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Panuveitis & Occlusive Vasculitis Leads to the Diagnosis of Disseminated
Coccidioidomycosis
Erin V. Frillarte, OD & Alyon J. Wasik, OD, FAAO
Southern Arizona VA Health Care System, Arizona
Abstract:
A 65 year old Caucasian female presents with severe panuveitis & occlusive vasculitis is
subsequently diagnosed with disseminated Coccidioidomycosis, a dimorphic fungus endemic to
regions in Arizona.
I. Case History
A. Patient Demographics: 65 year old Caucasian female
B. Chief Complaint:
1. Unilateral progressively painful red eye with blurred vision, excessive tearing,
and photophobia
C. Pertinent Ocular History:
1. Unremarkable
D. Pertinent Medical History:
1. Hypothyroidism
2. GERD
3. Allergies
4. Osteoporosis
5. Depression/Dysthymic disorder
6. Actinic Keratosis
E. Current Medication:
1. Levothyroxine
2. Omeprazole
3. Cetirizine
4. Venlafaxine
5. Aripiprazole
F. Other Salient Information:
1. Originally from Ohio, last there in 2008
2. Arizona resident for 20 years
II. Pertinent Findings
A. BCVA: OD 20/25; OS 20/70-1 PHNI
B. Anterior Segment:
1. Moderately diffuse bulbar injection with mild inferior fine keratic precipitates
and corneal edema OS
2. Moderate anterior uveitis OS, subsequently affecting OD
3. Koeppe nodules at pupillary margin greater superior and nasally, with
subsequently mild rubeosis due to occlusive vasculitis OS
C. Fundus Exam:
1. Mild to moderate vitritis/vitreous haze OS, subsequently affecting OD
2. Mild sheathing of veins, greater superiorly than inferiorly, leading to severe
occlusive vasculitis and marked arteriolar attenuation, along with intraretinal
hemorrhages concentrated in the posterior pole OS
3. Subsequent vascular fibrosis along superior arcades, and arteriolar attenuation
OD
D. Laboratory studies:
1. Coccidioidomycosis IDCF: positive & titer detectable
a. Coccidioidomycosis: IDTP negative
2. Varicella zoster virus serology: positive
3. RPR/FTA-ABS, Lyme titer, PPD, Bartonella henselae, toxoplasmosis, HIV:
negative/non reactive
4. Cytomegalovirus DNA: not detected
a. CD-4 count: WNL
5. ANA titer: 1:320
a. SM IGG autoantibodies: negative
b. DNA AutoAB, double strand: indeterminate
c. RF, C- & P-ANCA: negative
6. HLA B-27/B-44: detected
7. Sensitive C-RP, ESR: elevated
8. CBC, Blood Chemistry 7: unremarkable
E. Radiology studies
1. CT chest with contrast: nonspecific calcified 1.0 x 1.1 cm inferior right
middle lobe pulmonary nodule
2. PET scan: PET-negative pulmonary nodule, which was determined to likely
represent Coccidioidomycosis
3. MRI/MRA brain: unremarkable
F. Specialized tests
1. Intravenous Fluorescein Angiography: periphlebitis OD; periarteritis and
periphlebitis with mild occlusion OS
2. OCT Macular Cube: Central foveal thickness OD 239 μm, OS 261 μm w/
normal foveal contour OU
3. Gonioscopy: ATM sup; scleral spur nasal, temp, inf / no NVA 360° OU
G. Consultations
1. Rheumatology: rule out underlying systemic autoiummune diseases and assist
with management of immunosuppressive treatment
2. Infectious disease: manage and treat Coccidioidomycosis
III. Differential Diagnosis
A. Primary/Leading differentials
1. Sarcoidosis
2. Syphilis
3. CMV retinitis
4. Systemic lupus erythematosus
B. Secondary differentials
1. Other viral (herpes, HIV) retinitis
2. Intermediate uveitis
3. Fungal endophthalmitis
4. Toxoplasmosis
5. Ocular histoplasmosis
6. Tuberculosis
7. Lyme disease
8. Wegener’s granulomatosis
9. Behçet’s disease
IV. Diagnosis and Discussion
A. Rapidly progressive severe non-granulomatous bilateral panuveitis with severe
occlusive vasculitis OS>OD
1. Subsequent finding of disseminated Coccidioidomycosis through extensive
laboratory/radiology studies
2. No previous incidences reported in the literature
3. HLA-B27 & ANA positive but unlikely etiologies as there is no evidence to
suggest underlying systemic autoimmune disease process at this time
B. Disseminated Coccidioidomycosis
1. Spread to the skin, liver, brain and meninges, heart, GI tract, adrenals, kidney
and bladder rare affecting less than 1% with primary Coccidioidomycosis
2. Systemic antifungal treatment depends on the type and severity of infection
C. Ocular sequelae infrequently reported though true prevalence is unknown
1. Anterior segment may include: phlyctenular conjunctivitis, peripheral anterior
synechiae, granulomatous iridocyclitis with iris nodules and mutton-fat keratic
precipitates, and episcleral/conjunctival lid lesions
2. Posterior segment may include: Multiple yellow-white chorioretinal lesions
with pigmented border, juxtapapillary choroidal infiltrates with variable retinal
involvement, retinal hemorrhages, serous retinal detachment, vitreous haze, and
vascular sheathing
V. Treatment and Management
A. Topical Medications
1. Pred Forte and Cyclogel x 3 months
B. Systemic Medications
2. Oral Prednisolone, Valgancyclovir, Fluconazole and Azathioprine x 10
months
C. Anti-VegF intravitreal injection (Avastin) with repeated pan-retinal photocoagulation
for occlusive disease
D. Subtenon Kenalog injection for vitritis
E. Dilated fundus exam every 6 months
F. Patient education for possible reoccurrence
G. Photodocumentation of posterior segment findings
H. Intravitreal fluorescein angiography if vasculature is involved
I. Gonioscopy evaluation if rubeosis is found
J. Continue care with infectious disease, pulmonary, and rheumatology clinics
VI. Conclusion
A. Coccidioidomycosis is subclinical in most individuals, with ocular involvement being
regarded as rare and usually associated with disseminated disease.
B. The diagnosis of intraocular Coccidioidomycosis was made as a result of positive
serology testing but can sometimes be falsely negative which may result in the need
for direct examination of fluid samples.
C. Infectious disease specialists should be involved in the care of patients with this
condition, and as in severe cases like our patient, oral antifungals like Fluconazole
were recommended.
D. A rheumatology consult was important to rule out autoimmune disease and in the use
of steroid sparing agents, like Azathioprine, may be used in conjunction with
oral corticosteroids as an immunosuppressant.
E. The incidence of Coccidioidomycosis is increasing in the United States, and should be
considered as a differential diagnosis in patients who have been traveling or living in
highly endemic areas and present with ocular findings consistent with previously
reported ocular sequelae, as well as panuveitis and occlusive vasculitis not
attributable to other conditions.
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