Acquired angioedema masquerading as abdominal and joint pain: c1 esterase deficiency secondary to cryptic lymphoma
A 62 year-old man presented to the emergency room with recurrent, colicky generalized abdominal pain. He had presented with similar abdominal pain in the preceding 6 weeks, requiring two hospital admissions. Admissions were accompanied by asymmetric lower limb swelling and pain on one admission, and swelling of lips on this admission. Six weeks prior, a computed tomography scan of the abdomen identified mesenteric lymph nodes which were biopsied and found to have CD5- positive cells consistent with chronic lymphocytic leukemia (CLL). The patient denied associated difficulty swallowing or breathing. He had no previous history of angioedema, food allergies or new medications. Vital signs were normal and physical examination was remarkable for symmetric upper and lower lip swelling without hives. Given lip swelling and recently diagnosed lymphoma, acquired angioedema was suspected. C1 esterase deficiency was confirmed by low functional C1 esterase inhibitor level, (4%, normal: > 40%), low C1 esterase inhibitor antigen (/dl, normal: 21 – 39 mg/dl), low C4 complement (< 1.7 mg/dl, normal: 12 – 38 mg/dl), and low normal C1q level (113ug/ml, normal: 109 – 242 ug/ml). Sedimentation rate, C-reactive protein, CBC and liver functions were normal. Patient was given one dose of intravenous Methylprednisolone 125 mg and antihistamines emergently. He was subsequently commenced on C1 esterase replacement, Icatibant (a bradykinin B2 receptor antagonist) and chemotherapy for lymphoma. He has had no further episodes of abdominal pain or lip swelling. An epinephrine pen was made available to him should angioedema recur
Angioedema is easily recognized clinically as facial or lip swelling, airway obstruction with or without hives. Lymphatic malignancies are a recognised cause of rare C1 esterase deficiency leading to acquired angioedema. Abdominal pain and extremity pain or swelling in isolation can be the only symptom of acquired angioedema.
Clinicians should be aware of angioedema presenting with abdominal and extremity pain/swelling without lip swelling. Older patients presenting with new angioedema should prompt a consideration of acquired C1 esterase deficiency with suspicion for underlying malignancy especially lymphoma, adenocarcinoma, monoclonal gammopathy of undetermined significance, and less commonly autoimmune diseases.
Journal of Hospital Medicine
Abdulkareem, A., Donato, A. A., Shogbesan, O., Mohanty, E., & Mundorff, J. (2017). Acquired angioedema masquerading as abdominal and joint pain: c1 esterase deficiency secondary to cryptic lymphoma. Journal of Hospital Medicine, 12 (Supplement 2), Abstract 318. Retrieved from https://scholarcommons.towerhealth.org/gme_int_med_resident_program_read/193