|Year : 2017 | Volume
| Issue : 1 | Page : 34-36
Nodular Scleritis with Stargardt’s Disease: A Rare Case Report
Anubhav Chauhan, Lalit Gupta
Department of Ophthalmology, Dr Yashwant Singh Parmar Government Medical College, Sirmaur, Himachal Pradesh, India
|Date of Web Publication||1-Mar-2017|
Pine Castle, Near Mist Chamber, Khalini, Shimla - 171 002, Himachal Pradesh
Source of Support: None, Conflict of Interest: None
A 30-year-old female presented with recurrent red, painful right eye. Ocular examination revealed nodular scleritis and on complete workup, tuberculosis was found to be the causative agent. Stargardt’s disease in the same patient was noted as an incidental finding. To the best of our knowledge and after extensive literature search on the internet, this is probably the first reported case of both these diseases of total different etiologies occurring together.
Keywords: Ocular, scleritis, stargardt
|How to cite this article:|
Chauhan A, Gupta L. Nodular Scleritis with Stargardt’s Disease: A Rare Case Report. MAMC J Med Sci 2017;3:34-6
| Introduction|| |
Scleritis is a painful inflammatory eye disease while Stargardt’s disease is a form of hereditary macular dystrophy. Scleritis is an uncommon condition characterized by edema and cellular infiltration of the entire thickness of the sclera. Stargardt’s disease is characterized by diffuse accumulation of lipofuscin within the retinal pigment epithelium. We report a case of both the diseases in a young female.
| Case Report|| |
A 30-year-old female presented to a secondary care institute of the hilly state with a history of painful, red right eye for 1 week. There was no history of any systemic disease/any relevant personal/family history. She was a laborer by occupation and had never consulted an ophthalmologist before. On examination, her general physical and systemic examination was normal. Her visual acuity was 6/18 in both the eyes without any further improvement with pinhole. Surprisingly, diminution of vision never bothered her as she stated that she had decreased vision since childhood. Slit lamp examination revealed nodular scleritis at 12 O’clock position in the right eye, which was confirmed by instillation of 2.5% phenylephrine drops which did not constrict/blanch the area affected. The pupillary reactions, ocular movements, intraocular pressure, and color vision were normal. Fundus examination with direct ophthalmoscopy revealed “beaten bronze” appearance of bilateral macula. A suspicion of Stargardt’s disease arose which was confirmed by fundus fluorescein angiography showing characteristic macular hyperfluorescence and a “dark” choroid. Genetic analysis, B-scan ultrasonography, optical coherence tomography, and electroretinogram could not be performed due to nonavailability of these facilities at our institute [Figure 1][Figure 2][Figure 3][Figure 4][Figure 5][Figure 6].
The investigations carried out were complete hemogram with erythrocyte sedimentation rate (ESR), blood sugar, C-reactive proteins, rheumatoid factor, antinuclear antibodies, antineutrophil cytoplasmic antibodies, venereal disease research laboratories, serum angiotensin converting enzyme and calcium levels, Lyme titer, blood culture, toxoplasma, rubella, cytomegalovirus and herpes virus titers, viral markers (hepatitis A, B, and human immunodeficiency virus) tests, X-ray chest, Mantoux test, quantiferon-TB gold test, and computed tomography of head and orbit. An elevated ESR with positive Mantoux test and quantiferon-TB gold test was the only positive finding. The patient was started on antitubercular therapy with oral naproxen and there was a marked improvement in her signs and symptoms. She was also explained the prognosis regarding her Stargardt’s disease and was advised consultation at a higher facility for further investigations.
| Discussion|| |
Scleritis is a painful inflammatory eye disease with multiple etiologies. The mean age of presentation is 52 years and it is associated with systemic disease in 50% of patients. Scleritis can be anterior, with involvement of the sclera that is visible to the naked eye or posterior, with involvement of sclera that is enclosed by the orbital tissues. Anterior scleritis is further subdivided into diffuse, nodular, or necrotizing type. Common etiological agents are rheumatoid arthritis, Wegener’s granulomatosis, herpes zoster, tuberculosis, syphilis, intraocular tumors, and systemic lupus erythematosus. Oral nonsteroidal anti-inflammatory drugs, corticosteroids, immunosuppressive drugs such as methotrexate, azathioprine, mycophenolate mofetil and cyclosporine, and infliximab (an antibody to tumor necrosis factor) are treatment modalities.
Stargardt disease is a hereditary macular dystrophy, with a prevalence of 1:10,000 in the United States. It is characterized by decreased central vision within the first decades of life and the presence of yellowish white flecks limited to the macular region or beyond. Death of photoreceptors is seen. Clinical trials with the use of novel pharmaceuticals, gene augmentation therapy, and stem cells are ongoing or planned.
We would like to thank our patient.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Usui Y, Parikh J, Goto H, Rao NA. Immunopathology of necrotising scleritis. Br J Ophthalmol 2008;92:417-9.
Smith JR, Mackensen F, Rosenbaum JT. Therapy insight: Scleritis and its relationship to systemic autoimmune disease. Nat Clin Pract Rheumatol 2007;3:219-26.
Salvatore S, Fishman GA, McAnany JJ, Genead MA. Association of dark-adapted visual function with retinal structural changes in patients with Stargardt disease. Retina 2014;34:989-95.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]