Shoulder injury related to vaccine administration (SIRVA) is defined as “shoulder pain with limited range of motion within 48 hours after vaccine receipt in individuals with no prior history of pain, inflammation, or dysfunction of the affected shoulder before vaccine administration.” Corticosteroid injections (CSIs) have been proposed as a reasonable treatment modality for SIRVA, although evidence regarding efficacy is scanty. In this case series, we present two patients diagnosed with SIRVA who received CSI within 5 days of symptom onset and saw symptom resolution within 1 month. This is in comparison to a Centers for Disease Control and Prevention report that showed 65% of patients with SIRVA will have pain lasting longer than 1 month, and 25% will have pain lasting longer than 3 months. Our case series shows that CSIs may be an effective treatment modality for SIRVA. It would be reasonable to use CSIs as a first line treatment and should especially be considered in patients who have contraindications to nonsteroidal anti-inflammatory drugs.

Warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome (WS) is a combined immunodeficiency caused by gain-of-function mutations in the C-X-C chemokine receptor type 4 (CXCR4) gene. We characterize a unique international cohort of 66 patients, including 57 (86%) cases previously unreported, with variable clinical phenotypes. Of 17 distinct CXCR4 genetic variants within our cohort, 11 were novel pathogenic variants affecting 15 individuals (23%). All variants affect the same CXCR4 region and impair CXCR4 internalization resulting in hyperactive signaling. The median age of diagnosis in our cohort (5.5 years) indicates WHIM syndrome can commonly present in childhood, although some patients are not diagnosed until adulthood. The prevalence and mean age of recognition and/or onset of clinical manifestations within our cohort were infections 88%/1.6 years, neutropenia 98%/3.8 years, lymphopenia 88%/5.0 years, and warts 40%/12.1 years. However, we report greater prevalence and variety of autoimmune complications of WHIM syndrome (21.2%) than reported previously. Patients with versus without family history of WHIM syndrome were diagnosed earlier (22%, average age 1.3 years versus 78%, average age 5 years, respectively). Patients with a family history of WHIM syndrome also received earlier treatment, experienced less hospitalization, and had less end-organ damage. This observation reinforces previous reports that early treatment for WHIM syndrome improves outcomes. Only one patient died; death was attributed to complications of hematopoietic stem cell transplantation. The variable expressivity of WHIM syndrome in pediatric patients delays their diagnosis and therapy. Early-onset bacterial infections with severe neutropenia and/or lymphopenia should prompt genetic testing for WHIM syndrome, even in the absence of warts.

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A case report of recalcitrant allergic fungal sinusitis (AFS) refractory to systemic corticosteroids and multiple functional endoscopic sinus surgeries (FESSs) treated with anti-IgE antibody omalizumab is reported. AFS is often classified with chronic rhinosinusitis (CRS). Although similar symptoms are among the two diseases, AFS has a unique pathophysiology. Patients with AFS demonstrate type 1 hypersensitivity to fungal allergens, increased total serum IgE, increased CD8+ T-cell prevalence, and IL-4 and IL-5 response. Omalizumab should be considered in the treatment of AFS.