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

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Autoimmune Adrenal Insufficiency Antedates the Diagnosis of SLE, Does It Really Matter
June 2011
insufficiency, as proved by hyper-pigmentation,
hypotension, extreme fatigue and low serum cortisol
level (203 nmol/l) which failed to rise one hour after
intravenous injection of 0.25 mg co-syntropin two years
prior to her presentation with features of SLE (fulfilling
the ARC criteria) that manifested as malar rash, discoid
rash, photosensitivity, painless oral ulcers, arthritis,
hemolytic anemia and renal involvement.
As SLE is a multi system disorder, many of the
clinical features are common with adrenal insufficiency,
Among 20 reported cases with positive anticardiolipin
antibodies with adrenal failure, only four cases
developed features of SLE [5,9] .
In our case, the autoimmune adrenal insufficiency
antedated the clinical diagnosis of SLE. This could be
due to the fact that, the immune destruction of the
adrenals started earlier than other tissue in the body
or long term steroid therapy for autoimmune adrenal
insufficiency may have masked the clinical presentation
of SLE. Another explanation may be that many of the
non-specific clinical features of SLE are common with
adrenal insufficiency. This per se can create a great
confusion in clinical diagnosis in the beginning, but
with close and careful follow-up, definite diagnosis can
be made easily, as other symptoms appear with time.
This is what happened with our patient. Moreover, it
is very unlikely that these two clinical conditions are
not related pathologically and only co-existed in the
same patient.
The etiology of hypoadrenalism in SLE is
unknown, but proposed mechanisms may be adrenal
vascular thrombosis and infarction, hemorrhage due
to abnormal coagulation, vasculitis and a direct organ
specific autoimmune insult [2,4,6,8] . Early reports of the
association suggested the presence of antiphospholipid
antibodies (APLA) in these patients, and hence the
adrenal failure has been related to their presence [3,4,6]
but APLA were not found in our patient despite being
diagnosed with SLE.
In SLE, adrenal involvement may be the first
clinical manifestation of this syndrome, whereas a few
patients may have history of adrenal insufficiency in
the past [2,8] . In some cases of adrenal damage due to
hemorrhage, incomplete destruction of adrenal cortex
may leave enough residual function to prevent acute
adrenal crisis, with later development of chronic
adrenal insufficiency [2] .
The association between SLE and adrenal
insufficiency has been described earlier in the
literature. In all reported cases, the diagnosis of
adrenal insufficiency was made after the diagnosis
of SLE [5,6] and in some cases, both conditions have
been described simultaneously [6,9-11] . We describe for
the first time in the literature, autoimmune adrenal
insufficiency antedating the diagnosis of SLE with
negative APLA. This proves the hypothesis of the
presence of other pathological mechanisms rather than
APLA, in this association.
CONCLUSION
This case suggests the need for increased suspicion
of SLE in patient with adrenal insufficiency and
systemic complaints. This clinical suspicion should be
high when patient with adrenal insufficiency present
with arthalagia, skin rash, anemia and raised ESR. To
confirm the diagnosis, ANA and anti-ds DNA should
be done, but APLA may not be found in these patients.
Therefore, we advise careful follow up of such patients
since they may evolve into connective tissue disease.
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