Antireconverin antibodies in ocular chronic graft versus host disease: A new cause of nonparaneoplasic autoimmune retinopathy

Eur J Ophthalmol. 2023 Jul;33(4):NP105-NP110. doi: 10.1177/11206721221123779. Epub 2022 Sep 1.


Introduction: A case of a 48-year-old male with a nonparaneoplasic autoinmune retinopathy (nPAIR) due to chronic graft versus host disease (GVHD) after an allogenic stem cell transplantation (ASCT) is described.

Case report: The patient developed a bilateral rapidly progressive loss of visual acuity with bilateral optic disc edema and bilateral cystoid macular edema (CME) in the funduscopy, a ring scotoma in the visual field (VF) and photoreceptors dysfunction in the electroretinogram (ERG) 210 days after the ASCT. After ruling out other causes, the suspicion of autoimmune retinopathy (AIR) led to the study of antirecoverin antibodies which resulted positive. The exclusion of neoplasia discarded diagnosis of paraneoplasic autoinmune retinopathy (PAIR) and the temporal relationship with BMT led to the diagnosis of nonparaneoplasic autoinmune retinopathy (nPAIR) due to chronic graft versus host disease (GVHD). Oral corticosteroids led to resolution of the CME.

Conclusions: Diagnosis of AIR requires a high index of suspicion based on the typical findings on visual field, optical coherence tomography (OCT) and ERG, which force requesting antirecoverin antibodies. However, diagnosis is often delayed because of the need to exclude other causes. Knowing typical symptoms and signs in for a quick action is important because an earlier diagnosis and treatment will improve visual prognosis since the loss of vision already established is irrecoverable. To our knowledge, this is the first reported case in the literature of nPAIR with CME and optic disc edema due to GVHS after ASCT.

Keywords: Allogenic stem cell transplantation; antirecoverin antibodies; bone marrow transplant; cystoid macular edema; graft versus host disease; nonparaneoplasic autoimmune retinopathy.

Publication types

  • Case Reports

MeSH terms

  • Autoimmune Diseases* / complications
  • Autoimmune Diseases* / diagnosis
  • Bronchiolitis Obliterans Syndrome*
  • Graft vs Host Disease* / complications
  • Graft vs Host Disease* / etiology
  • Humans
  • Macular Edema* / diagnosis
  • Macular Edema* / drug therapy
  • Macular Edema* / etiology
  • Male
  • Middle Aged
  • Papilledema*
  • Retinal Diseases* / diagnosis
  • Retinal Diseases* / drug therapy
  • Retinal Diseases* / etiology
  • Tomography, Optical Coherence / methods