Q:

7/22/2013
My patient is a 63 year old African American male with a history of a CVA (no neurologic sequelae) who was well until late last year when he began to suffer with severe nasal congestion. He was diagnosed with severe nasal polyposis and recurrent sinusitis (the later of which was thought to be due to obstruction). He had undergone a polpectomy and was completely asymptomatic for about 2 months (no surprise there) and then his symptoms were just as severe as before the procedure and so he was sent to me for an evaluation. I performed the requisite skin testing which was extensive (including about 9 different molds) and negative with good control. I repeated his CT which showed extensive disease but there was no mention of hyperechoic areas or of anything other than inflammation. I then sent him back to his ENT who then sent him back to me for an immunodeficiency work up. What did I find?

Normal Ig, IgG subclasses, ok pneumococcal titers, no tetanus titers, to diphtheria titers (pt could not remember ever having a tetanus shot let alone a DTaP), normal complements, CBC remarkable for 20% eosinophilia (same on repeat), IgE 550, negative aspergillis antibodies, good H.flu titers.

I gave him a DTaP and...no repsonse! Still has eosinophilia and now ENT says lots of mucin and maybe AFS which he said no to before. Any ideas? Thank you!

A:

Thank you for your inquiry.

Chronic eosinophilic rhinosinusitis with nasal polyps is a well-recognized subcategory of sinusitis and not an uncommon problem in the practice of Allergy-Immunology. You are correct in that an immune workup is usually unnecessary because this disease is probably not infectious, per se, in origin. And in my opinion the failure to respond to DTaP is not relevant to his rhinosinusitis and nasal polyps. Although a superantigen response against bacteria has been implicated in some patients, for the most part, the pathophysiology is inflammatory, and the sinusitis is of hyperplastic nature as if arising de novo from the mucosa. In addition, it does not appear to be due to obstruction of the ostia, as has been implicated for acute infectious sinusitis (see Snidvongs abstract copied below).

I have also copied other abstracts below that will help you understand this condition. The Sok/Ferguson article has an excellent discussion of the differential diagnosis of eosinophilic chronic rhinosinusitis, and the Meltzer/Hamilos article is a summary of recent consensus guidelines. It has a nice discussion on chronic eosinophilic rhinosinusitis as well. It is available to you free of charge online. For your convenience, I have copied the link to this article as well so that you may access it.

These articles will give you a more in-depth understanding of the pathogenesis and treatment of chronic eosinophilic rhinitis with nasal polyps.

In the meantime, here are some suggestions:
1. It is possible that your patient is in the early stage of, and will develop, asthma-exacerbated respiratory tract disease. In such patients, there is an overproduction of leukotrienes and therefore an empiric trial of antileukotriene therapy is warranted. Montelukast is one option but there is some evidence that zileuton may be more efficacious in this disorder. He should also, of course, be observed for any sign of asthma.

2. You did not state whether he was aspirin intolerant, so I assume that there is no history of such. Although we have good data on the efficacy of aspirin desensitiztion in the treatment of eosinophilic rhinosinusitis associated with asthma-exacerbated respiratory tract disease, we have no data, to my knowledge, as to the administration of aspirin in patients without intolerance to this drug. But should he develop aspirin intolerance, desensitization has been shown to be highly efficacious in controlling the sinusitis of this syndrome. Usually a dose of 325 mg to 650 mg (in the majority, the higher of the two) is required.

3. The staple of therapy for patients who are not aspirin intolerant is corticosteroids. Topical nasal corticosteroids should be administered on a daily basis. Some studies have found that budesonide ampules 1 mg daily to b.i.d. as a nasal wash is superior to intranasal coticosteroid sprays. With polyps, should you use an intranasal spray, quite often doubling the normal dose is required, and at least one nasal steroid, mometasone in double dose, has been shown to be helpful.

4. Before initiating topical nasal steroids or simultaneously with their initiation, a course of oral corticosteroids is indicated. This is necessary to shrink the polyps and allow better penetration of the intranasal application. A starting dose of 40 to 60 mg of prednisone a day, tapering over 10 days, is a reasonable dose.

Despite these therapies, some patients need recurrent polypectomies, but many can be managed with the treatments noted above without the necessity of repeated surgeries.

Thank you again for your inquiry and we hope this response is helpful to you.

Laryngoscope. 2013 May;123(5):1070-4. doi: 10.1002/lary.23721. Epub 2013 Apr 2.
Eosinophilic rhinosinusitis is not a disease of ostiomeatal occlusion.
Snidvongs K, Chin D, Sacks R, Earls P, Harvey RJ.
Source
Australian School of Advanced Medicine, Macquarie University, Sydney, Australia.
Abstract
Objectives/Hypothesis: Ostiomeatal complex (OMC) occlusion may play a role in the pathogenesis of some chronic rhinosinusitis (CRS) subgroups, but its role in diffuse mucosal inflammation is strongly debated. The association between radiological OMC occlusion and its draining sinuses in patients with eosinophilic rhinosinusitis (ECRS) compared to non-ECRS is investigated.
Study Design: Case-control study.
Methods: Patients with CRS who underwent endoscopic sinus surgery were investigated. Preoperative computed tomography scans were evaluated. Structured histopathology reporting was performed. The study group was patients with high tissue eosinophil >10/high power fields (HPF), and the control group was patients with low tissue eosinophil ¡Ü 10/HPF. The radiological relationship of OMC occlusion to the draining sinuses was analyzed in each group.
Results: Seventy patients with a mean age of 49.7 ¡À 14.1 years were analyzed. Forty-one (58.6%) patients had high tissue eosinophil >10/HPF. All patients with ECRS had maxillary disease, and there were 36.2% without OMC occlusion. There was no association of OMC occlusion to either the anterior ethmoid (ECRS: odds ratio [OR], 1.84; 95% confidence interval [CI], 0.24-14.14; P = .55; non-ECRS: OR, 1.57; 95% CI, 0.34-7.33; P = .56) or frontal sinuses (ECRS: OR, 0.67; 95% CI, 0.12-3.82; P = .65; non-ECRS: OR, 1.58; 95% CI, 0.45-5.54; P = .47). For patients with non-ECRS, maxillary sinus diseases was present in 96.2% of those with OMC occlusion and 50% of those without (OR, 25.0; 95% CI, 2.77-226.08; P < .001).
Conclusions: OMC occlusion is not associated with draining sinuses for patients with ECRS. Simple surgical interventions directed at the OMC are unlikely to be of benefit to this CRS subgroup.

Curr Allergy Asthma Rep. 2006 May;6(3):203-14.
Differential diagnosis of eosinophilic chronic rhinosinusitis.
Sok JC, Ferguson BJ.
Abstract
Eosinophilic chronic rhinosinusitis (ECRS) encompasses a wide variety of etiologies. To date, a unifying pathophysiologic mechanism remains elusive. Eosinophilia is frequently, but not exclusively, caused by immunoglobulin (Ig)E-mediated hypersensitivity and is dominated by the associated cytokine milieu of Th2 inflammation. The provisional subcategories of ECRS include superantigen-induced eosinophilic chronic rhinosinusitis, allergic fungal sinusitis, nonallergic fungal eosinophilic chronic rhinosinusitis, and aspirin-exacerbated eosinophilic chronic rhinosinusitis. Within each subcategory, recent findings supporting distinct mechanisms that promote eosinophilic infiltration are presented, and, therefore, targeted therapeutic interventions with specific antibacterial, antifungal, or immune modulation may be indicated.

Mayo Clin Proc. 2011 May; 86(5): 427¨C443.
doi: 10.4065/mcp.2010.0392
PMCID: PMC3084646
Rhinosinusitis Diagnosis and Management for the Clinician: A Synopsis of Recent Consensus Guidelines
Eli O. Meltzer, MD and Daniel L. Hamilos, MD

Sincerely,
Phil Lieberman, M.D.

AAAAI - American Academy of Allergy Asthma & Immunology