Autoimmune Thrombotic Thrombocytopenic Purpura: Two Rare Cases Associated with Juvenile Idiopathic Arthritis and Multiple Sclerosis

Abstract

Secondary thrombotic microangiopathies are associated with several underlying conditions, with most of them being resolved after the treatment of background disease. Thrombotic thrombocytopenic purpura (TTP) is a rare microangiopathy presenting with anemia, thrombocytopenia, and neurological deficits, occurring most often in various autoimmune diseases due to inhibition of ADAMTS13 by autoantibodies, as well as in pregnant women with or without an autoimmune substrate. In this article, we report two newly diagnosed TTP cases, who have not been published so far. The first is a 27-year-old woman with a history of polyarticular rheumatoid factor negative juvenile idiopathic arthritis, who presented with thrombocytopenia, anemia, schistocytes on blood smear, headache, and active arthritis. Originally she was treated successfully with plasma exchange, intravenous prednisone, and vincristine, and a few months after the TTP episode, she was commenced on rituximab, resulting in remission of primary disease and no relapse of TTP. The second case refers to a 29-year-old pregnant woman complaining of dizziness and fatigue with microangiopathic hemolytic anemia. She was treated with plasma exchanges, intravenous prednisolone, and INN human normal immunoglobulin with full remission of the TTP episode. Six and half years later, she was diagnosed with multiple sclerosis and was commenced on interferon beta-1 alpha, with no recurrent episode of TTP. These cases broaden the spectrum of autoimmune disorders manifested or complicated clinically by TTP. Furthermore, biological agents such as rituximab appear to be an effective treatment option for refractory cases of TTP related to systemic rheumatic disease, indicating an alternative therapeutic solution in persistent cases of this disorder.

Hemostasis biomarkers in multiple sclerosis

“…Lower levels of ADAMTS13 were found in MS, the deficiency of which is associated with microangiopathic hemolytic anemia . Acquired ADAMTS13 deficiency and thrombotic microangiopathy have been described in two MS patients treated with interferon‐beta .…”

Coagulation Pathways in Neurological Diseases: Multiple Sclerosis

“…Case reports of autoimmunity affecting hemostasis components in MS shold be considered in light of the acquired dysregulation of coagulation, with a focus on those components that are mainly targeted and that may contribute to clinical worsening. Interestingly, a few cases have been reported with TTP episodes (170) and acquired ADAMTS13 deficiency in the context of IFN-b treatment for MS (171, 172). Despite the limitation of their low number, these reports highlighted acquired deficiency induced by auto-antibodies against ADAMTS13.…”

Thrombotic Thrombocytopenic Purpura: Revisiting a Miss and an Inevitable Consequence

“…It can be categorized as congenital or acquired, and many cases are idiopathic. However, many case reports have suggested an association with autoimmune diseases [9], infection [10], pregnancy [11], bone marrow transplantation [12], and certain drugs such as cyclosporine A, tacrolimus, mitomycin, or ticlopidine [13], which were not suggested in this patient's history. Also, in the literature, atypical TTP has been described in patients presenting with strokes or myocardial infarction with normal or near-normal laboratory values [14,15].…”