Literature DB >> 24551698

Systemic Sclerosis Presenting with Simultaneous Retinal Vasculitis in One Eye and Optic Neuritis in the other along with Severe Immune Haemolytic Anaemia.

Avijit Moulick1, Biswanath Sharma Sarkar1, Anirban Jana2, Pradipta Guha1, Anjan Das3.   

Abstract

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.

Entities:  

Keywords:  Autoimmune haemolytic anaemia; Optic neuritis; Retinal vasculitis; Systemic sclerosis

Year:  2013        PMID: 24551698      PMCID: PMC3919342          DOI: 10.7860/JCDR/2013/6699.3816

Source DB:  PubMed          Journal:  J Clin Diagn Res        ISSN: 0973-709X


  4 in total

1.  A case of systemic sclerosis complicated by autoimmune hemolytic anemia.

Authors:  Kazuaki Katsumata
Journal:  Mod Rheumatol       Date:  2006       Impact factor: 3.023

2.  Anemia and scleroderma: frequency, causes, and marrow findings.

Authors:  M P Westerman; R C Martinez; T A Medsger; R S Totten; G P Rodnan
Journal:  Arch Intern Med       Date:  1968-07

3.  Neurologic manifstations in progressive systemic sclerosis.

Authors:  R M Gordon; A Silverstein
Journal:  Arch Neurol       Date:  1970-02

4.  Bilateral optic neuropathy associated with the crest variant of scleroderma.

Authors:  A Boschi; B Snyers; M Lambert
Journal:  Eur J Ophthalmol       Date:  1993 Oct-Dec       Impact factor: 1.922

  4 in total
  1 in total

Review 1.  Autoimmunity in visual loss.

Authors:  Axel Petzold; Sui Wong; Gordon T Plant
Journal:  Handb Clin Neurol       Date:  2016
  1 in total

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