Systemic Sclerosis Presenting with Simultaneous Retinal Vasculitis in One Eye and Optic Neuritis in the other along with Severe Immune Haemolytic Anaemia Keywords: Systemic sclerosis, Retinal vasculitis, Optic neuritis, Autoimmune haemolytic anaemia
نویسنده
چکیده
Systemic Sclerosis (SSc) is a connective tissue disorder which involves multiple systems in a chronic progressive manner. Micro–angiopathic haemolytic anaemia is a distinguished feature of “scleroderma renal crisis”, which is manifested by severe hypertension, a rapidly progressing renal dysfunction and hyperreninaemia and is seen in patients with an early, diffuse form of the disease. A nervous system involvement is rare, though entrapment neuropathies have been reported. Who presented with a sequential loss of vision in both eyes; due to retinal vasculitis in right eye and optic nerve demyelination in the left eye. She also had severe Coombs’ negative haemolytic anaemia in absence of any renal dysfunction or hypertension. Both the ophthalmologic and the haematologic manifestations are very rare and both responded well to oral prednisolone therapy. We are presenting a case of a 40 year old female with typical multisystem involvement in systemic sclerosis. [table/Fig-1]: Ophthalmoscopy revealed evidence of retinal vasculitis in her right eye manifested by superficial retinal hemorrhages, cotton wools spot, and retinal venous dilatation [table/Fig-2]: Thick patch of skin below her right eye www.jcdr.net Avijit Moulick et al., Systemic Sclerosis Presenting with Simultaneous Optic Neuritis, Retinal Vasculitis and Immune Haemolytic Anemia Journal of Clinical and Diagnostic Research. 2013 Dec, Vol-7(12): 2978-298
منابع مشابه
P46: Optic Neuritis as the First Manifestation of the Primary Antiphospholipid Antibody Syndrome
Optic neuritis is commonly associated with diseases such as Multiple Sclerosis, infections, taking special drugs, etc. but recently we have observed a patient that presented retro bulbar optic neuritis as a manifestation of antiphospholipid syndrome. A 35 y/o young woman with the history of chickenpox admitted to the Qhaem hospital (Mashhad, Iran) with the c/o progressive pain and blurred visio...
متن کاملOcular Manifestations of Systemic Lupus Erythematosus: A Review of the Literature
About one-third of patients suffering from systemic lupus erythematosus have ocular manifestations. The most common manifestation is keratoconjunctivitis sicca. The most vision threatening are retinal vasculitis and optic neuritis/neuropathy. Prompt diagnosis and treatment of eye disease is paramount as they are often associated with high levels of systemic inflammation and end-organ damage. In...
متن کاملA challenging entity: multiple sclerosis or collagen tissue disorders: A case series of 6 patients
Background: Multiple sclerosis and other demyelinating processes are sometimes difficult to differentiate from the neurological involvement in autoimmune diseases. Distinguishing multiple sclerosis from other lesions due to autoimmune diseases is crucial to avoid unsuitable or delayed treatments. Methods: Charts of 6 patients diagnosed with mimicking multiple sclerosis between 1996 and 2014 we...
متن کاملOptic neuritis and retinal vasculitis as primary manifestations of systemic lupus erythematosus.
Systemic lupus erythematosus (SLE) is a common multisystem disorder. However, retinal vasculitis as a primary manifestation of SLE is uncommon, accounting for only 4% of causes of retinal vasculitis. The postulated mechanism appeared to be vaso-occlusion of the retinal arterioles by thrombosis, with resultant ischaemia. Optic neuropathy in SLE is also rare, with a prevalence of 1%. This is a ca...
متن کاملBilateral simultaneous optic neuritis in childhood systemic lupus erythematosus. A case report.
The authors describe an 11-year-old girl with systemic lupus erythematosus (SLE) who developed simultaneous bilateral acute optic neuritis. Severe initial visual loss followed by permanent visual deficit occurred in both eyes despite therapeutic intervention. Recurrence of optic neuritis in one eye caused the vision to deteriorate further. The most probable pathogenesis is occlusive vasculitis ...
متن کامل