ADRENAL FAILURE: WHEN ANTIPHOSPHOLIPID SYNDROME LEAVES SCARS

PV282 / #36 Case Report Poster Topic: AS03 - Antiphospholipid Syndrome Introduction We report a case of a 56-year-old male with a previous history of chronic mild thrombocytopenia, assumed to be a consequence of alcohol consumption (despite the absence of other alcoholic stigmas). He was admitted to the Internal Medicine Department with a 3-month history of extreme fatigue, anorexia, and weight loss (20%). Upon admission, he was hypotensive (100/54mmHg). Case Presentation With Investigation Blood tests revealed normocytic normochromic anemia (Hb 9.9 g/dL), thrombocytopenia (94 000x10^6/L), elevated activated partial thromboplastin time (76.4s, N 28-40), hyperkaliemia (7.11 mmol/L, N 3.5-5.2) (but normal sodium) and elevated inflammatory parameters (CRP 2.25 mg/dL, ESR 118 mm/h). An extensive workup study was conducted to exclude malignancy and infection. A PET-FDG showed intense uptake in both adrenal glands, with heterogeneity and areas of necrosis, especially in the right adrenal gland (Figure 1A). The endocrinology department was consulted, and hormonal assessments revealed a low serum cortisol (1.9 μg/dL; N 6.2-19.4) and a high adrenocorticotropic hormone (626.0pg/ml; N 7.2-63.3). PAI was assumed and intravenous hydrocortisone (200 mg/day) was started, with subsequent clinical (blood pressure, constitutional symptoms) and laboratory (blood cells count and inflammatory markers) improvement. The main causes for PAI, namely autoimmune Addison’s disease, tuberculosis and human immunodeficiency virus infection, were excluded. At this moment, the Rheumatology department was consulted. Further workup revealed a positive lupus anticoagulant antibody (2 times in 12 weeks apart), ANAs 1/1280 (homogeneous nuclear pattern), anti-dsDNA antibodies elevation (517 UI/mL) and a weekly positive antinucleosome antibody. MRI scans showed atrophy of the adrenal glands (Figure 1B and 1C). The patient was diagnosed with SLE and APS, and after PAI control, he was discharged under glucocorticoid tapering (prednisolone 15 mg/day and fludrocortisone 0.05 mg/day), warfarin and hydroxychloroquine 400mg/day. Later on, azathioprine was also started (100 mg/day) and the patient remained asymptomatic and with normal laboratory parameters. Figure 1 A: PET-FDG at diagnosis moment, showing intense uptake in both adrenal glands; B: abdominal MRI (T2) 3 months after the diagnosis, showing atrophy of both adrenal glands; C: abdominal MRI (T2) 6 months after the diagnosis, showing almost complete disappearance of both adrenal glands, comparing to the previous MRI. Literature Review Antiphospholipid syndrome (APS) is a multisystemic autoimmune disorder characterized by recurrent arterial, venous and/or microvascular thrombotic events. The disease rarely affects the endocrine system, especially at presentation. The involvement of the adrenal gland, although rare, can be severe. Possible mechanisms behind adrenal manifestations include multiple microthrombosis of the suprarenal vein leading to infarction and adrenal hemorrhage, atrophy and finally failure (primary adrenal insufficiency [PAI]).[1][2] Discussion This case illustrates one of the rarest and still most severe consequences of APS. Patients with APS and adrenal hemorrhage, typically have bilateral involvement and develop adrenal insufficiency, just like our patient. [3] The disease can be fatal, thus early diagnosis and treatment as well as a close follow-up and multidisciplinary approach is needed to improve the prognosis of this rare disease. References: [1.] Hochberg MC. Chapter 135: Clinical features of systemic lupus erythematosus. In: Rheumatology 8th Ed. Elsevier;2022:P1113. [2.] Bouki K. Hormones (Athens) 2023;22(3): 521-31. [3.] Meade-Aguilar JA. Clin Immunol 2024;260:109906.

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