The cooccurrence of immune thrombocytopenia (ITP) and systemic sclerosis (SSc) is very rare and poses challenges to disease management, according to 2 cases presented in the journal Joint Bone Spine.

Clinical management of the first patient was extremely difficult, with platelet levels remaining extremely low (2×109/L) even after treatment with corticosteroids, intravenous immunoglobulins (IVIg), rituximab, and romiplostim. The second case also had low platelet counts (8×109/L) resistant to treatment with IVIg and corticosteroids. Both patients were diagnosed with ITP and diffuse subcutaneous SSc, while patient 2 also had antitopoisomerase antibodies.

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Patient 1, a 29-year-old female with a history of SSc, was admitted to the hospital due to epistaxis and rectal bleeding with a platelet count of 4×109/L. Through testing, ITP was diagnosed, and the patient was started on high-dose pulse methylprednisolone and IVIg. She did not respond to treatment, and her platelet count dropped further to 2×109/L. Rituximab and romiplostim treatment was then initiated, but at 14 days, the patient’s platelet count was too low to count, and she had developed severe headache, nausea, and vomiting.

An acute subdural hematoma with signs of brain herniation was detected, and the patient began platelet transfusions. Despite the transfusions, platelet counts remained low, so the patient underwent an emergency splenectomy followed by subcutaneous romiplostim treatment. Her platelet counts continued to climb, and she was discharged 2 weeks later with a platelet count of 123×109/L. Romiplostim was discontinued 1 month after hospital discharge, and the patient had not experienced recurrence after 4 years of follow-up.

The second patient, a 66-year-old female with a 13-year history of severe diffuse cutaneous SSc combined with arthritis mutilans of the hands, was admitted to the hospital due to mild epistaxis and a low platelet count of 8×109/L. A diagnosis of severe ITP was made following testing, and she was started on IVIg without corticosteroids due to concern over the risk of scleroderma renal crisis (SRC).

Platelet counts increased after 3 days of IVIg but subsequently decreased again, so IVIg was resumed but with the addition of rituximab. The platelet count continued to drop despite platelet transfusions, so high-dose dexamethasone in combination with romiplostim, hydroxychloroquine, and IVIg transfusions were added to the treatment regimen.

Two weeks after the first infusion, rituximab was added to the treatment. Platelet counts had increased to 337×109/L 60 days after hospital discharge, and romiplostim was eventually tapered and stopped after 3 months. No recurrence had been seen as of 6 months after discharge.

“Despite the clinical heterogeneity of the 2 cases, our observations suggest that severe ITP can occur in SSc patients and that combination therapy with corticosteroids, IVIg, rituximab, and [thrombopoeitin receptor agonists] may be used to reverse persistent thrombocytopenia. Splenectomy might be considered in patients with acute life-threatening bleeding,” the authors wrote.


Boleto G, Avouac J, Godeau B, Allanore Y. Severe immune thrombocytopenia in two patients with systemic sclerosis. Joint Bone Spine. Published online March 31, 2023. doi:10.1016/j.jbspin.2023.105560