Scleritis and retinal vasculitis associated with a type II mixed cryoglobulinemia.

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 2mm/ h. Interferon-gamma release assay (Quantiferon 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. 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. After a first course of oral non-steroidal anti-inflammatory drugs and topical dexamethasone, his ophthalmic condition worsened with an infero-temporal 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.