From the Research
There is no established association between positive Anti-SS-A (Sjögren's syndrome antigen A) antibodies and Thrombotic Thrombocytopenic Purpura (TTP). TTP is a rare blood disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ dysfunction due to microvascular thrombosis, typically associated with ADAMTS13 deficiency, either acquired through autoantibodies against ADAMTS13 or through genetic mutations 1.
The pathophysiological mechanisms of TTP and autoimmune conditions associated with SS-A antibodies, such as Sjögren's syndrome and systemic lupus erythematosus, differ significantly. SS-A antibodies target intracellular ribonucleoproteins and are not directly involved in the platelet aggregation and microvascular thrombosis seen in TTP.
Some studies have explored the relationship between autoimmune disorders and thrombotic events, including the presence of antiphospholipid antibodies as a risk factor for thrombotic events in patients with connective tissue diseases and idiopathic thrombocytopenic purpura 2. However, these findings do not establish a direct link between SS-A antibodies and TTP.
If TTP is suspected clinically, appropriate testing should focus on ADAMTS13 activity and inhibitor levels rather than SS-A antibody status. This approach is supported by studies highlighting the importance of ADAMTS13 in diagnosing and managing TTP 3. Patients with autoimmune disorders who have SS-A antibodies may develop various hematological abnormalities, but TTP specifically is not considered a manifestation of SS-A positivity.
In summary, the current evidence does not support an association between positive Anti-SS-A antibodies and TTP, and clinical evaluation should prioritize ADAMTS13 testing over SS-A antibody status when suspecting TTP.