Transcript Immunoglobulin A Nephropathy as a Systemic Underlying

Immunoglobulin A Nephropathy as a
Systemic Underlying Cause of Bilateral
Anterior Scleritis
Aruoriwo Oboh-Weilke, MD
Florian A. Weilke, MD
InnovisHealth
Fargo,ND
PURPOSE
We report a case of a patient who
presented with a bilateral anterior scleritis
which occured 5 months apart. The patient
had been diagnosed with biopsy proven
Immunoglobulin A (IGA) nephropathy 4
years prior to her ocular complication of
scleritis.
INTRODUCTION
 Scleritis is an ocular inflammatory condition
caused by an immune-mediated vasculitis which
results in inflammation and destruction of the
sclera 1.
 It causes significant pain and may lead to
structural changes of the globe in association
with visual morbidity 1.
 Scleritis can be divided into four clinical types:
 Nodular anterior, diffuse anterior, necrotizing
anterior and posterior scleritis 8
INTRODUCTION
 IgA nephropathy is the most common
biopsy-proven pattern of glomerulonephritis
worldwide 4. Both clinical and histologic
factors, have been suggested to impact on
prognosis 4. The clinical picture of this renal
disease includes, proteinuria, acute kidney
injury, and the nephrotic syndrome 4.
CASE REPORT
 A 31 year old caucasian female was referred for
an ophthalmology consultation by her internist.
The patient stated she had noticed redness of her
left eye for one week. This was accompanied by
boring left ocular pain. The patient had seen an
optometrist who started her on topical steroids.
She reported worsening of the symptoms on this
regimen. She then saw her internist who started
her on systemic steroids and promptly referred her
to the eye clinic.
CASE REPORT
 On review of systems, the patient admitted to blurry
vision, pain on eye motion and blood in her urine. On
further questioning about her medical problems, she
stated that she had been diagnosed and treated for
IgA nephropathy for 4 years. She previously had
proteinuria and microscopic hematuria as
manifestations of her renal condition and had been
followed by her nephrologist. Her diagnosis of IgA
nephropathy had been confirmed by a renal biopsy.
Her last follow-up with her nephrologist was 1 year
prior to this presentation and she was noted to have
a stable renal status at that time.
CASE REPORT
 On physical examination, the patient’s VA was OD
20/25 and OS 20/40
 EOM were full, however, there was pain on
motion.
 Pupils and IOPs were normal.
 Slit Lamp Exam revealed diffusely inflammed
temporal conjunctival and underlying scleral
vessels with scleral and episcleral edema. There
was minimal blanching with the application of
topical phenylephrine.
 Dilated fundus exam was normal and did not
reveal any choroidal folds
RESULTS
 The patient was continued on the oral steroids and
tapered according to her symptoms. Her
symptoms resolved after 2 weeks.
 A work-up for more common causes of scleritis
was undertaken.
 CBC, ESR, RF, ANA, c-ANCA, p-ANCA, uric acid
and ACE levels were all negative.
 A 24 hour urine collection revealed proteinuria. An
appointment was made for the patient to see her
nephrologist, to evaluate whether there was any
worsening of her renal status.
RESULTS
 5 months after this episode, the patient
presented with similar symptoms and signs
in the right eye. A diagnosis of diffuse
anterior scleritis was made and she was
initially treated this time with NSAIDS. She
responded well and showed resolution after
1 week.
OD few days after presentation
DISCUSSION
 Scleritis is an ocular inflammatory condition that is
immune-mediated. It is usually associated with an
underlying systemic immunological disease in
about 50% of the cases 2. Several well known
etiologies are Connective tissue diseases such as
rheumatoid arthritis, ankylosing spondylitis,
systemic lupus erythematosus, and with
vasculitidies such as Wegner's granulomatosis
and polyarteritis nodosa 1. It can also be
associated with infectious disease such as
syphilis, tuberculosis and herpes zoster 1. There
have however, only been a few reports in the
literature citing IgA nephropathy as an underlying
systemic cause .
DISCUSSION
 IgA nephropathy is a renal disease that may
progress over a period of 20 years to chronic
kidney disease, and then to end-stage renal
disease requiring renal replacement therapy 5.
 Studies have shown that the use of corticosteroids
can reduce proteinuria and prevent progression to
end-stage renal disease 7.
 IgA nephropathy is generally considered to be an
immune-complex-mediated glomerulonephritis 6.
DISCUSSION

3Nomoto
et al performed a follow-up study of 113 patients
all known to have various types of primary glomerular
diseases. The patients were followed for 33 months to
determine the clinical spectrum of primary
glomerulonephritis. The study revealed six patients
exhibited scleritis. All of these six patients with scleritis
were noted to have IgA nephropathy. None of the other
patients diagnosed with other forms of glomerulonephritis
showed scleritis during the study period. This suggests that
a similar autoimmune mechanism may be responsible for
the manifestation of IgA nephropathy and the development
of scleritis.
CONCLUSION
 While evaluating a patient with scleritis, it is
important to perform a complete review of systems
and to pay close attention to the patient’s past
medical history.
 This case report draws attention to another
underlying cause of a well known ocular entity. A
work-up which includes evaluation of the renal
function should be entertained in patients
presenting with scleritis.
REFERENCES
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1
External Disease and Cornea. Basic and Clinical Science
Course 1999-2000; 219-224
2 Scleritis and IgA nephropathy.
Arch Intern Med. 1980 Jun;140(6):783-5.
3 Scleritis in IgA nephropathy: a case report.
Zhonghua Yi Xue Za Zhi (Taipei). 1995 Oct;56(4):279-82.
4 Clinicopathologic correlation in IgA nephropathy. Semin
Nephrol. 2008 Jan;28(1):10-7.
5 Natural History of Primary IgA Nephropathy. Semin
Nephrol. 2008 Jan;28(1):4-9.
REFERENCES
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6
Pathogenesis of IgA nephropathy. Contrib Nephrol.
2007;157:1-7.
 7 Treatment of IgA nephropathy: corticosteroids,
tonsillectomy, and mycophenolate mofetil. Contrib Nephrol.
2007;157:37-43.
 8 Atlas of Clinical Ophthalmology. Mosby,2000; 5.22-5.23