Primary Sjogren syndrome with diffuse alveolar hemorrhage, cryoglobulinaemia and thrombotic microangiopathy

Vijaya Prasanna Parimi, Liza Rajasekhar

Pdf Page Numbers :- 26-27

 Vijaya Prasanna Parimi^1,* and Liza Rajasekhar²

¹Department of Rheumatology, Krishna Institute of Medical Sciences, Minister Road, Secunderabad-500003, Telangana, India

²Department of Rheumatology, Nizam’s Institute of Medical Sciences, Punjagutta, Hyderabad, Telangana 500082

*Corresponding author: Dr. P. Vijaya Prasanna, Consultant Rheumatologist, Department of Rheumatology, Krishna Institute of Medical Sciences, Minister Road, Secunderabad-500003, Telangana, India. Mobile: +91 9849145372; Email: prasanna.parimi.vijaya@gmail.com

Received 15 October 2015; Revised 3 December 2015; Accepted 16 December 2015; Published 25 December 2015

Citation: Parimi VP, Rajasekhar L. Primary Sjogren syndrome with diffuse alveolar hemorrhage, cryoglobulinaemia and thrombotic microangiopathy. J Med Sci Res. 2016; 4(1):26-27. DOI: http://dx.doi.org/10.17727/JMSR.2016/4-008

Copyright: © 2016 Parimi VP, et al. Published by KIMS Foundation and Research Center. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

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Abstract

 Sjogren syndrome is an autoimmune exocrinopathy with various extra glandular manifestations. Cryoglobulinemia, small vessel vasculitis has been reported in 5-10% of patients with primary Sjogren syndrome. It may be associated with a higher prevalence of extra glandular disease in Sjogren patients. Thrombotic microangiopathy (TMA), a potentially lethal condition characterized by microvascular thrombosis due to endothelial injury, is rare in SS. Its most characteristic histological finding is intravascular and intraglomerular fibrin thrombi. The association of Sjogren syndrome, cryoglobulinemia and thrombotic microangiopathy has not been previously reported. We report the first case of Sjogren syndrome with these three manifestations, occurring in combination.

Keywords: Sjogren syndrome; alveolar hemorrhage; cryoglobulinemia; thrombotic microangiopathy; pulmonary-renal syndrome