Graves' Disease, Hypoparathyroidism, Systemic Lupus Erythematosus, Alopecia, and Angioedema: Autoimmune Polyglandular Syndrome Variant or Coincidence?

Abstract

Data on coexisting Graves' disease (GD), hypoparathyroidism, and systemic lupus erythematosus (SLE) are limited. The thyroid and parathyroid glands may be extra sensitive to irradiation damage in an underlying autoimmune condition. A 34-year-old black woman presented with tetanic-like cramps, easy skin bruising, fatigue, weight gain, nocturia and back pain. She was previously diagnosed with GD in 2001 and underwent radioiodine therapy (RAI) in 9/01 using 6 mCi. PostRAI (November 2001) she developed hypocalcemia and hypothyroidism (2/02). In 2007, SLE was diagnosed. In October 2009, s-calcium and PTH were still low at 7.1 mg/dl and 9 pg/mL, respectively, although the patient denied symptoms on vitamin D and calcium supplementation. To identify possible autoimmune damage of the parathyroids, we evaluated the presence of activating antibodies to the CaSR and also analyzed the DNA sequence of all 6 translated exons and flanking intronic sequences of her CaSR gene for a functionally significant CaSR mutation but neither was positive. The initial autoimmune damage to her thyroid and possibly parathyroid glands followed by irradiation of them seems to have contributed to her developing both hypoparathyroidism (11/01) and hypothyroidism (2002). The patient could potentially have had parathyroid autoantibodies in 2001 that disappeared by 2009 when she was tested for them. We consider that the multiple autoimmune conditions developed over the past decade of her life with the concurrent irradiation contributing to her brittle hypoparathyroidism. Select patients with GD and perhaps parathyroid autoantibodies with a slowly developing destructive impact on the parathyroid glands may then develop overt hyoparathyroidism with rather low dose RAI ablation. This patient adds to the evolving spectrum of polyglandular syndrome variants.Here, we report a patient presenting with an interesting sequential combination of Graves' disease (GD), hypoparathyroidism, alopecia, and systemic lupus erythematosus (SLE). Hypocalcemia

Morphea and Lichen Sclerosus in a Patient with Hyporthyroidism

“…Melcescu et al tried to estimate the coexistence of Graves' disease, hypoparathyroidism and connective tissue diseases, mainly SLE. However, study results did not indicate such a relationship 14 . Allam and Ghozzi 15 and Berlger-Czop et al 16 presented cases of scleromyxedema associated with hypothyroidism.…”

The various faces of autoimmune endocrinopathies: Non-tumoral hypergastrinemia in a patient with lymphocytic colitis and chronic autoimmune gastritis

“…AG can occur concomitantly with lymphocytic colitis (LC) (Bohr, 1998; Bohr et al, 1996a; Cindoruk et al, 2002; Koskela et al, 2011; Leung et al, 2009; Pardi et al, 2002a; Remaine, 2000; Rostami et al, 2000; Triantafillidis et al, 2003). In the setting of polyglandular syndromes, other autoimmune diseases including type 1(A) diabetes mellitus, Graves' disease, Hashimoto's thyroiditis, Addison's disease, hypoparathyroidism, lymphocytic hypophysitis, premature ovarian failure, hypogonadism, autoimmune hepatitis, alopecia areata, vitiligo, Sjogren's syndrome, multiple sclerosis, celiac disease, and Crohn's disease may be present or evolving (Betterle et al, 2002; Brown, 2009; Dittmar and Kahaly, 2010; Kahaly, 2009; Koch et al, 2000; Manetti et al, 2007; Melcescu et al, 2010; Michels and Gottlieb, 2010; Mitchell and Pearce, 2012; Triantafillidis et al, 2003; Vrijman et al, 2012). Gastrin stimulates enterochromaffin-like cells which increase histamine secretion.…”

Case report: a 10-year-old girl with primary hypoparathyroidism and systemic lupus erythematosus

“…An autoimmune aetiology of the hypoparathyroidism in our patient was considered after her hypocalcaemia improved with glucocorticoid treatment. Autoimmune hypoparathyroidism is a major component of autoimmune polyglandular syndrome type 1 (APS1) along with chronic mucocutaneous candidiasis and primary adrenal insufficiency (Addison's disease) [8][9][10][11][12]. APS1 commonly manifests in childhood and our patient presents with one of the major components.…”

“…Therefore, monitoring for chronic mucocutaneous candidiasis and Addison's disease would seem appropriate in the future. Considering an alternative APS diagnosis, there have been occasional reports of hypoparathyroidism occurring in other APS types [10,11]. The patient does not conform to an APS2 (Addison's disease, thyroid disease and/or type 1 diabetes) or APS3 (thyroid disease plus one other non-Addison's disease autoimmunity) diagnosis.…”