Recurrent Burkholderia gladioli Suppurative Lymphadenitis associated with Neutralizing Anti-IL-12p70 Autoantibodies

Abstract

IntroductionBurkholderia gladioli is a rare human pathogen, and pul-monary infections occur almost exclusively in cystic fibro-sis, chronic granulomatous disease and lung transplant pa-tients [1–3]. A single case of disseminated disease wasdescribed in a woman with anti-interferon gamma (IFNγ)autoantibodies [4]. Other opportunistic infections are com-mon with defects in the IFNγ/Interleukin-12 (IL-12) path-way [5]. While high-titer neutralizing anti-IL-12 autoanti-bodies can occur with thymoma, their association with in-fections has not been clearly established [6–8]. We report acase of severe B. gladioli infection associated with autoan-tibodies to IL-12p70 in a Southeast Asian woman withrecurring B. gladioli suppurative lymphadenitis.Case ReportA 45-year-old Cambodian woman presented with 2 monthsof progressive left-sided cervical lymphadenitis and tonsil-litis. Approximately 2 years earlier she had similar findingson the right side of her neck when excisional lymph nodebiopsy revealed B. gladioli. She received 3 weeks of oralantibiotics followed by 6 weeks of meropenem for pre-sumed melioidosis. Although she reported continued painand restricted neck motion, her acute infection appeared tohave resolved.As an adolescent in Cambodia, she had worked in ricepaddies before immigrating to the United States over30 years earlier. She had not been in Cambodia since thattime. She had intractable bloody diarrhea during her preg-nancy 13 years prior with large rectal granulomata.Postpartum, she developed perirectal and pelvic abscesseswith fistulae which required multiple surgeries and6monthsof oral antibiotics before resolution. The etiologic agent wasnever identified. Her daughter is healthy without recurrentinfections.She was a thin, chronically-ill appearing woman withbilateral tender, fluctuant cervical and submandibularlymphadenopathy and tonsillar swelling. Notable labs in-cluded a normocytic anemia, leukocytosis without elevatedband forms, and negative HIV serologies. There was noevidence of a monoclonal gammopathy on serum proteinelectrophoresis with immunofixation. Her cervical lymphnodes were massively enlarged and suppurative upon exci-sion, and histopathology revealed granulocytes and histio-cytes with intracellular gram-negative bacilli (Fig. 1) anddid not suggest any lymphoproliferative disease. B. gladioliwas isolated from all excised tissues and confirmed bycomparative 16S ribosomal RNA analysis by the Centersfor Disease Control and Prevention (CDC). Complement,superoxide production, immunoglobulin levels, and T cellquantitation were normal. An intradermal tuberculin test was