Abstract Background/Aims We present a case of a 54-year-old female who developed autosplenectomy secondary to systemic lupus erythematosus (SLE). The patient had a historical diagnosis of Sjögren’s syndrome (anti-Ro +, anti-La +, ANA 1/640) and was previously stable. This dramatically seroconverted into SLE after contracting a viral illness. She presented with florid synovitis, pericardial chest pain, proteinuria (with preserved renal function) and low albumin, requiring hospital admission. Her bloods showed the following: dsDNA 176 (previously normal), C3 0.4, C4 0.03, CRP 169. An extensive infection screen was negative, including a transoesophageal echo, and CTCAP. An enlarged axillary lymph node was identified on CT, which was biopsied to exclude lymphoma, given the history of Sjögren’s. She was initially treated with IV methylprednisolone, and after developing vasculitic lesions on her peripheries, she received rituximab and cyclophosphamide. She then developed bilateral pulmonary embolisms and was anticoagulated with warfarin. Her antiphospholipid syndrome (APS) screen was negative before and after developing the emboli. The CTCAP showed an absent spleen; however, this was present and normal on ultrasound 14 years prior. Therefore, our patient required long-term prophylactic phenoxymethylpenicillin, as well as prophylactic co-trimoxazole, routinely given with her chemotherapy. Methods Autosplenectomy in adults is a rare, recognised phenomenon of SLE and there are only a handful of cases in the literature. It is thought to be due to spontaneous splenic infarction secondary to an underlying vasculitis. Cells seen in the peripheral blood film include target cells, spherocytes and Howell-Jolly bodies. These patients are vulnerable to infection, particularly encapsulated bacteria such as streptococcal pneumonia. Case reports highlight the risk of pneumococcal infection and several patients have presented with pneumococcal sepsis, who were subsequently found to have autosplenectomy secondary to SLE. Patients with known autosplenectomy should all be offered prophylactic antibiotics to reduce the risk of infection. Introducing a pneumococcal vaccine as standard treatment for the management of SLE is something to consider, to prevent cases of pneumococcal sepsis developing where autosplenectomy has not been detected. Results Several case reports link autosplenectomy with coexistent APS or positive antiphospholipid-associated antibodies, and this phenomenon may make splenic infarction more likely. Literature describes a link between autosplenectomy and thrombocytosis in SLE. Classically, thrombocytopenia would be expected, but if thrombocytosis is noted, autosplenectomy should be considered as a complication. This was the case in our patient, as her platelets were 500-700 whilst she was unwell. Conclusion This case highlights an important but rare complication of SLE. Thrombocytosis should be a trigger for considering autosplenectomy, with the overall aim of recognising the development of this condition before fulminant, life-threatening sepsis develops. Once autosplenectomy has been diagnosed, coexistent APS should be considered, as well as antibiotic prophylaxis. Disclosure L. Maltas: None. O. Jones: None.
Maltas et al. (2026) studied this question.