I had a patient with secretory immunoglobulin A, sIgA, immunodeficiency, lack of ability to produce it, apparently a genetic mutation, presumably from birth. This was dramatically different from another patient(s) with other Ig deficiency (IgG, IgM, ...), although all had some bronchiectasis.
The patient with sIgA deficiency had chron…
I had a patient with secretory immunoglobulin A, sIgA, immunodeficiency, lack of ability to produce it, apparently a genetic mutation, presumably from birth. This was dramatically different from another patient(s) with other Ig deficiency (IgG, IgM, ...), although all had some bronchiectasis.
The patient with sIgA deficiency had chronic sinusitis, and only mild bronchiectasis, which was found on an immunoglobulin screening assay for bronchiectasis. He had been mostly healthy to retirement age, but I first met him in extremis, low SpO2, with chest x-ray white-out, pulmonary edema versus pneumonitis or likely both, unlikely to be fibrosis, as it cleared shortly after starting Prednisone & Azithromycin. Need for supplemental O2 declined over weeks, to complete recovery. He had occasional repeat bouts, with years between attacks, although chronic sinusitis was a continuing complaint. The impressive point though was his relatively mild disease, doing quite well without sIgA, which was in contrast to other patients with other immunodeficiency. Maybe that's why an IgA, mucosal vaccine was not considered earlier? IgA seems to have less importance to protecting the lung, though just conjecture. I understand though sIgA can be very important to mucosal infection and spread of disease. Just another viewpoint...
I had a patient with secretory immunoglobulin A, sIgA, immunodeficiency, lack of ability to produce it, apparently a genetic mutation, presumably from birth. This was dramatically different from another patient(s) with other Ig deficiency (IgG, IgM, ...), although all had some bronchiectasis.
The patient with sIgA deficiency had chronic sinusitis, and only mild bronchiectasis, which was found on an immunoglobulin screening assay for bronchiectasis. He had been mostly healthy to retirement age, but I first met him in extremis, low SpO2, with chest x-ray white-out, pulmonary edema versus pneumonitis or likely both, unlikely to be fibrosis, as it cleared shortly after starting Prednisone & Azithromycin. Need for supplemental O2 declined over weeks, to complete recovery. He had occasional repeat bouts, with years between attacks, although chronic sinusitis was a continuing complaint. The impressive point though was his relatively mild disease, doing quite well without sIgA, which was in contrast to other patients with other immunodeficiency. Maybe that's why an IgA, mucosal vaccine was not considered earlier? IgA seems to have less importance to protecting the lung, though just conjecture. I understand though sIgA can be very important to mucosal infection and spread of disease. Just another viewpoint...