Abstract Background/Aims Autosplenectomy, defined as spontaneous splenic atrophy in the absence of trauma or surgical intervention, is a rare phenomenon most frequently associated with sickle cell disease. In autoimmune disorders such as systemic lupus erythematosus (SLE) or Sjögren’s syndrome, complete anatomical autosplenectomy is exceptionally uncommon, with only isolated case reports documented. The pathophysiology is presumed to involve chronic immune-mediated vasculopathy, immune complex deposition, and microvascular thrombosis, leading to progressive infarction and fibrosis of splenic tissue. Recognition of this entity has important clinical implications, particularly in preventing life-threatening infections related to asplenia. Methods A 54-year-old female with known Sjögren’s syndrome, previously off hydroxychloroquine, presented with worsening polyarthralgia, fatigue, and systemic inflammation. Examination revealed synovitis and peripheral oedema. Laboratory evaluation demonstrated anaemia, elevated inflammatory markers, low complements (C3/C4), and high dsDNA titres consistent with an SLE flare. Autoantibody profile was positive for ANA, ENA, anti-Ro, anti-La, and anti-RNP/Sm. Blood cultures grew Staphylococcus aureus but echocardiography excluded endocarditis. Extensive infectious, haematological, and malignant causes of systemic inflammation were excluded. A CT thorax, abdomen, and pelvis (CT TAP) revealed complete absence of splenic tissue, despite prior scans confirming a normal spleen. Further imaging showed mesenteric panniculitis and reactive lymphadenopathy, with lymph node biopsy excluding malignancy. There was no surgical history, trauma, or evidence of sickle cell disease or other haemoglobinopathy. Results Comprehensive evaluation ruled out infiltrative (amyloidosis, sarcoidosis), infectious (tuberculosis), and malignant (lymphoma) causes of splenic atrophy. Serial imaging confirmed prior normal splenic anatomy followed by complete disappearance of splenic tissue, strongly suggesting autoimmune-mediated autosplenectomy. The presumed mechanism involved chronic immune complex deposition, vasculopathy, and microinfarction resulting in fibrosis and atrophy. The patient was diagnosed with SLE/Sjögren’s overlap with autoimmune-mediated autosplenectomy and commenced on immunosuppressive therapy. Post-splenectomy vaccination and antibiotic prophylaxis were initiated. Despite disease control, she continued to experience fatigue and pain requiring supportive management. Conclusion This case represents a rare example of autoimmune-mediated autosplenectomy in SLE/Sjögren’s overlap syndrome. It reinforces the potential for chronic autoimmune vasculopathy to cause progressive splenic destruction, a phenomenon more commonly associated with sickle cell disease. Recognition of anatomical or functional asplenia in autoimmune patients is crucial, as it carries a significant risk of overwhelming infection. Clinicians should maintain a high index of suspicion, review previous imaging where possible, and ensure timely prophylaxis and patient education. Our case adds to the limited literature on autoimmune-driven splenic atrophy and underscores the need for vigilance in monitoring splenic function in systemic autoimmune diseases. Disclosure K. Shahzad: None. F.A. Khan: Honoraria; UCB. Other; EULAR Congress Sponsorship (UCB), BSR 2025 (Novartis). Z. Asghar: None. M. Babiker: None.
Shahzad et al. (2026) studied this question.