Immune-Mediated Etiology of Acquired von Willebrand Syndrome in Systemic Lupus Erythematosus and in Benign Monoclonal Gammopathy: Therapeutic Implications

Acquired von Willebrand syndrome (AVWS) is an uncommon, underdiagnosed, and heterogeneous disease which is increasingly recognized as a cause of bleeding diatheses. Systemic lupus erythematosus (SLE) is an infrequent cause of AVWS. Herein, we report a case of AVWS diagnosed during the initial presentation of SLE in a previously healthy young man with no family history of bleeding diathesis who presented with worsening epistaxis, gastrointestinal bleeding, and anasarca. He was found to have severe anemia and prolonged activated partial thromboplastin time (aPTT) with severely decreased levels of von Willebrand factor (VWF) measurements in addition to markedly decreased factor VIII levels. Further evaluation revealed nephrotic syndrome and interstitial lung disease due to SLE. He initially received combination therapy with intravenous immunoglobulin (IVIG) and von Willebrand factor/factor VIII concentrates without significant improvement. Treatment with steroids, cyclophosphamide, and rituximab was followed by clinical improvement evidenced by cessation of bleeding. The short follow-up did not allow us to definitely prove the therapeutic effect of immunosuppressive treatment on AVWS in SLE patients. This case adds to the literature supporting the relationship between AVWS and SLE and highlights the importance of combination therapy in the treatment of severe AVWS as well as the role of IVIG, cyclophosphamide, and rituximab in AVWS associated with SLE.

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An atypical manifestation of acquired von Willebrand syndrome (AVWS) associated with systemic lupus erythematosus (SLE)

Annals of Hematology ◽

10.1007/s00277-013-1767-4 ◽

2013 ◽

Vol 93 (1) ◽

pp. 173-175 ◽

Cited By ~ 6

Author(s):

Priyanka Kasatkar ◽

Kanjaksha Ghosh ◽

Shrimati Shetty

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Systemic Lupus ◽

Acquired Von Willebrand Syndrome ◽

Von Willebrand ◽

Atypical Manifestation

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Faculty Opinions recommendation of Novel therapies for immune-mediated inflammatory diseases: What can we learn from their use in rheumatoid arthritis, spondyloarthritis, systemic lupus erythematosus, psoriasis, Crohn's disease and ulcerative colitis?

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.727869572.793564281 ◽

2019 ◽

Author(s):

Ladislav Senolt

Keyword(s):

Rheumatoid Arthritis ◽

Systemic Lupus Erythematosus ◽

Ulcerative Colitis ◽

Crohn’S Disease ◽

Crohn's Disease ◽

Lupus Erythematosus ◽

Inflammatory Diseases ◽

Novel Therapies ◽

Systemic Lupus ◽

Immune Mediated

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Dual threat of comorbidity of celiac disease and systemic lupus erythematosus

Journal of International Medical Research ◽

10.1177/03000605211012258 ◽

2021 ◽

Vol 49 (5) ◽

pp. 030006052110122

Author(s):

Yimin Ma ◽

Duanming Zhuang ◽

Zhenguo Qiao

Keyword(s):

Systemic Lupus Erythematosus ◽

Celiac Disease ◽

Lupus Erythematosus ◽

Clinical Manifestations ◽

Failure To Thrive ◽

Severe Malnutrition ◽

Systemic Lupus ◽

Electrolyte Disorders ◽

Diagnostic Challenge ◽

Immune Mediated

Celiac disease (CD) is a chronic immune-mediated intestinal disease that is characterized by production of autoantibodies directed against the small intestine. The main clinical manifestations of CD are typically defined as those related to indigestion and malabsorption. These manifestations include unexplained diarrhea or constipation, abdominal pain, bloating, weight loss, anemia, failure-to-thrive in children, and decreased bone density. Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by heterogeneous clinical manifestations, which may also involve the gastrointestinal tract. Comorbidity of CD and SLE is rare, and the overlapping symptoms and nonspecific clinical presentation may pose a diagnostic challenge to clinicians. We report here a case of SLE with CD, which mainly manifested as recurrent diarrhea, uncorrectable electrolyte disorders, and severe malnutrition. Through review, we hope to further improve our understanding and diagnostic level of this combination of diseases.

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