed a necrotizing granulomatous infection. Serum was tested positive for syphilis and Treponema pallidum infection. Results of additional HIV tests were negative. The adrenal tumor proved to be an expression of gummatous syphilis.
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              Addison’s disease associated with syphilis and fracture of the first lumber vertebra

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                Author and article information

                Journal
                Endocrinol Diabetes Metab Case Rep
                Endocrinol Diabetes Metab Case Rep
                EDM
                Endocrinology, Diabetes & Metabolism Case Reports
                Bioscientifica Ltd (Bristol )
                2052-0573
                06 July 2018
                2018
                : 2018
                : 18-0030
                Affiliations
                [1 ]Department of Endocrinology , Newcastle Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK
                [2 ]Institute of Genetic Medicine , Newcastle University, Newcastle Upon Tyne, UK
                [3 ]Departments of Ophthalmology , Newcastle Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK
                [4 ]Departments of Infectious Disease and Tropical Medicine , Newcastle Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK
                [5 ]Radiology , Newcastle Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK
                Author notes
                Correspondence should be addressed to S A Tee Email tsun83@ 123456gmail.com
                Article
                EDM180030
                10.1530/EDM-18-0030
                6038009
                e1f0b4d6-7e38-4c1e-9c2c-73cc8e2f257d
                © 2018 The authors

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.

                History
                : 25 April 2018
                : 08 June 2018
                Categories
                Unique/Unexpected Symptoms or Presentations of a Disease

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