The disease usually manifests within the first 6 years of life with recurrent bacterial infections of the upper respiratory tract, i.e. chronic purulent rhinitis often complicated by nasal septum perforation and nasal polyps, sinusitis and otitis media (unpublished observations and [3,4]). Involvement of the lower respiratory tract typically manifests in the second decade of life with recurrent spastic bronchitis, bacterial pneumonia and eventually bronchiectasis. Isolated pathogens from the respiratory tract commonly include Haemophilus influenzae, Streptococcus pneumoniae, Staphylococcus aureus, Klebsiella species, Escherichia coli and Pseudomonas aeruginosa, the latter two associated with chronic stages of the disease. Severe viral infections are noticeably absent and normal antibody titres against several viruses could be demonstrated in most patients. Postnasal drip syndrome, which can usually be found in patients with TAP deficiency syndrome, probably promotes bacterial lung infections, especially in those patients who have undergone surgery of the paranasal sinus. Interestingly, histology of the paranasal sinus and nasal polyps may reveal a necrotizing granulomatous inflammation with a close resemblance to Wegener's granulomatosis (WG) ([2], see also [33, 34]), while granulomata could not been demonstrated in biopsies from lung and bronchi (unpublished observations).