51-year-old female with Systemic Lupus Erythematosus (SLE) treated with cellcept 1g BID and prednisone 3mg daily
Three-day history of acute shortness of breath
No changes in vision
Past medical history: SLE, Chronic kidney disease stage V, anemia of chronic disease, hypertension, diabetes
Past ocular history: Negative
Started on pulse steroids of IV solumedrol and dexamethasone for a presumed lupus flare
She then developed pancytopenia with neutropenic fever
Blood cultures were positive for cryptococcus neoformans
Ophthalmology consulted to r/o ocular involvement
VA: OD: 20/40; OS: 20/40
Pupils: Round and reactive OU, no RAPD
IOP: 15; 17
EOM: Full OU
Slit lamp exam: Significant for 1+ NS OU
Sarcoidosis, Behcet, sympathetic ophthalmia, VKH
Intraocular lymphoma, leukemic infiltrates
Lumbar puncture performed which was positive for cryptococcal neoformans antigen
Crypotococcus neoformans initially inoculates the pulmonary system. The organism then spreads hematogenously overcoming the blood brain barrier and reaching the choroid where the organism multiplies and subsequently invades the retina followed by vitreous cavity.
The patient was treated for cryptococcal meningitis:
Induction therapy: AmphoB IV (5mg/Kg) + flucytosine for 14 days.
Consolidation therapy: Fluconazole 400mg PO daily for eight weeks.
At four weeks follow up the patient denies any changes in VA and she was still receiving treatment with fluconazole 400mg PO daily.
Prognosis and Future Directions
There has been a 10-fold increase in fungemia in inpatients over the past 20 years.
Related to increased patient risk factors: IV drug use, indwelling catheters, trauma, hyperalimentation, GI surgery, diabetes, immunosuppression, HIV/AIDS
Ocular prognosis for fungal endogenous endophthalmitis has improved over the recent years. Historically the rates of fungal endophthalmitis was 9-45% but more recent reports demonstrate a rate of 2-9%.
Newer agents such as fluconazole and especially voriconazole have good intraocular penetration with broad spectrum activity against organisms. Chorioretinitis without vitreous involvement can be treated with systemic treatment alone.
Patients with vitritis or active macular disease require a vitreous tap and injection of antimicrobials, and rarely in patients with progressive disease, a pars plana vitrectomy may be necessary.
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