Q J Med 2014; 107:585–586 doi:10.1093/qjmed/hct235 Advance Access Publication 19 November 2013
Clinical picture Scleritis and retinal vasculitis associated with a type II mixed cryoglobulinemia peripheral ulcerative keratitis leading to the use of high doses of topical dexamethasone and artificial tears with a favorable outcome at follow-up 3 months later.
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The patient, a 64-year-old man, with a past history of an IgM monoclonal gammopathy of undetermined significance (MGUS), presented with a 5-day history of ocular pain and redness in his left eye. The best-corrected visual acuity was conserved at 25/25 in both eyes. Topical instillation of phenylephrine did not blanch leading to the diagnosis of temporal anterior non-necrotizing scleritis (Figure 1a). Fluorescein fundus angiograms revealed a bilateral temporal peripheral retinal vasculitis (white arrows) (Figure 1b). Apart from the ophthalmic symptoms, clinical examination remained normal. Erythrocyte sedimentation rate was 2 mm/ h. Interferon-gamma release assay (QuantiferonÕ TB Gold-in-Tube, Cellestis) was negative. Screening for antineutrophil cytoplasmic antibodies, rheumatoid factor yielded negative findings. Transient antinuclear antibodies without specificity (controlled negative) and a type II mixed cryoglobulinemia with a monoclonal IgM kappa and polyclonal immunoglobulins were found nonassociated with hepatitis C virus. No other complication of this cryoglobulinemia was found with a normal creatinine clearance without proteinuria. Bone marrow biopsy was performed, without plasmocytosis, confirming the MGUS diagnosis. About one-half of patients with scleritis have an underlying disease, mostly rheumatoid arthritis or vasculitis, such as granulomatosis with polyangiitis. Cryoglobulins, immunoglobulins that reversibly precipitate at temperatures below 378C, lead to vasculitis affecting small vessels. Skin, glomeruli and peripheral nerves are often involved. However, ophthalmologic presentations, affecting both anterior and/or posterior segment, are rare.1 A decrease temperature of the cornea from the core temperature has been proposed to explain the cryoprecipitation in the anterior segment associated with a deposition of circulating immune complexes.2 After a first course of oral non-steroidal anti-inflammatory drugs and topical dexamethasone, his ophthalmic condition worsened with an infero-temporal
Figure 1. (a) Scleritis of the left eye after instillation of topical phenylephrine. (b) Retinal temporal vasculitis (white arrow) on the fluorescein fundus angiogram (left eye).
! The Author 2013. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For Permissions, please email: [email protected]
586
Clinical picture
Photographs and text from: S. Salah Jr , L. Imbeau Jr and M.L. Le Lez, Department of Ophthalmology, Hopital Bretonneau, University of Tours, 2 Boulevard Tonnele´, 37000 Tours, France; S. Regina, Department of Hematology, Pole Sante´ Le´onard de Vinci, Avenue du Pr Alexandre Minkowski, 37175 Chambray les Tours, France; B. Lioger, Department of Internal Medicine, Hopital Bretonneau, University of Tours, 2 Boulevard Tonnele´, 37000 Tours, France. email: [email protected]
References 1. Johnson CC, Ohlstein DH. Peripheral ulcerative keratitis and necrotizing scleritis initiated by trauma in the setting of mixed cryoglobulinemia. Case Rep Ophthalmol 2011; 2:392–7. 2. Telander DG, Holland GN, Wax MB, Van Gelder RN. Rubeosis and anterior segment ischemia associated with systemic cryoglobulinemia. Am J Ophthalmol 2006; 142:689–90.
Conflict of interest: None declared.
Downloaded from by guest on November 15, 2015
Clinical picture Scleritis and retinal vasculitis associated with a type II mixed cryoglobulinemia peripheral ulcerative keratitis leading to the use of high doses of topical dexamethasone and artificial tears with a favorable outcome at follow-up 3 months later.
Downloaded from by guest on November 15, 2015
The patient, a 64-year-old man, with a past history of an IgM monoclonal gammopathy of undetermined significance (MGUS), presented with a 5-day history of ocular pain and redness in his left eye. The best-corrected visual acuity was conserved at 25/25 in both eyes. Topical instillation of phenylephrine did not blanch leading to the diagnosis of temporal anterior non-necrotizing scleritis (Figure 1a). Fluorescein fundus angiograms revealed a bilateral temporal peripheral retinal vasculitis (white arrows) (Figure 1b). Apart from the ophthalmic symptoms, clinical examination remained normal. Erythrocyte sedimentation rate was 2 mm/ h. Interferon-gamma release assay (QuantiferonÕ TB Gold-in-Tube, Cellestis) was negative. Screening for antineutrophil cytoplasmic antibodies, rheumatoid factor yielded negative findings. Transient antinuclear antibodies without specificity (controlled negative) and a type II mixed cryoglobulinemia with a monoclonal IgM kappa and polyclonal immunoglobulins were found nonassociated with hepatitis C virus. No other complication of this cryoglobulinemia was found with a normal creatinine clearance without proteinuria. Bone marrow biopsy was performed, without plasmocytosis, confirming the MGUS diagnosis. About one-half of patients with scleritis have an underlying disease, mostly rheumatoid arthritis or vasculitis, such as granulomatosis with polyangiitis. Cryoglobulins, immunoglobulins that reversibly precipitate at temperatures below 378C, lead to vasculitis affecting small vessels. Skin, glomeruli and peripheral nerves are often involved. However, ophthalmologic presentations, affecting both anterior and/or posterior segment, are rare.1 A decrease temperature of the cornea from the core temperature has been proposed to explain the cryoprecipitation in the anterior segment associated with a deposition of circulating immune complexes.2 After a first course of oral non-steroidal anti-inflammatory drugs and topical dexamethasone, his ophthalmic condition worsened with an infero-temporal
Figure 1. (a) Scleritis of the left eye after instillation of topical phenylephrine. (b) Retinal temporal vasculitis (white arrow) on the fluorescein fundus angiogram (left eye).
! The Author 2013. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For Permissions, please email: [email protected]
586
Clinical picture
Photographs and text from: S. Salah Jr , L. Imbeau Jr and M.L. Le Lez, Department of Ophthalmology, Hopital Bretonneau, University of Tours, 2 Boulevard Tonnele´, 37000 Tours, France; S. Regina, Department of Hematology, Pole Sante´ Le´onard de Vinci, Avenue du Pr Alexandre Minkowski, 37175 Chambray les Tours, France; B. Lioger, Department of Internal Medicine, Hopital Bretonneau, University of Tours, 2 Boulevard Tonnele´, 37000 Tours, France. email: [email protected]
References 1. Johnson CC, Ohlstein DH. Peripheral ulcerative keratitis and necrotizing scleritis initiated by trauma in the setting of mixed cryoglobulinemia. Case Rep Ophthalmol 2011; 2:392–7. 2. Telander DG, Holland GN, Wax MB, Van Gelder RN. Rubeosis and anterior segment ischemia associated with systemic cryoglobulinemia. Am J Ophthalmol 2006; 142:689–90.
Conflict of interest: None declared.
Downloaded from by guest on November 15, 2015
