Late Presentation of SLE and Secondary Anti-phospholipid Syndrome, Following Initial Evans Syndrome

Geetha Wickrematilake^1,

¹Department of Rheumatology, District General Hospital, Nuwara Eliya, Sri Lanka

Cite this paper

Geetha Wickrematilake. Late Presentation of SLE and Secondary Anti-phospholipid Syndrome, Following Initial Evans Syndrome. American Journal of Medical Case Reports. 2017; 5(9):252-253. doi: 10.12691/AJMCR-5-9-7

Abstract

Background: Evans syndrome associated with secondary anti-phospholipid syndrome has not been reported in case studies and we report a case of SLE and secondary anti-phospholipid syndrome diagnosed seven years after the initial diagnosis of Evans syndrome. Case presentation: A 21-years-old female was diagnosed with Evans syndrome since the age of 14 years, and was on treatment for seven years. At the onset of disease her immunological screening for ANA, Ds-DNA were negative. She was treated with immunosuppressants and steroids and the disease was complicated with Cryptococcal meningitis. Seven years later at the age of 21 years she started to have joint arthritis, and the ANA test converted to positive together with a positive Ds-DNA. Later on she had a single episode of unprovoked deep vein thrombosis in a leg (below knee), and became positive for lupus anticoagulant and IgM anticardiolipin antibodies. Her cardiac imaging (Echo-cardiogram) revealed that she had developed moderate pulmonary hypertension. She was treated with physiotherapy, Methotrexate, Hydroxychloroquine, Warfarin and Sildenafil with good control of the disease. Her lupus is in remission with treatment with no anaemia. Conclusion: Haematological manifestations of systemic lupus erythematosus could vary. Hence, Lupus should be kept in mind always during management of complicated hematological conditions as the patient might fulfill cumulative SLE classification criteria over time.

Keywords

systemic lupus erythematosus, anti-phospholipid syndrome, Evans syndrome, anti-nuclear antibody, haemolytic anaemia

Copyright

This work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

References

[1]	Dhingra KK, Jain D, Mandal S, et al. Evans syndrome: a study of six cases with review of literature. Hematology 2008; 13: 356-360.
[2]	Savasan S, Warrier I, Ravindranath Y. The spectrum of Evans’ syndrome. Arch Dis Child 1997; 77: 245-8
[3]	Savasan S, Warrier I, Ravindranath Y. The Spectrum of Evan’s syndrome. Arch Dis Child. 1997; 77(3): 245-8.
[4]	Karpatkin S. Autoimmune thrombocytopenic purpura. Blood 1980; 56: 329-343.
[5]	Michel M, Chanet V, Dechartres A. The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases. Blood 2009 114: 3167-3172.
[6]	Costallat GL, Appenzeller S, Costallat LT. Evans syndrome and systemic lupus erythematosus: clinical presentation and outcome. Joint Bone Spine. 2012 Jul; 79(4): 362-4.
[7]	Asherson RA. Antiphospholipid antibodies and syndromes. In: Lahita RG, ed. Systemic lupus erythematosus. 2 nd ed. New York: Churchill Livingstone; 1992. p. 612-3.
[8]	Kumar S, Benseler SM, Kirby-Allen M, et al. B-cell depletion for autoimmune thrombocytopenia and autoimmune hemolytic anemia in pediatric systemic lupus erythematosus. Pediatrics 2009; 123: e159-63.