Intraocular inflammation as the main manifestation of Rickettsia conorii infection

Abstract

Objective: To report the clinical features and management of seven cases of intraocular inflammation caused by Rickettsia infection and review published literature.

Methods: Rickettsia conorii or Rickettsia spp. infection was diagnosed based on the following criteria: (1) positive serology according to the European Guidelines, (2) titer normalization after specific treatment, and (3) complete resolution of ophthalmic disease and accompanying symptoms after antibiotic therapy.

Results: Seven patients were referred for uveitis of unknown etiology. All came from regions where Mediterranean spotted fever is prevalent. One patient met the European guidelines criteria for Rickettsia spp. infection, while the other six cases met the criteria for R. conorii infection. The main symptoms were visual loss, floaters, eye redness, photophobia, and ocular pain. Predominant ophthalmic signs included vasculitis, choroiditis, vitritis, and macular edema. All patients required antibiotic treatment that resulted in the remission of the infection. Doxycycline was the first choice and the only antibiotic used to treat four patients. One patient needed ciprofloxacin as a second antibiotic after not responding to doxycycline. Two patients had doxycycline as a second antibiotic after not responding primarily to sulfonamides (which had been given after 2-3 days of doxycycline gastric intolerance); one of these patients needed ciprofloxacin as a third antibiotic.

Conclusion: Intraocular inflammation can occur as the main manifestation of Rickettsia conorii or Rickettsia spp. infection. It should be considered as a differential diagnosis for uveitis especially for patients living in countries where this infection is endemic in the world. Antibiotic treatment remains effective in the management of Rickettsia infection.

Keywords: Mediterranean spotted fever; Rickettsia conorii; intraocular inflammation; uveitis.

Figure 1 (A)

Case 5. Fundus photo of the right eye shows a hyperemic disc with slightly indistinct borders especially nasally. The arteries are attenuated and the veins exhibit severe dilation and tortuosity with focal constrictions at areas of arterio-venous crossings. The posterior pole shows multiple intraretinal hemorrhages and cotton wool spots and retinal thickening. The perifoveal vessels are also dilated and tortuous and the foveal reflex is dull. There is a white-centered hemorrhage at the fovea.

Figure 1 (B)

Case 5. After treatment with sulfonamides and doxycycline, fundus photo shows that disc vessels are still dilated, but with distinct disc borders. There is marked decrease in vessel tortuosity and dilation. There is also an evident decrease in retinal edema and hemorrhages as compared to Figure 1A.

Figure 2 (A and B)

Case 4. Angiogram of both eyes shows papillary, peri-papillary, and choroidal fluorescence along the superior and inferior arcades. The macula area of the left eye also shows staining along the superior peri-foveal region.

Figure 2 (C and D)

Case 4. After treatment with sulfonamides, doxycycline, and ciprofloxacin, the angiogram of both eyes show normal disc fluorescence. There is no choroidal staining and no angiographic evidence of macular edema.

Figure 3 (A)

Case 2. Angiogram of the left eye shows masking of the retinal, choiroidal, and disc fluorescence, which is due to vitreous haze (vitritis). The veins are slightly dilated with simultaneous laminar filling of the superior and inferior temporal arcades. The perifoveal vessels appear dilated.

Figure 3 (B)

Case 2. After treatment with doxycycline, angiogram shows less masking due to vitreous haze. There are no choroidal hyperflourescence and the perifoveal vessels appear non-dilated.

Figure 3 (C)

Case 2. Anterior segment photo showing posterior synechiae in the left eye of the patient following severe anterior iridocyclitis.