CC: acute on chronic cough, shortness of breath for 1 week
HPI: 57 year old M with history of thymoma and chronic cough that presents with worsening cough and shortness of breath. Several months prior to presentation he developed a daily cough which is productive of yellowish green sputum. He was initially treated with omeprazole, loratadine, and fluticasone nasal spray, as well as nasal decongestants but had no improvement in symptoms. He also reports frequent rhinosinusitis infections, with productive nasal discharge and maxillary/frontal sinus pressure. These infections temporarily resolve with antibiotics from his primary care physician, yet they recur after several weeks. He presents to the ED now because his cough has worsened in regards to frequency throughout the day and in the amount of sputum production. In addition, he has developed worsening shortness of breath over the last week. He normally can ambulate multiple blocks but can now barely walk around his bedroom. Patient denies chest pain, fevers, chills, myalgias, night sweats, weight loss, or sick contacts. Patient is found to be hypoxic to 89% on RA and to have bibasilar rales when auscultating lung fields. Chest x-ray showed no consolidation but CT chest did demonstrate evidence of bronchiectasis which explained the patients acute on chronic symptoms. Sputum sample revealed patient to be infected with the encapsulated organism, H. influenza. Given the evidence of chronic rhinosinusitis infections, infection with encapsulated organism, bronchiectasis, and history of thymoma, a immunodeficiency work up was initiated. Secondary immunodeficiency causes like diabetes, renal dysfunction, cirrhosis, HIV, and nephrotic syndrome returned negative. Patient’s work up revealed low immunoglobulin levels, low CD4 count which in the setting of a prior thymoma led to the diagnosis of Good Syndrome. Patient has been treated with IVIG with decrease in infection frequency.
Sputum Culture: H. Influenzae
HIV Ab/Ag negative
Flow Cytometry: CD4 392, CD8 882, CD4/CD8 ratio 0.4
Tetanus Antibody Titer: <0.05 in the setting of vaccination within last year
IgA 13 (79 – 455)
IgG 207 (650 – 1600)
IgM < 3 (40 – 240)
IgE < 2
UA: no protein
Morning Report Pearls:
Always go back to your basics when identifying types of immunodeficiencies:
Once you identify the area of immune system defect, you can focus the work up but always rule out secondary causes of immunodeficiencies first, as these are more common:
When the type of immunodeficiency is narrowed then further work up can be sought out. In this case, patient had strong evidence in favor of antibody deficiency. The work up consists of:
This patient was found to be deficient in all immunoglobulins and have low tetanus titers despite recent vaccination which went towards the diagnosis of antibody defect which can be seen in CVID. However given the history of thymoma, Good Syndrome remained on the differential which involves also cell mediated defects. This was confirmed by a low CD4 count.