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Ocular manifestations of cryoglobulinemia: a reappraisal

Eye volume 38, pages 585–593 (2024)Cite this article

Subjects

Abstract

Background/Objectives

To describe frequency and type of ocular manifestations in patients with cryoglobulinemic vasculitis (CV), as well as management approaches and outcomes.

Subjects/Methods

This was a retrospective, observational, cohort study of patients who were diagnosed with CV at a single center and regularly underwent a comprehensive ocular assessment.

Results

Ophthalmologic manifestations were recorded in 16 patients (28%). The diagnoses included dry eye disease and primary Sjögren syndrome in 5 and 2 patients, respectively; peripheral ulcerative keratitis and anterior scleritis in 1 patient each; hyperviscosity syndrome and hypertensive retinopathy in 2 patients each; and Purtscher- like retinopathy in 3 patients. Twelve patients (75%) were anti-HCV/HCV RNA-positive, 11 of whom achieved a sustained virologic response (SVR) following treatment with interferon-α2b plus ribavirin or direct-acting antivirals. All patients were treated with ocular lubricants. Systemic therapeutic measures, including glucocorticoids, immunosuppressive and biologic agents, induced the disappearance or ≥50% reduction of cryoglobulins and major signs of vasculitis in 11 patients (68.7%). In the remaining 5 patients (31.3%), cryoglobulins and CV manifestations remained unchanged or decreased by <50%. The corresponding ophthalmologic assessment showed a variable degree of improvement in the ocular symptoms in all but 2 patients (87.5%). The best corrected visual acuity following treatment improved in 26 eyes, was unchanged in 3 eyes, and worsened in 3 eyes.

Conclusions

Eye involvement is not a rare event in CV patients. A timely diagnosis and the correct employment of the available therapeutic measures may result in a favorable outcome of the ocular and extra-ocular manifestations.

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Fig. 1: Clinical evolution of anterior scleritis associated with Purpura Arthralgia Weakness Mixed Cryoglobulinemia Syndrome.
Fig. 2: Typical images of Purtscher-like retinopathy.

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Data availability

Data are available upon request and are with the corresponding author. The datasets generated during and/or analyzed during the current study are available from the corresponding author on reasonable request.

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Author information

Authors and Affiliations

  1. Department of Ophthalmology and Neuroscience, University of Bari “Aldo Moro”, Medical School, Bari, Italy

    Rosanna Dammacco & Giovanni Alessio

  2. Department of Surgery, Medicine, Dentistry and Morphological Sciences, University of Modena and Reggio Emilia, Modena, Italy

    Luca Cimino

  3. Ocular Immunology Unit, Azienda USL-IRCCS, Reggio Emilia, Italy

    Luca Cimino & Luca De Simone

  4. Department of Biomedical Sciences and Human Oncology, University of Bari “Aldo Moro”, Medical School, Bari, Italy

    Franco Dammacco

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Contributions

RD, GA and FD conceived and designed the study, retrieved, collected and had full access to all the data in the study, and take responsibility for the integrity of the data and the accuracy of the analysis. RD wrote the manuscript. LC and LDS revised the manuscript for important intellectual content and supplied additional images of Purtscher like retinopathy. All authors reviewed the manuscript, approved the draft submission, and accept responsibility for all aspects of this study.

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Correspondence to Rosanna Dammacco.

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RD, LC, LDS, GA and FD certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or nonfinancial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials.

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Dammacco, R., Cimino, L., De Simone, L. et al. Ocular manifestations of cryoglobulinemia: a reappraisal. Eye 38, 585–593 (2024). https://doi.org/10.1038/s41433-023-02738-y

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