Rhinologic Symptoms and Quality-of-Life in Patients with Churg-Strauss Syndrome Vasculitis

Methods

Ethical approval for this study was obtained from the Royal Free Hospital Medical School Research Ethics Committee, London, U.K. A postal enquiry was performed of 49 CSS patients who were members of the Stuart Strange Vasculitis Trust, a U.K. patient self-help group. This obtained basic information including circumstances leading to patients’ diagnoses of CSS and current treatment regimens.

Our symptom enquiry included a selection of relevant nasal symptoms as part of a rhinosinusitis disease-specific QOL instrument, the Sino-Nasal Outcome Test (SNOT-22). This was the widely used modification of the original SNOT-20 ⁴ and was previously validated in the Royal College of Surgeons of England's National Comparative Audit of Surgery for Nasal Polyposis and Chronic Rhinosinusitis. ⁵ In addition, we included three additional symptoms that we have previously found relevant to patients with vasculitis ⁶ : epistaxis, nasal crusting, and nasal deformity. A symptom severity product (SSP) was calculated for each symptom. This was the product of severity scoring for each symptom on a scale from one to five and the percentage of CSS patients reporting the symptom. SSP values were compared with figures for WG patients from our previous study using the two-tailed t-test, Statistica Version 6. Finally, the SNOT-22 score average for the CSS group was compared with the preoperative general chronic rhinosinusitis (CRS) population average published in the National Comparative Audit (n = 2803; average, 42.0; 95% CI = 41.2–42.7) using the student t-test, Statistica Version 6.

Results

Group Demographics and Diagnosis Pattern

Twenty-five patients with CSS responded (51%); 10 (40%) men and 15 (60%) women. Twenty-three were white (92%), 1 was Asian (4%), and 1 was of African origin (4%). The average age at diagnosis was 55.4 years (range, 31.7–79.3 years; SD, 14.5). The average age at the time of the survey was 56.3 years (range, 33–81 years; SD, 14.6). The average reported delay in diagnosis was 18.5 months (range, 1–71 months) from presenting to the physician with worsening of symptoms that eventually led to the diagnosis. Ten (40%) patients reported worsening of their asthma or new pulmonary findings as the most prominent event. A significant proportion of patients (28%) reported that their initial diagnosis was mostly related to worsening of their nasal symptoms. The frequency of predominant presenting symptoms leading to the diagnosis of CSS with respect to the various bodily systems is summarized in Fig. 1

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Figure 1

Predominant presenting bodily system in Churg-Straus syndrome patients leading to their diagnosis (n = 25). LRT, lower respiratory tract; CNS, central nervous system; PNS, peripheral nervous system.

Sinonasal Symptom Pattern and QOL

Active sinonasal symptoms were reported by 80% of CSS patients (20/25). Ninety-five percent (19/20) of these reported nasal obstruction and excessive rhinorrhea. Eighty-five percent (17/20) reported anosmia and 80% (16/20) reported excessive sneezing. As many as 75% of patients (15/20) reported some degree of nasal crusting, 65% (13/20) had purulent nasal discharge, and 60% (12/20) reported each of facial pain, postnasal discharge, and epistaxis to varying degrees. The SSP for each rhinologic symptom in CSS is shown in Fig. 2, with comparative data for patients with WG. The average SNOT-22 score of nasally affected CSS patients (n = 20) was 41.55. This did not differ significantly from that of the general CRS population ⁵ (SNOT-22 = 42.0; p = 0.68; t-test, Statistica Version 6).

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Figure 2

Rhinologic Symptom Severity Product values for Churg-Straus Syndrome (CSS, n = 20) and Wegener's granulomatosis patients (WG, n = 127) with nasally active vasculitis (*statistical significance; two-tailed t-test, Statistica Version 6).

Discussion

With up to 28% of patients presenting with worsening nasal symptoms, it is clear that rhinologists play an important role in the diagnosis of CSS. The significant delay in the diagnosis highlights the need to increase awareness of this condition. Despite this, it is likely that diagnosis will remain difficult in the ear, nose, and throat (ENT) setting, with the relatively common findings of nasal polyps and rhinitis being the main presentations, and negative ANCA testing in many more patients than is encountered in WG. From our experience, a careful enquiry of recent or adult-onset asthma is valuable. It is also worth remembering that although CSS is rare, complex, and with many of its clinical features rather unfamiliar to the ENT surgeon, it can at times present with external clinical signs such as vasculitic cutaneous lesions or CSS-related mononeuritis multiplex, which may include the cranial nerves. Similarly, simple routine clinical investigations can lead to the diagnosis in suspected CRS and polyp patients (Table 1). Once the diagnosis is made, referral to other clinicians opens up a whole new set of therapeutic possibilities, leading to significant improvements in patients’ QOL.

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Table 1

Clinical tests that may aid diagnosis of Churg-Straus syndrome (CSS) in suspected chronic rhinosinusitis/polyp patients

Test	Features in CSS	Comment
FBC	Eosinophilia of >10% of total white cell count	Masked by systemic steroid treatment (e.g. for asthma)
ESR	Raised	Even in the absence of infection
Serum creatinine	Raised	Simple low-cost tests, abnormal in >25% of CSS patients
Urinalysis	Proteinuria, microscopic hematuria
Chest x ray	Peripheral, nonsegmental patchy lung consolidation	If present, often bilateral, atypical, and variable patterns
ANCA	Perinuclear pattern and MPO+ (pANCA MPO)	Negative in up to 50% of CSS patients; other positive ANCA patterns not diagnostic
CFTR profile	Any pattern	No specific link to CSS but required for severe nasal polyposis generally

FBC = full blood count; ESR = erythrocyte sedimentation rate; CFTR = cystic fibrosis transmembrane conductance regulator gene; MPO = myeloperoxidase.

This study includes a good number of patients with a rare condition, and our sampling of patients from the community minimizes the selection bias that often affects cohorts from subspecialist units. On the other hand, there is an inherent reliance on patients’ memories and inability to clinically confirm the diagnosis of CSS in each patient. Even if this was possible, pinpointing the diagnosis with certainty remains difficult due to the poor sensitivity of laboratory tests and modest positive predictive values of the continuously evolving criteria for CSS diagnosis.^7–9 Each patient from our study group confirmed that the diagnosis of CSS was reached by their hospital specialist, and we believe that patients suffering from such a serious disease belonging to a self-help group are likely to provide as accurate information as any retrospective search of medical notes.

In a retrospective case note review of vasculitis patients in Norfolk, U.K., ¹⁰ only 33% of 18 CSS patients were documented to have active nasal disease at the time of the study, and none were documented by their physicians to have had predominantly nasal symptoms at initial presentation. This is a sharp contrast to our patient-reported figures of 80% and 28%, respectively, indicating possible physician underestimation of nasal morbidity in CSS. Our study confirms the mostly rhinitic symptom pattern in CSS but it is somewhat surprising to see that 60–75% also report crusting and troublesome epistaxis, albeit in less severe forms than in WG with which these symptoms have been more commonly associated. The prevalence of nasal crusting in our community-based group of CSS patients is also much higher than a previously reported figure of 14.2% in a clinically selected series from a subspecialist unit. ¹¹

In our experience, nasal polyps in these patients appear to respond to both systemic and topical steroids but this remains somewhat speculative and, to our knowledge, no prospective studies have been performed to investigate this aspect. The significant proportion of patients undergoing nasal surgery is undoubtedly related to the high prevalence of severely recurrent nasal polyps, confirming the important role of surgery in CSS. Polyps in CSS are typically of the histologically benign eosinophilic type, and the main purpose for biopsy in this situation is to exclude other nasal pathology. In fact, nasal biopsies in CSS are even less likely to confirm vasculitis than the modest positive rates seen in WG, and the clinician almost always needs to rely on other criteria outside the ENT region for CSS diagnosis.

We also find that although 80% of CSS patients report active nasal symptoms, only 32% were using topical nasal steroids. This may be a reflection of the modest efficacy of this treatment modality in CSS-related rhinitis leading to patient noncompliance. Alternatively, it could, perhaps, be a result of underprescription by the patients’ own physicians who may hold the general view that nasal symptoms are insignificant in this setting of multisystem disease. If the latter were true, the patients themselves seem to disagree, as evidenced by their high SNOT-22 scores. These scores show that sinonasal morbidity in CSS patients is on a par with that of the general CRS population, which has already been shown to have a similar impact on patients’ QOL to other common chronic conditions such as diabetes mellitus and rheumatoid arthritis. ¹²

Conclusion

The diagnosis of CSS is challenging and often associated with significant delay. This is especially true in the ENT setting where patients predominantly present with the common symptoms of rhinitis and nasal polyps. The patient-reported prevalence of active nasal symptoms in CSS is higher than previously published figures, and although the symptom pattern is mostly rhinitic, it also includes crusting, epistaxis, and sinusitis to varying degrees. It is also concluded that CSS-related nasal disease impacts significantly on patients’ QOL to a degree that may be underestimated by the physicians who care for them.