Author: Rosita Lucía Wakfie Corieh (Spain)
Co-authors: Francisco Javier Vicente Andreu, Ana María Álvarez Gómez, Olga Sánchez-Pernaute, Inés Hernanz Rodríguez, Ester Carreño
Purpose
We present the case of a patient with acute bilateral anterior uveitis associated with autoimmunity phenomena, with antecedents of allogeneic transplantation of identical HLA hematopoietic progenitors 2 years prior to presentation due to classical Hodgkin lymphoma stage IV-B.
Setting/Venue
A 26-year-old man with a history three years ago of bulky mediastinal stage II-A Hodgkin lymphoma at diagnosis and subsequent bone progression (stage IV-B) treated with 4 lines of chemotherapy and immunotherapy with Nivolumab 10 months prior, followed by allogeneic transplantation of hematopoietic progenitors of sibling with identical HLA, went to the emergency room for redness of both eyes, showing bilateral acute anterior uveitis (BAAU).
Methods
We examined the best-corrected visual acuity (BCVA), intraocular pressure (IOP), performed slit-lamp biomicroscopy (SLB), dilated fundus examination (DFE) and spectral domain optical coherence tomography of the macula (SD-OCT m) and of the nerve fiber layer of the retina (SD-OCT CFNR). Autoimmunity blood tests. Bone marrow aspirate. Peripheral blood study with fluorescence by in situ hybridization (FISH). Emission tomography-computed tomography (PET-CT) and body magnetic resonance imaging (MRI).
Results
The patient had BCVA in the right eye 20/20 and in the left eye 20/25, showing 1+ cells in the anterior chamber and fine retrokeratic precipitates, without vitritis and with normal DFE. IOP, SD-OCT m and SD-OCT CFNR were normal. The presentation of uveitis occurred just after discontinuing cyclosporine A. The episode of BAAU completely resolved with topical corticosteroids (dexamethasone). He was on prophylactic treatment with acyclovir and trimethopin-sulfamethoxasol. Later he had Raynaud's phenomenon and an episode of autoimmune thyroiditis (with positive antithyroglobulin and antimicrosomal antibody). No intercurrent infections (cytomegalovirus, toxoplasmosis, human immunodeficiency virus and tuberculosis), mucocutaneous, musculoskeletal or neurological involvement. In the study of peripheral blood, a complete graft was observed without evidence of receptor cells, an immunophenotype with a decrease in CD4 T lymphocytes (cluster of quadruple differentiation) and the rest of subpopulations of lymphocytes were normal. After the diagnosis of uveitis, the patient presented presumed autoimmune pancytopenia that spontaneously improved.
Conlusions
Immune reconstitution inflammatory syndrome (IRIS), previously known as immune restoration disease (IRD) or immune reconstitution syndrome (IRS), is characterized by paradoxical worsening of treated opportunistic infection or the unmasking of previously subclinical, untreated infection in patients with HIV after initiation of antiretroviral therapy. Inmune recovery uveitis (IRU) is the most common form of immune reconstitution inflammatory syndrome in HIV-infected patients with cytomegalovirus retinitis who are receiving highly active antiretroviral therapy (HAART). Our patient presented non-infectious IRU after reconstitution with allogeneic bone marrow transplantation. To our knowledge, there are no similar cases reported in the literature. The management of these patients includes ruling out concomitant infectious pathologies, as well as other systemic affectations and study of bone marrow and peripheral blood.
Financial Disclosure
None
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