Page 204 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

180 Constantina Yiannakis, Lorna Ward and Matthew David Morgan

Otolaryngologic

Up to 77% of patients with GPA will have upper respiratory tract symptoms at
presentation in contrast to 29% of those with MPA [7]. Nasal disease is more common than
deafness and middle ear involvement in both GPA and MPA. More than 90% of patients with
GPA develop ear and upper airway disease [12, 13]. Typical nasal symptoms of GPA include
rhinitis, nasal stuffiness and pain, epistaxis, and brown or bloody crusting. The characteristic
“saddle-nose deformity” is secondary to septal erosion due to chronic nasal inflammation.
Patients with GPA are at increased risk of nasal and sinus infections due to chronic damage
which increases the difficulty in differentiating secondary infection from disease activity.
Middle ear involvement in GPA presents as conductive hearing loss with the commonest
cause being Eustachian tube dysfunction secondary to nasopharyngeal disease [14, 15].

Sensorineural hearing loss is due to inner ear disease which may also be associated with
vestibular dysfunction with some patients presenting with vertigo and balance disturbance
[15, 16]. The mechanism of inner ear involvement in GPA is poorly understood when
compared to middle ear involvement [17].

Involvement of the trachea may present with pain, cough, hoarseness, wheezing or
stridor, however it is often asymptomatic at onset. The development of subglottic or bronchial
stenosis can lead to severe dyspnea requiring tracheostomy or reconstruction [9].

Pulmonary

Pulmonary involvement occurred at presentation in 63% of patients with GPA compared
to 29% of those with MPA [7]. Pulmonary manifestations range from new-onset asthma and
„antibiotic-resistant? pneumonia to life-threatening alveolar hemorrhage. In those with GPA
the symptoms may be due to single or multiple, unilateral or bilateral lung nodules and
granulomata, and may be misdiagnosed as cavitating infectious or neoplastic lesions;
mediastinal and hilar lymphadenopathy may also occur [18]. Pulmonary capillaritis is
responsible for lung hemorrhage and rapidly changing alveolar infiltrates and should be
suspected in patients with falling hemoglobin levels in conjunction with hemoptysis and
dyspnea; those with MPA may also develop pulmonary interstitial fibrosis [19].

Other indicators of pulmonary involvement include wheezing, productive or non-
productive cough, and exertional dyspnea.

Renal

This is often a severe manifestation of AAV [20] that affects an estimated 77% of
patients with GPA, and 92% of those with MPA at presentation [7]. Renal vasculitis presents
with proteinuria, hematuria, and red cell casts due to focal necrotizing glomerulonephritis
[21]. It can lead to rapid deterioration of renal function with eventual dialysis dependency.
Hematuria may be microscopic or frank, while proteinuria is usually insufficient to cause
nephrotic syndrome [22].

Renal biopsy is the most appropriate method of identifying and prognosticating
glomerulonephritis in AAV [23, 24].

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