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EyeNet Magazine
>> Morning
Rounds
Morning Rounds
The Sinus Problem
That Seemed So Simple
By Robert C. Wang, MD, and Narsing Rao, MD
Edited by Thomas Oetting, MD
One late Friday afternoon, 68-year-old Marty Alvarez presented as an emergency consult at our university. For about two months, he had struggled with severe reddening, tearing and discomfort in both eyes. He had tried artificial tears and topical anti-
allergy drops, but they provided no relief.
Mr. Alvarez had a complex medical history that included 16
years of type 2 diabetes and more than five years of treatment
with timolol maleate (Timoptic) and brimonidine tartrate (Alphagan)
for primary open-angle glaucoma. Fortunately, he had no diabetic
retinopathy. His eyes were markedly inflamed, severely hyperemic
and "weeping." His vision was 20/200 in the right eye and
20/300 in the left. The slit lamp examination revealed marked
superficial punctate keratopathy and severe vascular congestion
in both eyes. An area of uveal "show" from necrosis was seen
superior temporally on the right eye (below).
What's Your Diagnosis?
As both images indicate, the
right eye showed severe scleritis with scleromalacia supernasally.
Instillation of 2.5 percent phenylephrine resulted in little blanching of the episcleral vessels. No retinal or
retinal-vascular lesions were noted on dilated fundus exam. There was marked glaucomatous cupping, with a cup-to-disc ratio of 0.85 in both eyes. IOP was 30 mmHg in each eye.
According to Mr. Alvarez, he had no lung disease, kidney disease, ear inflammation, back pain or skin lesions. However, he had lost about 35 pounds over the last seven months. When asked further about other medical problems, he mentioned casually that he had recently undergone routine sinus surgery.
A Nagging Headache
About five months earlier, Mr. Alvarez explained, he began to experience sinus problems for the first time in his life.
He described severe pain and pressure over his cheeks and forehead that didn't respond after several courses of antibiotic therapy. A course of oral corticosteroids brought some improvement in his sinus symptoms; however, it also resulted in poor blood-sugar control and hospitalization for diabetic ketoacidosis.
Two months after his sinus headache started, a computed tomography scan was performed. It revealed pansinusitis with bilateral extension into the middle meatus. Mr. Alvarez's otolaryngologist scheduled him for sinus surgery. While waiting for surgery, he developed profound mixed hearing loss on the right and severe anemia.
The sinus surgery was uneventful, and it relieved Mr. Alvarez's symptoms of pain and pressure. The pathology report of the ethmoid and maxillary sinus tissue described the presence of reactive fibrosis and abundant chronic inflammatory cell infiltration. The histopathologic diagnosis was "marked chronic sinusitis."
After we evaluated Mr. Alvarez's medical history and our findings,
our initial diagnosis was necrotizing scleritis. This was
strongly supported by the vascular injection and thinning
of the underlying sclera. Mr. Alvarez was readmitted to the
hospital and started on high-dose intravenous (IV) steroids.
Because of his previous episode of poor blood-sugar control
while he was on steroids, a team approach was employed during
his hospitalization. An internist kept tabs on Mr. Alvarez's
diabetes, and a rheumatologist was consulted to further assess
his underlying systemic inflammatory condition. Initial lab
studies revealed an elevated erythrocyte sedimentation rate
of 80 millimeters per hour (the normal range is 0–15
mm/hour) and anemia, with a hematocrit of 30 ml/dl (normal
is 41–51ml/dl) and a hemoglobin of 10 grams/dl (normal
is 13.8–17.2 g/dl). His platelet count was 408,000 per
microliter (normal is 130,000–400,000/ µl). Urinalysis
was within normal limits, with no red blood cells or casts.
An antineutrophil cytoplasmic antibody (ANCA) titer revealed
elevated c-ANCA levels of 1:160 and a normal p-ANCA reading
of <1:20.
A Vicious Inflammation
Scleritis is an inflammatory process that can lead to rapid destruction of the eye and permanent loss of vision. Generally, it develops in the fourth to sixth decades and is more common in women. Half of all patients with scleritis have bilateral disease, and 25 to 50 percent harbor a systemic disease as well.
The causes of necrotizing scleritis
are mainly infectious in origin (due to viruses, bacteria, fungi, protozoa or other parasites) or autoimmune in nature. Autoimmune causes include rheumatoid arthritis, relapsing polychondritis, Wegener's granulomatosis, polyarteritis nodosa and inflammatory bowel disease (IBD).
Our leading diagnostic candidate was Wegener's granulomatosis. In retrospect, Mr. Alvarez's weight loss, anemia, hearing loss and pansinusitis clearly indicated that he had a systemic disease. He had no features of rheumatoid arthritis or any joint complaints, and IBD was unlikely, as he had no gastrointestinal problems. Both relapsing polychondritis and Wegener's granulomatosis can present with scleritis and
a saddle-nose deformity. However, polychondritis typically affects the ear pinna. Finally, Mr. Alvarez had no skin rashes, lesions or peripheral neuropathy to suggest polyarteritis nodosa.
Wegener's granulomatosis, an uncommon multisystem disease associated with necrotizing granulomatous vasculitis, typically affects males aged 8
to 80. The upper and lower respiratory tracts usually are involved, and epistaxis, rhinorrhea, chronic cough and saddle-nose deformity may occur. Chronic
pulmonary infiltrates, nodules and cavitary lesions may be found in the lungs. Wegener's vasculitis often affects the kidneys, causing granulomatous glomerulitis in 80 percent of patients.
The eyes are affected in 40 to 50 percent of Wegener's patients. Pseudotumor (with proptosis and orbital pain)
is a common finding. Patients also may have scleritis, peripheral ulcerative keratitis, conjunctivitis or dacryocystitis.
In rare cases, cotton-wool spots can be seen initially. In addition, there may be choroidal involvement, inflammation of the posterior sclera with serous retinal detachment or combined choroidal and retinal detachments.
Despite all the clinical signs and the specificity of the c-ANCA titer, a biopsy is needed before Wegener's can be definitively diagnosed. As c-ANCA can be negative on initial testing, evidence of necrosis with granulomatous reaction will be uncovered by pathologic examination. In Mr. Alvarez's case, histologic sections of sinus material were reviewed; they revealed necrotizing vasculitis with focal necrotizing granulomas and infiltrates of chronic inflammatory cells. A Verhoeff's stain showed destruction of the elastic lamina of the vessels. Stains for fungi and acid-fast organisms were negative.
Treatment and Recovery
Mr. Alvarez was started on oral prednisone at 60 milligrams per day, then
50 mg per day of cyclophosphamide (Cytoxan) was added to his regimen. He remained in the hospital for five days and dramatically improved within three days of therapy. One week after he went home from the hospital, his scleritis had completely resolved.
The fact that his illness was mistaken for something much
simpler is sobering. An accurate diagnosis of Wegener's is
critical, as the disease is fatal if left untreated. Up to
37 percent of patients experience loss of useful vision. The
treatment of choice–IV steroids and immunosuppressive
therapy with cyclophosphamide–leads to remission in 90
percent of patients. However, prolonged cyclophosphamide therapy
increases a patient's risk of developing a secondary malignancy,
a not-uncommon occurrence in Wegener's patients. To monitor
disease activity, the physician can follow serum c-ANCA levels;
if a patient's titers fail to return to normal, that may herald
a relapse.
Mr. Alvarez's case illustrates that a seemingly simple history of sinusitis may be anything but simple. His anemia, weight loss and other symptoms strongly suggested that he had a systemic disease. Moreover, scleritis often is a harbinger of systemic disease. Clearly, physicians never can afford to overlook a patient's medical history.
Fortunately, Mr. Alvarez remains well to this day. He has
no signs of kidney disease–and on his most recent follow-up
exam, his vision was 20/30 in each eye.
Drs. Wang and Rao are both at
the Doheny Eye Institute at the University of Southern California
in Los Angeles; Dr. Wang is a fellow and Dr. Rao is professor
of ophthalmology, pathology and ocular inflammatory disease.
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