A 61 years old male patient not known to have any medical problem presented to the emergency department after he noticed swelling in his mouth which increased in size to involve both lips, he reported loose bowel movements in the last couple weeks and mild arthralgia. His physical examination was completely normal apart from severe angioedema at his lips. He used to smoke 5-6 cigarettes per day for 10 years, drinks alcohol occasionally, he denied any recent sexual activity or using any medications. Patient was started on iv antihistamine and steroids however his swelling started to gradually improve after two days. Laboratory workup showed normal complete blood count and electrolytes, total serum globulin was elevated however serum protein electrophoresis didn’t show any specific bands. ESR was markedly elevated (98MM/Hour) and cryoglobulinemia was positive. Hepatitis C PCR and HIV tests were negative, serum C4 and C3 were markedly decreased with level of 11.4 (normal 16-47mg/dl), 58 (normal=88-201) respectively. C1 esterase inhibitors level was normal and C1q which was done couple days after discharge was at low normal 5.6 (normal=5-8.6 mg/dl), functional assay for C1 esterase inhibitors was not done.
Discussion:
Angioedema is a disorder manifested by swelling of the subcutaneous tissue of non-gravitation areas in our bodies. It can occur in association with urticaria or as isolated phenomena. Mast cell degranulation secondary to allergen exposure is the presumed mechanism for the former while bradykinin pathway accounts for the latter. Isolated Angioedema when it occurs in an elderly patient without known precipitating event secondary causes should be sought particularly infectious, autoimmune and lymphoproliferative malignancy. We reported a case of elderly male patient who presented with angioedema. Workup reveal low complements 3 and 4 (C3, C4), low normal C1q level, elevated ESR, and cryoglobulin which favor the diagnosis of acquired angioedema (AAE).
Conclusions:
Angioedema is not an uncommon illness, however when it occurs for the first time in an elderly patient without clear precipitating event secondary causes should be looked for. Initial laboratory workup should start with C3 and C4 levels, if low then C1q and C1 inhibitor functional level should be done. The presence of low C1q level with 50 percent reduction in C1 inhibitor function confirm the diagnosis of AAE. We believe that C1q level was at the low normal range because it was done later in the patient’s presentation, on the other hand elevated ESR, positive cryoglobulin and markedly low complements on our case indicate the presence of autoantibodies that stimulate complement and bradykinin pathways which favor AAE. AAE secondary to cryoglobulinemia is rarely reported in the literature. Furthermore, Close follow up is essential which might uncover an autoimmune or malignant process.