Bilateral necrotizing scleritis and blindness in the myelodysplastic syndrome presumably due to relapsing polychondritis

Abstract

ABSTRACT.Purpose: The purpose of this study was to report a case of bilateral blindness, bilateral necrotizing scleritis, and bilateral deafness in a patient with myelodysplastic syndrome (MDS). In such a patient, the possibility of relapsing polychondritis (RPC) associated with MDS must be considered. Case Report/Methods: A 66-year-old patient suffered from myelodysplastic syndrome (MDS). Shortly before his death, he became bilaterally blind and deaf. A biopsy was taken from the conjunctiva and the bone marrow, and both eyes were obtained after death for further investigation. Findings of the clinical and laboratory work-up for the patient's hematologic disorder as well as an examination of the eyes by light microscopy and immunohistochemistry are presented. Results: Ocular sections showed a diffuse necrotizing scleritis with moderate uveitis and no identifiable infectious agent. Neither was there any evidence of a leukemic infiltration. -The deafness had been due to inner ear failure, and the patient died of a cardiac failure. Conclusions: Non-infectious scleritis associated with inner ear deafness is a strong indication of relapsing polychondritis (RPC). Furthermore, RPC can be associated with MDS. Thus, in addition to leukemic infiltration and infection involving ocular structures, ophthalmologists and otolargyngologists should be aware of the association between MDS and RPC and the potential complications.

The Diagnosis and Management of Anterior Scleritis

“…Relapsing polychondritis with necrotizing scleritis has been described as a paraneoplastic syndrome in myelodysplastic disease. 15 Patients with seronegative spondyloarthropathies, including ankylosing spondylitis, Reiter's syndrome, psoriatic arthritis, and arthritis with inflammatory bowel disease, can develop scleritis. Such patients with spondyloarthropathies are less likely to develop decreased vision or peripheral ulcerative keratitis than those with Wegener granulomatosis, rheumatoid arthritis, or relapsing polychondritis.…”

Patient Perception of Disease‐Related Symptoms and Complications in Relapsing Polychondritis

“…RP is defined by inflammation of cartilaginous structures and the disease course and clinical manifestations are highly variable. End organ complications due to inflammation of cartilage and proteoglycan rich structures are diverse and include deformity of the ears(2), nose(3) and chest(4), subglottic stenosis (5), obstructive lung disease, tracheomalacia(6), bronchomalacia(7), recurrent pneumonia(8), mitral and aortic regurgitation(9, 10), large and medium vessel aneurysms(11), blindness(12), deafness(13), renal insufficiency(14), chronic pain, and sudden death(15–17). The protean aspects of RP pose diagnostic challenges.…”

Visual loss in relapsing polychondritis: rapid progression of orbital inflammation

“…Most commonly involved organs are ear, nose, peripheral joints and upper respiratory tracts, and ocular involvement is also reported in 20% to 60% of RPC patients; most common ocular manifestations are episcleritis, scleritis and conjunctivitis . Orbital inflammation or optic neuropathy is also rarely reported; however, visual loss from this condition is extremely rare, and only two cases were reported . Here, we report an RPC patient with rapid progression of orbital inflammation eventually resulting visual loss 4 days after first ocular manifestation.…”

“…There have been a few case reports of orbital inflammation associated with RPC; however, visual loss associated with RPC is extremely rare; only two case reports mentioned blindness associated with RPC . In one case, orbital inflammation was confirmed with post‐mortem pathological examination, but optic nerve involvement was not present . In other case, the patient got blind, but no abnormality was found in orbit CT and MRI .…”