Atypical Lymphoproliferative Disorder and Acquired Angioedema in Systemic Lupus Erythematosus and Sjögren's Syndrome: A Diagnostic Challenge.

Abstract

We report a case of a 33-year-old woman presenting with facial erythema, pharyngitis, and progressive cervical lymphadenopathy with tongue, pharyngeal, and laryngeal edema. Laboratory evaluations revealed an elevated soluble interleukin-2 receptor level, positive antinuclear, double-stranded DNA, and Sjögren's syndrome-related antigen A (SS-A) antibodies, and decreased complement levels. Salivary gland studies confirmed Sjögren's syndrome, and together with serological findings, the patient fulfilled criteria for systemic lupus erythematosus (SLE). Although Positron emission tomography-computed tomography (PET-CT) findings raised suspicion for malignant lymphoma, a cervical lymph node biopsy demonstrated interfollicular expansion, proliferation of small blood vessels with prominent endothelial cells, and large immunoblasts, consistent with atypical lymphoplasmacytic and immunoblastic proliferation (ALPIBP). Despite clinical evidence of angioedema on endoscopic examination, complement levels and C1-inhibitor activity were normal, suggesting a non-complement-mediated mechanism. The patient was initially treated with hydroxychloroquine for SLE; however, worsening symptoms required prednisolone therapy, leading to significant clinical improvement. Recurrence during steroid tapering was managed by adjusting the steroid dose and adding mycophenolate mofetil. This case emphasizes the importance of comprehensive evaluation to differentiate ALPIBP from malignant lymphoma and to recognize atypical acquired angioedema in autoimmune disease.