Journal List > J Korean Rheum Assoc > v.15(1) > 1003653

Ha, Koh, Kim, Kim, Ahn, and Cha: A Case of Primary Adrenal Insufficiency Presenting as the Initial Clinical Manifestation of Primary Antiphospholipid Antibody Syndrome

Abstract

Primary adrenal insufficiency is one of the rare clinical manifestations of antiphospholipid antibody syndrome. The pathogenesis is mainly ascribed to the hemorrhagic infarction secondary to thrombosis. We describe a 19-year-old man who developed symptoms of adrenal insufficiency and then was diagnosed of primary antiphospholipid antibody syndrome, which is the first reported case in adult patients in Korea. The possibility of adrenal failure should be considered in the management of antiphospholipid antibody syndrome and also, when primary adrenal insufficiency is accompanied by no definite etiology, the antiphospholipid antibody syndrome should be considered as a possible etiology.

References

1. Asherson RA, Hughes GR. Recurrent deep vein thrombosis and Addison's disease in “primary” antiphospholipid syndrome. J Rheumatol. 1989; 16:378–80.
2. Satta MA, Corsello SM, Della Casa S, Rota CA, Pirozzi B, Colasanti S, et al. Adrenal insufficiency as the first clinical manifestation of the primary antiphospholipid antibody syndrome. Clin Endocrinol (Oxf). 2000; 52:123–6.
crossref
3. Presotto F, Fornasini F, Betterle C, Federspil G, Rossato M. Acute adrenal failure as the heralding symptom of primary antiphospholipid syndrome: report of a case and review of the literature. Eur J Endocrinol. 2005; 153:507–14.
crossref
4. 김동훈, 이수현, 김현주, 유한욱, 윤종현. 소아에서 부 신출혈을 동반한 일차성 항인지질항체증후군. 대한방 사선의학회지. 1999; 41:1025–7.
5. 신선혜, 공옥녀, 유석동, 손석만, 김인주, 김용기 등. 전신성 홍반성 루푸스 및 항인지질 항체 증후군에 동 반된 부신기능저하증 1예. 대한내분비학회지. 2004; 19:542–5.
6. Wilson WA, Gharavi AE, Koike T, Lockshin MD, Branch DW, Piette JC, et al. “International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop.” Arthritis Rheum. 1999; 42:1309–11.
7. Levine JS, Branch DW, Rauch J. The antiphospholipid syndrome. N Engl J Med. 2002; 346:752–63.
crossref
8. Espinosa G, Cervera R, Font J, Asherson RA. Adrenal involvement in the antiphospholipid syndrome. Lupus. 2003; 12:569–72.
crossref
9. Gonzalez G, Gutierrez M, Ortiz M, Tellez R, Figueroa F, Jacobelli S. Association of primary antiphospholipid syndrome withprimary adrenal insufficiency. J Rheumatol. 1996; 23:1286–7.
10. Asherson RA, Hughes GR. Hypoadrenalism, Addison's disease and antiphospholipid antibodies. J Rheumatol. 1991; 18:1–3.
11. Arnason JA, Graziano FM. Adrenal insufficiency in the antiphospholipid antibody syndrome. Semin Arthritis Rheum. 1995; 25:109–16.
12. Caron P, Chabannier MH, Cambus JP, Fortenfant F, Otal P, Suc JM. Definitive adrenal insufficiency due to bilateral adrenal hemorrhage and primary antiphospholipid syndrome. J Clin Endocrinol Metab. 1998; 83:1437–9.
crossref
13. Walz BA, Silver RD. Addison's disease in “primary” antiphospholipid syndrome. J Rheumatol. 1990; 17:115–6.

Fig. 1.
Black pigmentation of skin, lip, buccal mucosa, gingiva and palmar crease which is a characteristic of primary adrenal insufficiency.
jkra-15-87f1.tif
Fig. 2.
Both adrenal glands show swelling and low attenuation, which suggests adrenal infarction (A). After 6 months, both adrenal glands are atrophic (B), which suggests progressive destruction of adrenal glands as a sequale of adrenal infarction.
jkra-15-87f2.tif
TOOLS
Similar articles