Vol 43 # 2 June 2011 - Kma.org.kw

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KUWAIT MEDICAL JOURNAL
June 2011
Case Report
Autoimmune Adrenal Insufficiency Antedates the
Diagnosis of SLE, Does It Really Matter
Ebtihal Aljamaan
Department of Internal Medicine, Mubarak Alkabeer Hospital, Kuwait
Kuwait Medical Journal 2011; 43 (2): 136-138
ABSTRACT
Coincidence of primary adrenal insufficiency and systemic
lupus erythematosus (SLE) is a rare occurrence. Several
pathological processes have been suggested to explain the
association but variability of the reported cases suggests
a multi-factorial etiology, in which tissues and cells are
damaged by pathogenic autoantibodies and immune
complexes. Association of anticardiolipin antibodies
with thrombosis is well-established. In clinical settings,
the symptoms of adrenal insufficiency are masked by
multi-systemic nature of SLE and manifestations vary
according to tissues affected. We report a case of a
young girl, where her autoimmune adrenal insufficiency
antedated the diagnosis of SLE by two years with absence
of antiphospholipid antibodies.
KEY WORDS: anticardiolipin, antiphospholipid, autoimmune adrenalinsufficiency, SLE
INTRODUCTION
The clinical spectrum of Addison’s disease has
changed dramatically over the last 30 years, and
autoimmunity is now the most common cause of
primary adrenal insufficiency [1] . Adrenal insufficiency
is characterized by weight loss, fatigue, low blood
pressure, and sometimes darkening of the skin .This is
a consequence of hypocortisolism, and in some cases
hypoaldosteronism [1,2] . Autoimmune mechanism is
one of the etiologies of hypocortisolism. About 50-
60% of patients will develop additional autoimmune
endocrinopathies during their life as manifestations
of polyglandular syndromes type 1 and 2 [3,4] . The
treatment is usually hormonal replacement with
glucocorticoids and mineralocorticoid.
Systemic lupus erythematosus (SLE) is occasionally
accompanied by autoimmune disorders of endocrine
glands, most commonly the thyroid, but rarely the
adrenal glands [3] . Adrenal failure has been described
in adults with SLE [5,6] , and in three children [7] and in
several adults with antiphospholipid syndrome [3, 4,8] , a
condition that occurs most frequently in patients with
SLE. However, our case is very unique in describing the
co-existence of autoimmune adrenal insufficiency prior
to the diagnosis of SLE and in absence of antiphosolipid
antibodies. To the best of our knowledge this has not
been reported earlier in the literature.
CASE REPORT
A 20-year-old Kuwaiti girl reported to the
outpatient department of internal medicine with the
chief complaint of black spots over the tongue and
the gingiva (Fig. 1) that kept increasing in color over
a 12-month period. She also reported lethargy and
muscle weakness of a few months duration. There was
no history of skin rash, joint pain or connective tissue
disease symptoms.
Her past medical history revealed pulmonary
tuberculosis at age of six years, which was treated
successfully with multiple anti-tuberculous drugs for
an appropriate length of time. No family history of
connective tissue disease or endocrinopathy could be
elicited.
The physical examination revealed a thin,
averagely built young lady with a BMI of 21. Her
BP was 90/60 mmHg with no postural hypotension.
The skin was hyperpigmented over creases of both
hands (Fig. 2), old scars over the feet (Fig. 3) and
hyperpigmented spots over the tongue and gingiva.
The rest of systemic examination was normal.
The patient was admitted to the internal medicine
ward and investigated thoroughly. Her CBC, thyroid
function, renal and liver profiles were within
normal limits. Her serum electrolytes revealed mild
hyponatremia of 121 mmol/l and K + of 4.4 mmol/l.
Address correspondence to:
Dr. Ebtihal Aljamaan, MD, FRCPC, Senior Registrar, Department of Internal Medicine, Mubarak Alkabeer Hospital, Kuwait. Tel: +965-
99684148,E-mail: Bahali519@yahoo.com