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P237 IGG4 Related disease- a unique case mimicking chronic rhinosinusitis with polyposis
Abstracts: Poster Sessions / Ann Allergy Asthma Immunol 117 (2016) S22eS124
P237 IGG4 RELATED DISEASE- A UNIQUE CASE MIMICKING CHRONIC RHINOSINUSITIS WITH POLYPOSIS G. Rosner*, L. Fonacier, S. Salzman, Mineola, NY. Background: IgG4-related disease (IgG4-RD) has emerged as a unique immune-mediated condition that links multiple fibroinflammatory disorders. The common features include tumor-like swelling of involved organs, a lymphoplasmacytic infiltrate enriched in IgG4-positive plasma cells, and a variable degree of fibrosis with a characteristic “storiform” pattern. The organs most frequently involved are pancreas, biliary tract, salivary and lacrimal glands. Case report: A 46-year-old female with recurrent sinus and ear infections, severe asthma and allergic rhinitis not responsive to Immunotherapy (IT) was referred for immune workup. She has failed to improve despite IT, oral antihistamines, intranasal steroids and leukotriene inhibitors. Within the last 20 years the patient had undergone 4 separate sinus surgeries plus 3 nasal polypectomies. Labwork showed a markedly elevated IgG4 level of 319 mg/dL(nl 486), total IgE level of 237mg/dL and an absolute eosinophil count of 696 cells/mcL. She mounted protective titers to Streptococcus pneumoniae and Haemophilus influenza type b. Allergic bronchopulmonary aspergillosis testing was negative. Pulmonary function testing revealed moderately severe obstruction. Biopsy of a nasal polyp revealed many eosinophils and plasma cells and an IgG/IgG4 ratio of 100%. Discussion: IgG4-RD is an increasingly recognized syndrome of unknown etiology. Previous case reports have discussed sinonasal involvement in patients with IgG4-RD, but to our knowledge this is the first report of IgG4-RD manifesting as nasal polyposis. Practitioners should consider this diagnosis in patients presenting with recalcitrant chronic rhinosinusitis with polyposis.
P238 IGG4-RELATED DISEASE: CAN WE MAKE A DIAGNOSIS WITHOUT FIBROSIS? R. Schapira*1, J. Hall2, R. Rose2, J. Freiler1, 1. San Antonio, TX; 2. Fort Sam Houston, TX. Introduction: IgG4-related disease (IgG4-RD) is an increasingly reported immune-mediated condition characterized by deposition of lymphoplasmacytic infiltrates rich in IgG4+ plasma cells accompanied with tumefactive lesions of involved organs and storiform fibrosis. Clinicians should be aware that IgG4-RD can involve almost any organ and may mimic other conditions. We propose a screening protocol in patients with asymptomatic lymphadenopathy showing progressively transformed germinal centers (PTGC) and increased IgG4+ plasma cells. Methods: An asymptomatic 59-year-old female with history of breast cancer in remission underwent routine screening mammography which revealed unilateral axillary lymphadenopathy. Results: Excisional lymph node biopsy revealed follicular hyperplasia with PTGC and increased intrafollicular plasma cells. Immunohistochemistry demonstrated germinal centers with increased numbers of IgG4+ plasma cells and >40% IgG4+ among the total IgG+ plasma cells. Ancillary studies for clonality were negative. She was referred to our clinic for consideration of possible IgG4-RD. History, physical and laboratory screening for conditions that have been ascribed to IgG4-RD, which consisted of CBC, CMP, UA, C3/C4, Ig subclasses, CRP and TSH, were unrevealing. Conclusion: Initial evaluation of asymptomatic lymphadenopathy with PTGC and increased IgG4+ plasma cells should include a thorough history, physical and selected laboratory studies. As the histologic characteristics of lymph nodes in IgG4-RD are not
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specific watchful waiting seems prudent for those with a low index of suspicion for this disorder. As the long-term prognosis is not known continued surveillance was recommended. For patients with concerning features further diagnostic examination should be considered.
P239 ACUTE CARDIAC DISEASE IN A PATIENT WITH HYPER IGE SYNDROME (HIES) M. Nassiri*1, A. Castilano2, H. Watti*3, A. Abdulbaki3, S. Bahna3, 1. Bossier City, LA; 2. Shreveport LA, LA; 3. Shreveport, LA. Introduction: Immunodeficiency diseases are often complicated with a variety of comorbid infections or malignancy, but rarely heart disease. Case: A 25-y-o male with typical HIES with STAT-3 mutation had recurrent skin and lung fungal and bacterial infections on prophylactic voriconazole and trimethoprim-sulfa alternating with doxycycline. He presented to the ED with acute onset dyspnea, chest pain, and diffuse ST segment elevation. Troponin was very high (6.32 ng/mL). Two- dimensional echocardiogram showed new onset heart failure with ejection fraction 20-25% and global hypokinesis. Biopsy showed lymphocytic myocarditis. Viral culture was negative. After treatment of heart failure, he was discharged on a 3-wk prednisone taper as well as a defibrillator life vest due to risk of sudden cardiac arrest. Five weeks later, he returned to the ED with palpitations and lightheadedness. He was found to have atrial tachycardia and unchanged cardiac function. Empirically, IVIG (400 mg/kg) was given in addition to standard heart failure therapy. Prednisone and azathioprine were not considered to avoid further immunosuppression. Monthly IVIG was planned and as of to date, he received two doses but so far had no improvement in cardiac function. Conclusion: To the best of our knowledge, this is the first reported case of lymphocytic myocarditis in a patient with HIES. Because of the risk of immunosuppressive therapy, an empiric trial of IVIG is attempted.
P240 POLYSACCHARIDE-SPECIFIC ANTIBODY DEFICIENCY WITH INTACT RECALL OF PNEUMOCOCCAL CONJUGATE VACCINE ANTIGENS BY POLYSACCHARIDE ANTIGEN A. Al-Kaabi*, J. Orange, Houston, TX. Introduction: Responses to pneumococcal polysaccharide vaccine (PPV23) are routinely used to evaluate patients with suspected specific antibody deficiency. According to the 2015 practice parameter, the degree of responsiveness classifies the patient into one of four phenotypes. This system does not consider if the protective titers are due to intact recall to previously administered conjugated polysaccharide vaccine (PCV13). This could incorrectly label the degree of non-responsiveness as mild when it is actually severe. Methods: A pediatric patient with recurrent infection having received all recommended scheduled vaccinations was evaluated by measuring pneumococcal titers pre and post PPV23. Levels were repeated after five months and subsequently quantified after PCV13 re-administration. Results: All prevaccine titers were non-protective. Post PPV23, 13 titers boosted impressively classifying the patient as having “mild” deficient response phenotype. Repeat testing within 6 months demonstrated notable decreases in titers, which may classify the patient as “memory” phenotype. Out of the 13 responsive titers, 12 are found in PCV13. Thus, PCV13 was administered resulting in a major boost in the corresponding titers.
P237 IGG4 RELATED DISEASE- A UNIQUE CASE MIMICKING CHRONIC RHINOSINUSITIS WITH POLYPOSIS G. Rosner*, L. Fonacier, S. Salzman, Mineola, NY. Background: IgG4-related disease (IgG4-RD) has emerged as a unique immune-mediated condition that links multiple fibroinflammatory disorders. The common features include tumor-like swelling of involved organs, a lymphoplasmacytic infiltrate enriched in IgG4-positive plasma cells, and a variable degree of fibrosis with a characteristic “storiform” pattern. The organs most frequently involved are pancreas, biliary tract, salivary and lacrimal glands. Case report: A 46-year-old female with recurrent sinus and ear infections, severe asthma and allergic rhinitis not responsive to Immunotherapy (IT) was referred for immune workup. She has failed to improve despite IT, oral antihistamines, intranasal steroids and leukotriene inhibitors. Within the last 20 years the patient had undergone 4 separate sinus surgeries plus 3 nasal polypectomies. Labwork showed a markedly elevated IgG4 level of 319 mg/dL(nl 486), total IgE level of 237mg/dL and an absolute eosinophil count of 696 cells/mcL. She mounted protective titers to Streptococcus pneumoniae and Haemophilus influenza type b. Allergic bronchopulmonary aspergillosis testing was negative. Pulmonary function testing revealed moderately severe obstruction. Biopsy of a nasal polyp revealed many eosinophils and plasma cells and an IgG/IgG4 ratio of 100%. Discussion: IgG4-RD is an increasingly recognized syndrome of unknown etiology. Previous case reports have discussed sinonasal involvement in patients with IgG4-RD, but to our knowledge this is the first report of IgG4-RD manifesting as nasal polyposis. Practitioners should consider this diagnosis in patients presenting with recalcitrant chronic rhinosinusitis with polyposis.
P238 IGG4-RELATED DISEASE: CAN WE MAKE A DIAGNOSIS WITHOUT FIBROSIS? R. Schapira*1, J. Hall2, R. Rose2, J. Freiler1, 1. San Antonio, TX; 2. Fort Sam Houston, TX. Introduction: IgG4-related disease (IgG4-RD) is an increasingly reported immune-mediated condition characterized by deposition of lymphoplasmacytic infiltrates rich in IgG4+ plasma cells accompanied with tumefactive lesions of involved organs and storiform fibrosis. Clinicians should be aware that IgG4-RD can involve almost any organ and may mimic other conditions. We propose a screening protocol in patients with asymptomatic lymphadenopathy showing progressively transformed germinal centers (PTGC) and increased IgG4+ plasma cells. Methods: An asymptomatic 59-year-old female with history of breast cancer in remission underwent routine screening mammography which revealed unilateral axillary lymphadenopathy. Results: Excisional lymph node biopsy revealed follicular hyperplasia with PTGC and increased intrafollicular plasma cells. Immunohistochemistry demonstrated germinal centers with increased numbers of IgG4+ plasma cells and >40% IgG4+ among the total IgG+ plasma cells. Ancillary studies for clonality were negative. She was referred to our clinic for consideration of possible IgG4-RD. History, physical and laboratory screening for conditions that have been ascribed to IgG4-RD, which consisted of CBC, CMP, UA, C3/C4, Ig subclasses, CRP and TSH, were unrevealing. Conclusion: Initial evaluation of asymptomatic lymphadenopathy with PTGC and increased IgG4+ plasma cells should include a thorough history, physical and selected laboratory studies. As the histologic characteristics of lymph nodes in IgG4-RD are not
S93
specific watchful waiting seems prudent for those with a low index of suspicion for this disorder. As the long-term prognosis is not known continued surveillance was recommended. For patients with concerning features further diagnostic examination should be considered.
P239 ACUTE CARDIAC DISEASE IN A PATIENT WITH HYPER IGE SYNDROME (HIES) M. Nassiri*1, A. Castilano2, H. Watti*3, A. Abdulbaki3, S. Bahna3, 1. Bossier City, LA; 2. Shreveport LA, LA; 3. Shreveport, LA. Introduction: Immunodeficiency diseases are often complicated with a variety of comorbid infections or malignancy, but rarely heart disease. Case: A 25-y-o male with typical HIES with STAT-3 mutation had recurrent skin and lung fungal and bacterial infections on prophylactic voriconazole and trimethoprim-sulfa alternating with doxycycline. He presented to the ED with acute onset dyspnea, chest pain, and diffuse ST segment elevation. Troponin was very high (6.32 ng/mL). Two- dimensional echocardiogram showed new onset heart failure with ejection fraction 20-25% and global hypokinesis. Biopsy showed lymphocytic myocarditis. Viral culture was negative. After treatment of heart failure, he was discharged on a 3-wk prednisone taper as well as a defibrillator life vest due to risk of sudden cardiac arrest. Five weeks later, he returned to the ED with palpitations and lightheadedness. He was found to have atrial tachycardia and unchanged cardiac function. Empirically, IVIG (400 mg/kg) was given in addition to standard heart failure therapy. Prednisone and azathioprine were not considered to avoid further immunosuppression. Monthly IVIG was planned and as of to date, he received two doses but so far had no improvement in cardiac function. Conclusion: To the best of our knowledge, this is the first reported case of lymphocytic myocarditis in a patient with HIES. Because of the risk of immunosuppressive therapy, an empiric trial of IVIG is attempted.
P240 POLYSACCHARIDE-SPECIFIC ANTIBODY DEFICIENCY WITH INTACT RECALL OF PNEUMOCOCCAL CONJUGATE VACCINE ANTIGENS BY POLYSACCHARIDE ANTIGEN A. Al-Kaabi*, J. Orange, Houston, TX. Introduction: Responses to pneumococcal polysaccharide vaccine (PPV23) are routinely used to evaluate patients with suspected specific antibody deficiency. According to the 2015 practice parameter, the degree of responsiveness classifies the patient into one of four phenotypes. This system does not consider if the protective titers are due to intact recall to previously administered conjugated polysaccharide vaccine (PCV13). This could incorrectly label the degree of non-responsiveness as mild when it is actually severe. Methods: A pediatric patient with recurrent infection having received all recommended scheduled vaccinations was evaluated by measuring pneumococcal titers pre and post PPV23. Levels were repeated after five months and subsequently quantified after PCV13 re-administration. Results: All prevaccine titers were non-protective. Post PPV23, 13 titers boosted impressively classifying the patient as having “mild” deficient response phenotype. Repeat testing within 6 months demonstrated notable decreases in titers, which may classify the patient as “memory” phenotype. Out of the 13 responsive titers, 12 are found in PCV13. Thus, PCV13 was administered resulting in a major boost in the corresponding titers.