A Safe and Novel Outpatient Subcutaneous Vitamin B12 Desensitization Protocol in A Patient with Crohn's Disease and Vitamin B12 Allergy: A Case Report.

INTRODUCTION: Although rare, some patients may have a vitamin B12 allergy. Crohn's disease commonly leads to significant vitamin B12 deficiency, especially in those patients that have undergone ileal resection. In these difficult cases, vitamin B12 desensitization may be required. CASE PRESENTATION: Here, we report a successful case of a serial outpatient subcutaneous vitamin B12 desensitization protocol in a 35-year-old female with a past medical history of Crohn's disease status post ileal resection, subsequent vitamin B12 deficiency, and allergy to subcutaneous vitamin B12. CONCLUSION: This is the first subcutaneous vitamin B12 desensitization protocol reported to have been safely performed in the outpatient setting.

Antibody Deficiency, Chronic Lung Disease, and Comorbid Conditions: A Case-Based Approach.

New emerging pulmonary phenotypes associated with antibody deficiency, such as neutrophilic asthma, frequent exacerbations of chronic obstructive pulmonary disease, and unexplained interstitial lung disease, particularly in younger adults, are discussed in this review through a case-based approach. Also discussed in similar fashion are antibody deficiency syndromes that lead to end-stage lung disease and the indications for lung transplantation in primary immunodeficiency disease. These challenging cases require timely and individualized strategies for genetic and immunologic diagnosis, decisions about therapeutic approaches, and long-term monitoring.

Factors Associated with Self-Reported Drug Allergies in a Large Chronic Spontaneous Urticaria Cohort.

INTRODUCTION: Chronic spontaneous urticaria (CSU) is characterized by recurrent hives without a known trigger. While certain drugs are associated with urticaria exacerbations, the overall drug allergy incidence in CSU is unknown. We hypothesized that the incidence of drug allergy in CSU would be greater than the general population and that there would be distinguishing clinical features of drug-allergic CSU patients. METHODS: 362 adult CSU patients seen over a 10-year period at a University Allergy/Asthma clinic were identified. Patients reported no drug allergies or any drug allergy. Multiple drug allergies were defined as allergies to ≥ 2 chemically unrelated drugs. Using Chi-square or Wilcoxon analysis, we compared demographic features of CSU with and without drug allergy and with multiple vs. single drug allergy. RESULTS AND DISCUSSION: Overall, 202 CSU patients (56%) reported drug allergy. Drug allergic CSU patients were older, with a greater proportion of whites and higher BMI vs. CSU without drug allergy (p=0.002, p=0.047, p=0.004, respectively). Penicillin was the most common drug allergy, with urticaria the most frequently reported reaction. Female sex, white race, older age at the visit, and co-existing asthma were more common in multiple drug allergy (n=115) vs. single drug allergy (p=0.002, p=0.02, p=0.03, p=0.0002, respectively). CONCLUSION: In CSU, the prevalence of self-reported drug allergies was higher than the general population. Drug allergy is associated with older age, white race and higher BMI, while multiple drug allergy was also associated with asthma. These CSU sub-populations should be studied to avoid the potential for morbidity associated with less efficacious and more costly drugs.

Clinical implications of C6 complement component deficiency.

Background: Terminal complement component deficiencies are risk factors for neisserial infections. Objective: To review the clinical characteristics, the diagnosis and the management of patients with a terminal complement component deficiency. Methods: Pertinent articles were selected and reviewed in relation to a case presentation of C6 deficiency. Results: A case of a 56-year old patient with a history of meningitis, chronic rash, and C6 deficiency was presented, followed by discussion of clinical characteristics, diagnosis, and management of terminal complement component deficiencies. Clinical pearls and pitfalls were reviewed for the practicing allergist/immunologist and fellow-in-training. Conclusion: C6 deficiency is the most common terminal complement component deficiency and can present later in age with N. meningitidis infections. Patients can be screened for terminal complement component deficiency by checking CH50.

A 72-year-old woman with periorbital swelling.

As allergists, we are frequently consulted to evaluate patients with swelling presumed to be angioedema. Patients with presumed angioedema can have multiple possible underlying triggers. We present the case of a hospitalized 72-year-old woman with a history of hypertension and metastatic chordoma who developed marked periorbital swelling that precluded eye opening 2 days after a neurosurgical operation (chordoma resection and T10-11 hardware repair). After a detailed evaluation of her swelling, a broad differential diagnosis was made; she did not respond to high-dose antihistamines, systemic steroids, icatibant and angiotensin-converting enzyme inhibitor cessation. Ultimately, computed tomography imaging confirmed a specific diagnosis. The differential diagnosis for swelling is complex, and this case illustrated the importance of considering alternative causes of swelling when evaluating cases of possible angioedema.

Vocal Cord Dysfunction: The Spectrum Across the Ages.

Vocal cord dysfunction (VCD) is an upper airway disorder characterized by exaggerated and transient glottic constriction causing respiratory and laryngeal symptoms. Although the origin of VCD symptoms is in the upper airway, it is frequently misdiagnosed as asthma resulting in significant morbidity. VCD can coexist with asthma or mimic allergic conditions affecting the upper airway. VCD may be difficult to diagnose, because patients are intermittently symptomatic and VCD awareness in the medical community is underappreciated. Once VCD is diagnosed and treated, most patients report significant improvement in their symptoms as well as a decrease in asthma medication use.

Detrimental Association of Hypogammaglobulinemia With Chronic Lung Allograft Dysfunction and Death Is Not Mitigated by On-Demand Immunoglobulin G Replacement After Lung Transplantation.

BACKGROUND:: Hypogammaglobulinemia (HGG), immunoglobulin G (IgG) <700 mg/dL, is associated with infections, chronic lung allograft dysfunction, and death following lung transplantation. This study evaluates the use of on-demand intravenous IgG in lung transplant recipients with HGG. MATERIALS AND METHODS:: This single-center retrospective cohort study of adult lung recipients evaluated 3 groups, no, untreated (u), or treated (t) HGG at first IgG administration or a matched time posttransplant. Primary outcome was freedom from allograft dysfunction. Secondary outcomes included development of advanced dysfunction, rejection, infection burden, and mortality. RESULTS:: Recipients included 484 (no HGG: 76, uHGG: 192, tHGG: 216). Freedom from chronic allograph dysfunction was highest in the non-HGG group 2 years post-enrollment (no HGG 77.9% vs uHGG 56.4% vs tHGG 52.5%; P = .002). Freedom from advanced dysfunction was significantly different 2 years post-enrollment (no HGG 90.5% vs uHGG 84.7% vs tHGG 75.4%; P = .017). Patients without HGG and those with uHGG had less mortality at 2 years post-enrollment (no HGG 84.2% vs uHGG 81.3% vs tHGG 64.8%; P < .001). Gram-negative pneumonias occurred more often in the tHGG group ( P = .02). CONCLUSIONS:: Development of chronic lung allograft dysfunction, patient survival, rejection burden, and key infectious outcomes in lung transplant recipients were still problematic in the context of on-demand IgG therapy. Prospective studies are warranted.

A Prospective Observational Study of Hypogammaglobulinemia in the First Year After Lung Transplantation.

BACKGROUND: Immunosuppressive therapies have led to improved survival for lung transplant (LT) recipients but these therapies can lead to hypogammaglobulinemia (HGG) and potentially an increased risk of infection. Large prospective studies have not been performed to evaluate the impact of HGG on outcomes for LT recipients. METHODS: This is a single-center prospective observational study of LT recipients. Pretransplant and posttransplant IgG levels were measured and related to infection, rejection, antibiotic use, and immunosuppression use. RESULTS: One hundred thirty-three LT recipients were prospectively evaluated. Pretransplant IgG values were higher than IgG values at the time of transplant or any time thereafter (all P < 0.0001). Severe HGG (IgG < 400 mg/dL) was highest at the time of transplant (32.4%) while at 3, 6, 9, and 12 months posttransplant the prevalence of severe HGG was 7.4%, 7.5%, 8.9%, and 6.3%, respectively. Severe HGG was associated with 2 or more pneumonias (P = 0.0006) and increased number of antibiotic courses (P = 0.003) compared with the subjects without severe HGG. Pretransplant IgG level and less than 30% of pretransplant protective pneumococcal antibody levels were identified as pretransplant risk factors for severe HGG. In multivariate analysis, chronic obstructive pulmonary disease as the underlying disease and the use of basiliximab as the induction agent in conjunction with higher prednisone and mycophenolate dosing were most predictive of severe HGG (P = 0.005), whereas the combination of age, severe HGG and number of acute steroid courses were most predictive of total days of pneumonia (P = 0.0001). CONCLUSIONS: Our large prospective study identifies risk factors for severe HGG after LT and demonstrates that LT recipients with severe HGG are at increased risk for recurrent pneumonias and more antibiotic courses.

Co-existence of vocal cord dysfunction with pulmonary conditions other than asthma: A case series.

BACKGROUND: Vocal cord dysfunction (VCD) is defined as inappropriate movement of the vocal cords resulting in functional airway obstruction and symptoms including cough, wheezing, and dyspnea. VCD is often misdiagnosed with asthma but can also co-exist with asthma. The association of VCD with other serious pulmonary conditions has not been described to date. CASE REPORTS: We describe the first case series of two adult patients evaluated at a university asthma clinic who in addition to having VCD also had significant pulmonary pathology other than asthma. Patient 1 had VCD and pulmonary veno-occulsive disease which necessitated a lung transplant. Patient 2 had VCD and a patent ductus arteriosis who necessitated surgical closure. CONCLUSION: It is important to recognize that VCD can exist with pulmonary conditions other than asthma. Lack of improvement in respiratory symptoms after appropriate treatment for VCD should alert the clinician to evaluate for additional conditions.

A 10-year experience of a novel and safe modified environmental rush immunotherapy protocol.

BACKGROUND: Allergen immunotherapy (AIT) is an effective treatment option for allergic rhinitis. Although conventional AIT takes 6 months to reach maintenance dosing, rush AIT accelerates the build-up period and reaches the maintenance dose months earlier. However, accelerated schedules of AIT carry an increased risk of systemic reactions (SR). OBJECTIVE: We aimed to describe a novel 1-day, eight-step modified environmental rush immunotherapy (MERIT) protocol, characteristics of the patients who underwent this therapy, and the safety of this procedure. We also compared distinguishing features of those patients with SRs. METHODS: We retrospectively analyzed demographic and clinical data of 362 adult patients seen in an outpatient university allergy clinic, from January 2005 to January 2015, and who underwent MERIT protocol treatment for allergic rhinitis. RESULTS: In a univariate analysis, the factors significantly associated with SR were lower body mass index (BMI); younger age; a higher number of allergens in the extracts; and the presence of cat, dust mite, and certain weed pollens. In a multivariate analysis, cat, dust mite, and mugwort were significantly associated with SRs. Over the 10-year period, 50 patients experienced SRs (13.81%), with a total number of 68 SRs. Only 4.7% of the SRs occurred on the MERIT day. Most SRs occurred >30 minutes and were mild. Our MERIT protocol continuation rate for all the patients was 49.2% and did not seem to be influenced by having an SR versus no SR. CONCLUSION: We present a modified rush AIT protocol that seems to be effective and safe. Most patients tolerated therapy, and only a minority of patients developed SRs, which generally were mild. We identified novel risk factors for SRs that may help determine optimal dosing to decrease the risk of SRs. Ultimately, future studies will be needed to compare the safety of our MERIT protocol with traditional AIT.