Rare Instance of Simultaneous SLE, TTP Presentation Described in New Case Report

Investigators said they were able to confirm diagnoses of systemic lupus erythematosus (SLE) and thrombotic thrombocytopenic purpura (TTP) because they took a multidisciplinary approach.

Thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE) share overlapping clinical features, but they rarely arise simultaneously.

In a new case report in Thrombosis Update, investigators described their experience treating a 40-year-old woman who came to the hospital and eventually received both diagnoses.

The patient presented with perioral numbness over 3 days and a history of severe frontal headaches. She did not have any fever, rashes, nausea, or chest pain; however, there was an episode of transient aphasia. A CT examination of her head revealed no intracranial abnormalities.

A peripheral blood smear showed evidence of schistocytes, and the patient was given a direct Coombs test (which was negative) and an a disintegrin and metalloproteinase with thrombospondin type 1 motif, 13 (ADAMTS13) tests. While waiting for the ADAMTS13 results, the investigators calculated a PLASMIC score of 7, suggesting a high chance of severe ADAMTS13 deficiency.

The patient was started on methylprednisolone 60 mg intravenously twice daily and given a catheter for plasma exchange. Rheumatologic bloodwork was performed and a subsequent consult with the Rheumatology Department confirmed SLE.

The authors said plasma exchange was difficult for the patient due to intermittent hypotension and nausea. However, they were able to complete 5 sessions, which stabilized the patient’s platelets. Meanwhile, the ADAMTS13 tests came back and showed the patient’s ADAMTS13 activity was less than 5%, which confirmed their diagnosis of acquired TTP.

The patient was discharged and received 3 weeks of Rituxan (rituximab) infusions, 2 subsequent plasma exchange sessions, and daily prednisone on a slow taper. Six months later, the patient had ulcerations of the bilateral mucosae, occasional migraines, and dryness in the corners of her eyes. However, she did not have Raynaud’s phenomenon, chest pain, shortness of breath, joint swelling, or morning stiffness. The patient was prescribed hydroxychloroquine for her SLE and her TTP was in remission, the authors said.

Onechallenge to diagnosing TTP is its rarity, the investigators said. It also has symptoms similar to catastrophic antiphospholipid syndrome, but the investigators ruled that out because a lupus anticoagulant test was negative.

The confirmation of TTP and SLE was confirmed because although some of the overlapping symptoms of the conditions resolved with TTP treatment, oral ulcers persisted and the patient still met the immunological criteria for SLE. The authors said plasma exchange therapy has led to a dramatic improvement in mortality rates for TTP.

“The goal is to exchange circulating anti-ADAMTS13 for fresh ADAMTS13,” they wrote. In patients with SLE, they said it is particularly important to administer steroids prior to plasma therapy, and they said rituximab can provide additional benefits to patients, as it appeared to do in this case.

The investigators concluded by underscoring the importance of a multidisciplinary approach.

“It is important to emphasize that patients with similar presentations will need a multidisciplinary approach to their care as well as close monitoring to distinguish further flares from SLE-related complications such as catastrophic antiphospholipid syndrome vs TTP, which are managed similarly, but with important caveats related to anticoagulation, duration of steroids, and plasma exchange,” they wrote.


D. Uczkowski, H. Milonas. Initial presentation of systemic lupus erythematosus coinciding with thrombotic thrombocytopenic purpura. Thromb Update. 2022;8. doi: doi:10.1016/j.tru.2022.100118

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