Crit Care Clin 18 (2002) 749 – 765

Airway problems in patients with

rheumatologic disorders
Venkata Bandi, MDa, Uma Munnur, MDb,
Sidney S. Braman, MDc,*
a
Pulmonary/Critical Care, Department of Medicine, Baylor College of Medicine, 1 Baylor Plaza,
GPF 80, Houston, TX 77030, USA
b
Department of Anesthesiology, Baylor College of Medicine, 1 Baylor Plaza,
Houston, TX 77030, USA
c
Department of Internal Medicine, Brown University, Providence, RI 02912, USA

This article reviews problems associated with airway management in pa-

tients with rheumatologic disorders. Upper airway and tracheal disorders and the
impact of spinal involvement on airway management are discussed. Large and
small airways disease associated with rheumatologic diseases are not addressed in
this article.
The upper airway comprises mucosal, cartilaginous, articular, and neurologic
structures. Anatomically, the upper airway is defined as the respiratory passage
extending from the oral cavity and nares down to the mid-trachea. A variety of
rheumatologic diseases can present with, or lead to, upper airway obstruction.
Upper airway problems can present acutely as a life-threatening event or can be a
chronic, indolent process that leads to airway compromise during periods of
increased ventilatory demands and severe illness. Vocal fold immobility, crico-
arytenoid joint disease, and mucosal or chondral disease can lead to upper airway
obstruction. Langford et al [1] reviewed upper airway obstruction in rheumatol-
ogic diseases at length. Table 1, which has been adapted from their article,
documents some of their findings.

Laryngeal anatomy
The larynx is situated at the crossroads of the air and food passages. Nine
cartilages provide a framework for the muscles, ligaments, and mucosal folds that
ensure the proper functioning of the larynx. Vestibular folds and vocal folds are the

* Corresponding author. Box G-RIH, Brown University, Providence, Rhode Island 02912, USA.
E-mail address: Sidney_Braman@Brown.edu (S.S. Braman).

0749-0704/02/$ – see front matter D 2002, Elsevier Science (USA). All rights reserved.
PII: S 0 7 4 9 - 0 7 0 4 ( 0 2 ) 0 0 0 3 0 - 1
750 V. Bandi et al. / Crit Care Clin 18 (2002) 749–765

Table 1
Spectrum of upper airway manifestations leading to airway obstruction in rheumatologic diseases
Neurologic or
Mucosal or Cricoarytenoid muscular vocal
Rheumatologic disease chondral disease arthritis fold paralysis
RA + +++ + +
Juvenile rheumatoid arthritis 0 + 0
Systemic lupus erythematosus + + + + +
Scleroderma + 0 0
Sjögren’s syndrome + 0 0
Relapsing polychondritis + ++ + 0 0
Wegener’s granulomatosis + ++ + 0 +
Angioedema + ++ + 0 0
Abbreviations: 0, nonexistent to very rare; +, isolated case reports; + + +, frequency 5% to 20%; + + + +,
frequency greater than 20%
Modified from Langford CA, Van Waes C. Upper air way obstruction in the rheumatic diseases.
Rheum Dis Clin North Am 1997;23:345 – 63; with permission.

prominent structures in the larynx and are separated by the ventricle, a mucosal
cavity (Fig. 1). The vestibular folds protect the airway against aspiration and have
no direct role in speech. The vocal folds are a pair of midline structures that are
responsible for speech and that also aid in respiration and airway protection. Vocal
ligaments are located within the medial aspect of the vocal folds and extend from
the thyroid cartilage anteriorly to the arytenoids posteriorly.
The laryngeal introitus is a roughly triangular opening in the anterior wall of the
pharynx, with its base lying along the free edge of the epiglottis and its apex located

Fig. 1. Anterior coronal (A) and midsagittal (B) view of the laryngeal soft tissues. (From Langford
CA, Van Waes C: Upper airway obstruction in the rheumatic diseases. Rheum Dis Clin North Am
1997:23;345 – 63; with permission.)
 V. Bandi et al. / Crit Care Clin 18 (2002) 749–765 751

at the point between the two arytenoid cartilages. The internal cavity of the larynx
may be divided into three parts: the vestibule, the glottis, and the infraglottic area.
The vestibule, or supraglottic area, stretches from the free margin of the epiglottis
and aryepiglottic folds inferiorly to the lower margin of vestibular folds. The
ventricle is the laterally directed sac located between the lower surface of the
vestibular folds and the upper surface of the vocal folds. The vocal folds form the
‘‘rima glottides’’ in the space between them. The area within the larynx, beginning
at the free margin of the vocal folds and extending down to the lower margin
of the cricoid cartilage, is referred to as the subglottic or infraglottic area. With
the exception of the vocal folds, virtually the entire larynx is lined by respiratory,
pseudostratified, columnar, ciliated epithelium with goblet cells. The vocal folds
are covered with stratified squamous, nonkeratinizing epithelium. Beneath the
mucosa of the vocal folds is a potential space stretching from the tips of the vocal
processes to the anterior commissure, overlying the muscles of the vocal fold and
vocal ligament, known as Ranke’s space. This space is essential for the free flow of
the mucosa over the vocal folds during phonation but is prone to develop edema
with minimal trauma and inflammation.
Vocal fold movement is a complex process influenced by mucosa, crico-
arytenoid joints, arytenoids cartilages, and the muscles of the larynx and their
innervation. Many rheumatologic conditions alter the function of the vocal folds
and lead to a compromised airway.
The extrinsic muscles of the larynx, also known as the strap muscles, are
rarely involved in rheumatologic diseases. The intrinsic muscles of the larynx –
the posterior cricoarytenoid, the lateral cricoarytenoid, the transverse arytenoids,
the oblique arytenoids, and the thyroarytenoid –are all innervated by the recur-
rent laryngeal nerve. The posterior cricoarytenoid muscle arises from the midline
on the cricoid cartilage. This muscle is the only abductor of the vocal cords and
opens the glottis by a rotatory motion over the arytenoids cartilages around an
apparent axis of the cricoarytenoid joints. It tenses the vocal fold and has a role
in phonation.

Articulations of the laryngeal cartilages

Because the cricothyroid and cricoarytenoid joints are the two synovial
joints of the larynx, they can be affected by a number of rheumatologic
processes. The inferior horn of the thyroid cartilage articulates with the cricoid
cartilages at the cricothyroid joint. This joint has a well-developed capsule and
permits a rotary movement with the activity of the cricothyroid muscle. The
joint between the base of the arytenoid cartilage and the lamina of the cricoid
cartilage –the cricoidarytenoid joint– is also a synovial joint. The capsule of the
joint is loose, allowing rotatory, medial, and lateral gliding moments. Even
when the arthritic process affects the cricothyroid joint, it does not always lead
to airway problems. Because the vocal ligament and the vocal folds are at-
tached to the arytenoids posteriorly, any involvement of the cricoarytenoid joint
could lead to impaired vocal fold movement. This involvement can present
752 V. Bandi et al. / Crit Care Clin 18 (2002) 749–765

as pain, the sensation of having a foreign body in the throat, dysphagia, and
stridor. In chronic cricoarytenoid arthritis, mobility of the vocal fold is impaired
because of ankylosis of the joint. Rheumatoid arthritis (RA), systemic lupus ery-
thematosus (SLE), and relapsing polychondritis are the common causes of crico-
arytenoid arthritis.

Mucosal problems

Laryngeal involvement in RA
Laryngeal involvement in RA was first reported in the nineteenth century [2].
In post mortem studies, the prevalence of arthritis of cricoarytenoid joint
has varied between 45% and 88% [3– 5]. Geterud et al [6] studied 29 patients
with RA and 30 controls to assess the prevalence of laryngeal involvement and
the occurrence of extrathoracic airway obstruction in the disease. Laryngeal in-
volvement was assessed by physical examination, including direct fiberoptic
endoscopy, respiratory function tests, and low-voltage radiography. Physical
examination revealed laryngeal involvement in 17 patients with RA (59%);
extrathoracic airway obstruction was indicated by spirometry in 4 patients (14%);
and radiography revealed pathologic findings in 3 patients. One or more signs of
laryngeal involvement were found in 20 patients (69%). Seventy-five percent
of patients with laryngeal involvement had symptoms of breathing difficulties.
Lawry et al [7] reported in a separate study that abnormalities were detected with
indirect laryngoscopy in 32% of study participants with RA; with computed to-
mography (CT), abnormalities were found in 54%. Symptoms such as sore throat
and difficulty during inspiration were predictive of abnormalities detected by in-
direct laryngoscopy [7]. The predominant involvement of the larynx in RA is
secondary to cricoarytenoid joint disease. Even though cricoarytenoid arthritis
primarily affects patients with long-standing RA, it can also be present in patients
whose disease is newly diagnosed [8– 11].
Acute cricoarytenoid arthritis can present during an exacerbation of RA and
occasionally may be present at the initial diagnosis of RA [8,10]. Cricoaryte-
noid arthritis typically presents as a chronic arthropathy with slow progression
of airflow obstruction and an abrupt worsening of symptoms [12,13]. Chronic
presentation of cricoarytenoid arthritis is much more common and often goes
unrecognized, because the symptoms are nonspecific and correlation with the
endoscopic and radiologic evaluation is poor. Patients with chronic cricoaryte-
noid arthritis may be relatively asymptomatic at rest and during periods of
relatively good health. Any disease process that leads to increased airflow needs
(exertion, acidosis, infection) or reduction in the diameter of the airway (upper
respiratory infection) can precipitate sudden worsening [14]. The histology of
cricoarytenoid arthritis reveals synovial thickening with or without destruction of
the articular cartilage, synovial villous perforation, and pannus formation [4].
Patients with unrecognized cricoarytenoid arthritis are at risk during intubation
 V. Bandi et al. / Crit Care Clin 18 (2002) 749–765 753

and after extubation. Intubation, even for a short period of time, can lead to
mucosal edema and further compromise of the airway caliber, leading to stridor
and airway obstruction after extubation [15]. Laxity of joint capsule and large
synovial folds are noted to be the predisposing factors for intubation trauma of
the cricoarytenoid joints, potentially leading initially to hemarthrosis and finally
to cricoarytenoid joint dysfunction [16]. Wattenmaker et al [15] reviewed 128 in-
tubations for surgical procedures and concluded that the incidence of postextu-
bation stridor is 1% when using fiberoptic laryngoscopy and 14%when using
direct laryngoscopy.
Treatment for cricoarytenoid arthritis depends on the degree of airway ob-
struction. If the patient has severe airway obstruction, the airway must first be
secured. This process is best accomplished in an operating room with a surgeon
standing by in case the attempt to secure the airway by awake fiberoptic intubation
fails and a tracheostomy is required. If the patient has stridor but is still able to
maintain ventilation, the work of breathing can be lessened and the airflow can be
improved with the use of 80:20 helium:oxygen mixtures. Systemic glucocorti-
coids are often effective in reversing the obstruction in acute cricoarytenoid
arthritis [12]. Local periarticular steroid injections have been shown to improve
cricoarytenoid function [9,17]. In chronic cricoarytenoid arthritis the degree of
airflow limitation dictates the need for surgical intervention. One approach to
treating this condition involves lateralizing one of the affected vocal folds, either
using an endoscope or by laser cordotomy or arytenoidectomy [18,19] Another
approach is to establish a long-term tracheostomy for maintaining the airway.
In contrast to adult RA, cricoarytenoiditis is an unusual manifestation of
juvenile onset RA [20]. The published literature reveals that cricoarytenoiditis of
the pediatric airway is more likely to be acute and to require intubation [21 – 23].
A possible explanation for the age-related difference in cricoarytenoid symptoms
is that children have relatively narrow glottic openings, larger arytenoids, and a
greater amount of loose areolar tissue. These anatomic features allow more inflam-
matory swelling to occur around the epiglottis and arytenoids in a young child than
in an adult [23].

Implications for the intensivist

Cricoarytenoid arthritis should be suspected in RA patients presenting with
shortness of breath, asthma, or hoarseness of voice [24]. Pulmonary function tests
with flow volume loops may reveal blunting of the inspiratory loop in patients
with variable extrathoracic obstruction and flattening of the inspiratory and
expiratory loops in patients with fixed obstruction. These tests are not highly
sensitive or specific. If the index of suspicion is high, the best way to diagnose
cricoarytenoid arthritis is by flexible rhinolaryngoscopy. When a patient with
RA needs intubation, awake, fiberoptic intubation should be considered [15,23,
25 –27]. In the event of severe airway compromise, intubation should be at-
tempted only in the operating room with a surgeon standing by. Laryngeal mask
airway has been used for short periods of airway control with some success [28].
754 V. Bandi et al. / Crit Care Clin 18 (2002) 749–765

When the patient is ready for extubation, adequate preparation should be made
for management of postextubation stridor, should it occur.

Temporomandibular joint abnormalities in RA

The true incidence of temporomandibular joint (TMJ) abnormalities in RA
has not been established. Most rheumatologists agree that signs and symptoms of
TMJ dysfunction are common in RA. Arthritis in the TMJ can reduce the pa-
tient’s ability to open the mouth [4]. In a study of 65 patients with long-standing
RA, 55% had pain and tenderness in the TMJ, and 66% had limited TMJ
mobility (< 45 mm) [29]. In addition, 78% demonstrated radiographic evidence of
deformity, with 59% of these joints showing erosions. Redlund-Johnell [30]
reported that upper airway obstruction occurs significantly more often in patients
with arthritis of the TMJ than in patients with normal joints. Episodes of airway
obstruction were noted in 70% of patients with a severe arthritic destruction of
the TMJ. Upper airway obstruction was assumed to occur in these patients be-
cause of pharyngeal obstruction, as in other patients with micrognathia or sleep
apnea syndrome.

Rheumatoid nodules of the larynx

Although 25% of patients with RA have nodules, it is rare to see rheumatoid
nodules in the larynx. Typically, the symptoms associated with laryngeal rheu-
matoid nodules are hoarseness of voice and difficulty swallowing, both of which
are insidious in onset. Laryngeal nodules are located predominantly on the vocal
folds or vestibular folds. Laryngeal nodules do not differ histopathologically
from the subcutaneous nodules. Rheumatoid nodules by themselves do not cause
serious airway problems [31 – 34], and the symptoms produced depend on their
physical location. Patients treated with methotrexate are at an increased risk for
developing rheumatoid nodules, but it is not clear whether this observation ex-
tends to the nodules in the larynx [35].

Atlantoaxial instability
Atlantoaxial instability is present in 25% of all patients with RA. It is more
frequent in men and is independent of the duration of the disease and the pa-
tient’s age. Atlantoaxial instability is more likely to manifest in patients with
severe peripheral rheumatoid involvement. Subluxation is more common than
dislocation, and the symptoms are related to the degree of instability or ‘‘slip’’
[36]. The first cervical vertebra (C1) has no body or spinous process. It is ring-
like, with an anterior and a posterior arch and two lateral masses. The second
cervical vertebra (C2) forms the pivot over which the first vertebra rotates. The
odontoid process is an extension of the body of the second cervical vertebra and
projects into the ring of the C1 just posterior to the anterior arch of the C1. The
transverse ligament attaches to both lateral masses of the C1 behind the odontoid
 V. Bandi et al. / Crit Care Clin 18 (2002) 749–765 755

process and protects the cervical spinal cord, which lies immediately behind it.
There is a synovial bursa between C1 and the odontoid process, and involvement
of the bursa leads to weakening of the ligamentous structure and instability of
the spine.
Symptoms of instability at the C1-C2 level include upper neck pain, headaches,
muscle weakness, incontinence, vertigo, nystagmus, dysphoria, and death. Symp-
toms correlate poorly with radiographic findings, and neurologic impairments
correlate poorly with instability. Studies have reported a prevalence of cervical
spine involvement in 15% to 86% of patients with RA [37]. One community-based
study using plain radiography detected anterior atlantoaxial subluxation in 33% of
patients with RA [38,39]. The study also detected that 27% of patients had vertical
dislocations, 14% had lateral subluxations, 2% had posterior subluxations, and
21% had subaxial subluxations [38,39]. Other studies have reported that up to
73% of patients develop atlantoaxial subluxation within 10 years, and nearly 20%
develop significant subaxial disease [40]. Campbell [41] retrospectively reviewed
the role of routine cervical spine films in 128 asymptomatic RA patients under-
going elective orthopedic surgery and found that the incidence of unsuspected
C1-C2 subluxation was 5.5%. He concluded that preoperative cervical spine
assessment in asymptomatic patients with rheumatoid disease is unnecessary
before elective orthopedic surgery. Symptoms related to subluxations can vary
over time, but it is surprising how rarely any symptoms are recognized. Overall,
10% of patients with unrecognized atlantoaxial subluxation may die from spinal
cord or brainstem compression [42]. Symptoms of cervical spine involvement
include nonspecific neck pain and stiffness. Impingement of the second cervical
nerve root causes occipital headache. Cervical myelopathy usually presents with
paresthesias in a stocking-glove distribution and may be confused with peripheral
neuropathy. Spastic paraparesis and abnormal bowel or bladder function are late
findings. Progressive erosive cervical spine disease could lead to acquired
laryngeal deviation that may result in difficult intubation [43].

Implications for the intensivist

The intensivist should be aware of the possibility of cervical spine involve-
ment in a patient with RA. If possible, a thorough history should be taken. If the
patient is awake, alert, and cooperative, a simple ‘‘Sharp and Purser’’ clinical test
may be of help [44,45]. The practice of routine radiographic evaluation of the
cervical spine in all patients with RA is controversial [41,46]. Because cervical
entrapment is common even without signs or symptoms, intensivists should rea-
sonably assume that patients with RA are at increased risk for neurologic injury
from cervical manipulation and therefore should take the appropriate precautions.
These measures include avoiding hyperextension and maintaining the cervical
spine in midline without extension. On occasions one should resort to awake
fiberoptic intubation as a safe alternative. The study conducted by Wattenmaker
et al [15] indicates that fiberoptic intubation is safer than routine laryngoscopic
intubation in patients with RA.
756 V. Bandi et al. / Crit Care Clin 18 (2002) 749–765

SLE
Laryngeal involvement in lupus ranges from 1% [47] to 30% [48]. The
symptoms may be caused by mucosal, submucosal, or serosal involvement. In the
acute phase of SLE, mucosal ulceration, edema, and submucosal hematomas may
cause hoarseness and throat pain. Epiglottitis has been reported secondary to
mucosal involvement. Infectious complications of the glottis have also been
reported. Late effects of the disease include corditis, mucosal thickening, laryn-
geal scarring with stenosis, and laryngitis sicca with dry, thickened vocal cords.
Serosal effects of the disease process in the larynx include vocal cord paralysis,
perichondritis, and cricoarytenoid arthritis [48].

Mucosal inflammation
Oral ulceration, laryngeal erythema, and edema are the most common gross
clinical findings. Patients can also present with painless hard palate ulcers. Three
percent to 5% of patients will have nasal septal perforation. Generally, these
findings respond rapidly to corticosteroid therapy, suggesting that the laryngeal
process is secondary to SLE. Chronic mucosal involvement may result in
laryngitis, corditis, or laryngeal scarring with stenosis [48,49].

Infection
Laryngeal inflammation and edema are responsive to steroid therapy, except in
cases in which infection is documented. The typical pathogens involved are
Haemophilus influenzae and streptococcal infections. In developing countries,
diphtheria, laryngeal tuberculosis, trichinosis, syphilis, and leprosy need to be
considered. Scleroma of the larynx, a chronic granulomatous infection caused
by Klebsiella rhinoscleromatis, can cause progressive upper airway obstruction.
Histoplasma, Coccidioides, Blastomycosis, Candida, and Cryptococcus have also
been implicated in laryngeal inflammation in patients with SLE [50].

Vocal cord paralysis

Bilateral and unilateral vocal cord paralysis has been described with SLE [51].
Occasionally, this paralysis responds to corticosteroid treatment of the underlying
SLE. Vasculitis of the vasa nervorum has been hypothesized to be the cause of
steroid responsive vocal cord palsy in these patients.

Cricoarytenoid arthritis
Even though numerous cases of cricoarytenoid arthritis of the larynx leading
to respiratory difficulty have been reported, it is not as common a problem in SLE
as in RA. Some of these reports involve patients with RA and SLE overlap
disease [52]. Acute enlargement of the parotid glands may occur in approx-
imately 10% of patients with SLE and could lead to potential problems with
the airway [53].
 V. Bandi et al. / Crit Care Clin 18 (2002) 749–765 757

Inflammatory masses have been described in the larynx of patients and have
led to shortness of breath, respiratory failure, and even death. On rare occasions,
inflammatory masses have led to laryngeal abscess and mediastinitis [54].
Rare cases of drug-induced lupus leading to hoarseness, bilateral vocal cord
fixation, stridor, and oral ulceration have been reported [55 – 57].

Angioedema
Angioedema can occur in patients with SLE because of C1 esterase inhibitor
deficiency, hypocomplementemic urticarial vasculitis syndrome, or drug therapy.
The drugs implicated in angioedema in SLE are nonsteroidal anti-inflammatory
agents and angiotensin-converting enzyme inhibitors. Of the two, angiotensin-
converting enzyme inhibitors are much more likely to cause angioedema, which
can occur at any time during the treatment course [58]. Angioedema is associated
with dose titration and long-acting angiotensin-converting enzyme inhibitors.
Signs and symptoms of angioedema are swelling of the lip or tongue, inability to
swallow, drooling, odynophagia, stridor, and respiratory compromise. On rare
occasions the airway needs to be secured until the swelling resolves. It is im-
portant to secure the airway before the angioedema worsens. Securing the airway
is best accomplished in the operating room using awake fiberoptic intubation
with surgeons standing by in the event of an emergency. Airway control, sup-
portive care, and withdrawal of the offending agent are the only necessary treat-
ment options.

Scleroderma
Scleroderma frequently involves the head and neck. Upper airway involve-
ment is rare compared to RA and SLE. Eighty percent of patients with
scleroderma have head and neck involvement, and the head and neck manifesta-
tion is the initial symptom of the illness in 30% of these patients [59]. Facial skin
involvement is prominent, and in 20% of these patients, decreased ability to open
the mouth is the initial presenting complaint (followed by the distal dysphagia).
Thirty-five percent of patients eventually develop tight facial skin, 18% develop
telangiectasia, and 3% develop fascial calcinosis. Fascial calcinosis can severely
limit the ability to open the mouth, in turn posing a problem during airway access
[60]. There is significant alteration in oral structure and function, with 8% of the
patients having xerostomia. Extensive parotitis, gingivitis, and advanced dental
disease are common. Almost 49% of patients complain of a change in the voice,
but only a minority have cricoarytenoid involvement. Gastroesophageal reflux
is present in 90% of the patients and leads to pulmonary disease along with
laryngeal problems. Indirect laryngoscopy reveals interarytenoid or vestibular
fold edema in most patients. The skin and connective tissue changes of systemic
sclerosis frequently result in marked narrowing of the mouth; telangiectasia in the
nasal passages; tightened skin, which limits mobility of the neck; and hardening
758 V. Bandi et al. / Crit Care Clin 18 (2002) 749–765

of the tissue in the submental triangle, which limits the ability to align the oral,
pharyngeal, and laryngeal axis during laryngoscopy [61,62]. Venous access and
blood pressure monitoring can be difficult in these patients.

Wegener’s granulomatosis
Wegener’s granulomatosis is a well-defined syndrome of necrotizing granu-
lomatous vasculitis of the upper airway, lower airway, and the kidney (with less
frequent involvement of other organs). Almost all patients with Wegener’s gran-
ulomatosis initially have symptoms related to the upper respiratory tract, and
most eventually develop some kind of upper airway involvement [53]. More
than 90% of the patients have involvement of nose or paranasal sinuses with
mucopurulent rhinorrhea. Nasal collapse and saddle-nose deformity are noted in
50% of these patients. Inflammatory changes in the nasopharynx are common
[63]. The larynx and trachea may be involved in 24% of the patients, with
symptoms ranging from hoarseness and sore throat to dyspnea and stridor
[63 –67]. Subglottic stenosis is a major life-threatening upper airway mani-
festation of Wegener’s granulomatosis and occurs in approximately 20% of pa-
tients. Patients who are less than 21 years of age at the onset of Wegener’s
granulomatosis seem to develop subglottic stenosis more frequently [68]. Fifty-
one percent of the patients present with subglottic stenosis during an exacer-
bation of the disease. Tracheal involvement in Wegener’s is usually localized to a
narrow region of the subglottis just below the vocal folds [69]. Compromise to
the tracheal lumen can result from acute inflammation, scar formation, or both
[68]. Seventy-nine percent of patients with subglottic stenosis present with
dyspnea, 61% with voice changes and hoarseness, and 22% with cough. In
the series reported by Langford et al [70], tracheostomy was required in 42%
of the patients, and 26% required no surgical therapy for management of their
subglottic stenosis. Fifty percent of the patients were treated with intratracheal
dilation-injection therapy. The median number of dilation-injection treatments
was three (range, 1– 21). Carbon dioxide laser resections and laryngotracheo-
plasty are other less-effective alternatives. Vocal fold paralysis can occur in
Wegener’s granulomatosis as result of vasculitis affecting the cranial or periph-
eral nerves [1].

Relapsing polychondritis
Relapsing polychondritis is a rare disorder characterized by recurrent epi-
sodes of inflammation of the cartilaginous and connective tissue. The asso-
ciation of relapsing polychondritis with other connective tissue diseases and the
occasional finding of antibodies to type II collagen suggest an autoimmune
origin. McAdam et al [71] have proposed that the diagnosis of relapsing poly-
 V. Bandi et al. / Crit Care Clin 18 (2002) 749–765 759

chondritis can be made when three or more of the following manifestations

are present:

Bilateral auricular chondritis

Nonerosive seronegative inflammatory polyarthritis
Nasal chondritis with saddle nose deformity
Ocular inflammation
Respiratory tract chondritis
Vestibular damage

Involvement of the respiratory tract is of critical importance because of the high

morbidity and mortality associated with this complication [72]. Respiratory
distress as an initial symptom is reported in 18% of cases, with eventual
respiratory tract complications arising in up to 50% of patients. Laryngotracheal
involvement is well recognized. Laryngotracheal strictures are a poor prognos-
tic sign in persons 50 years of age or younger. The subglottic strictures tend to
occur more frequently in the younger age group and predominantly in women.
Respiratory tract involvement apart from the nasal cartilage has been described at
the level of the glottis or supraglottic structures, the trachea, and the first and
second order bronchi. Symptoms of airway involvement include cough, hoarse-
ness, aphonia, choking sensation, dyspnea, and tenderness over the thyroid car-
tilage and anterior cervical trachea. Airway obstruction can occur by several
mechanisms: (1) encroachment on the airway by inflammatory swelling in the
active stage of the disease; (2) the formation of a mass of fibrous tissue with
cicatricial contraction during the later stages of the disease; or (3) dissolution of
the tracheobronchial cartilage, with subsequent collapse of the airway during
respiration [73,74]. Thick secretions in the presence of glottic obstruction have
also been reported to increase morbidity [75]. Tracheal or bronchial obstruction
may be complicated by pulmonary infection because of impaired mucociliary
clearance, diminished effectiveness of coughing, pulmonary atelectasis, and
collapse. Airway involvement has been the main cause of death in most cases
in the series reviewed [76]. Medical management includes treatment with cor-
ticosteroids and immunosuppressive agents. Cyclosporine A and plasmapheresis
have been used with some success. Surgical management includes tracheostomy
in patients with localized subglottic involvement. Endoscopy and intubation are
fraught with hazard and have led to death on occasion. In indicated patients,
tracheostomy should be performed early instead of attempting bronchoscopy or
intubation. Tracheostomy itself may carry a serious risk by inducing fatal airway
obstruction. The small glottis resulting from cartilaginous destruction may make
intubation difficult, and a smaller endotracheal tube may have to be used. Once
intubation has been achieved, adequate ventilation may be maintained even in
patients with complete reversible airway collapse with the use of positive pressure
ventilation. Endotracheal prostheses such as Montgomery T-tubes (Boston Medi-
cal Products, Inc., Westborough, MA) and stents have been used [77]. Dynamic
obstruction from central airway collapse may respond to airway splinting [76,78].
760 V. Bandi et al. / Crit Care Clin 18 (2002) 749–765

Ankylosing spondylitis
Ankylosing spondylitis is an inflammatory arthropathy with infiltration of gran-
ulation tissue into bony insertions of ligaments and joint capsules. Relatively few
patients develop the complete ankylosis, the so-called ‘‘bamboo’’ spine [79]. If
the duration of the disease is 16 years or more, 75% of patients develop cervical
ankylosis and have a high risk of cervical fractures [80]. Many patients with
ankylosing spondylitis also have extraspinal joint involvement and nonarticular
manifestations of the inflammatory process [79]. Of relevance to intensivists are
the involvement of the TMJs, which limits mouth opening; cricoarytenoid arthritis,
which makes the vocal cords more susceptible to trauma [81,82]; and the pul-
monary system, with fibrosis and restricted chest expansion. The clinical spectrum
of ankylosing spondylitis is broad, and many patients with ankylosing spondylitis
can be safely intubated using direct laryngoscopy. Patients with restricted cervical
spine motion or with cervical spine subluxation or fractures should undergo awake
flexible fiberoptic intubation. The tracheal intubation of a patient with fixed
kyphosis of the cervical spine can be difficult, especially if the TMJs are also in-
volved. These patients are also at increased risk for occult cervical fracture. A
minor trauma such as falling out of bed, driving a motorcycle on rough terrain, or a
simple fall to the floor is sufficient to fracture an ankylosed spine. Because the
offending trauma is trivial, the patient may not remember these events. There are
case reports of cervical fractures occurring solely because of tracheal intubation.
Most cervical lesions occur through the intervertebral disc space between C5 and
C7, as has been shown in an experimental model of cervical fractures. After
transection of the ligaments connecting C5 and C6, every movement of the head
ends in dislocation or fracture. Any chin lift, jaw thrust, use of an esophageal
obturator airway, or orotracheal intubation pushes the cranial fragment of the
cervical spine ventrally. In contrast, in the blind nasal approach, the cranial frag-
ment can be dislocated dorsally simply by pushing the head onto the table for
stabilization. The use of a cervical collar does not prevent dislocation and serves
only as a sign of an unstable cervical spine [83]. Tracheal intubation of the patient
with ankylosing spondylosis is best achieved by techniques that minimize neck
movement [84]. Awake transnasal or transoral fiberoptic intubation or awake
retrograde intubation can be used safely [80,84 – 86] The laryngeal mask airway is
another option for ventilation during surgery and procedures of short-duration [87].

Sjögren’s syndrome
The cardinal manifestation of Sjögren’s syndrome is xerostomia. Dry mucosa,
oral ulcerations, thick, tenacious mucus, and a fissured, erythematous tongue are
the oropharyngeal features. Premature caries necessitating complete dental replace-
ment by the third decade is commonly seen in Sjögren’s syndrome. Nasal crusting
and perforation of the nasal septum have also been reported [53]. Salivary gland
involvement can be unilateral or bilateral and, on occasion, massive, causing
 V. Bandi et al. / Crit Care Clin 18 (2002) 749–765 761

airway compromise [88]. Airway compromise resulting from dryness, crusting,

tenacious mucus, ulcerations, and swelling of vocal folds has been described [89].
Lymphocytic infiltration and pseudolymphoma of the larynx have also been
reported [90].

Summary
The intensivist should be aware of the upper airway manifestations of the
common rheumatologic disorders which may lead to ICU admission or which may
potentially pose a problem during airway management. Information should be
obtained from the patient, the patient’s family, and the patient’s primary physician,
if possible. One should be fully prepared with various options in case a problem
arises with an airway. Equipment for managing a difficult airway should be
available. Alternate methods of managing the airway (eg, the laryngeal mask
airway, fiberoptic scopes, and the WU Scope) (Achi Corporation, Fremont, CA) are
of great help in dealing with airway problems. The potential for cervical spine
instability exists in patients with rheumatologic disorders. Intubating with care and
avoiding spinal movement both seem to be more important than any particular
mode of intubation in preserving neurologic function. One should make a
concentrated and serious effort to be as gentle as possible and to avoid even
minimal trauma to the mucosa in these patients, because they are at risk for mucosal
edema and subsequent postextubation stridor. In cases of stridor, helium-oxygen
mixtures may be of help and may eliminate the need for reintubation. When
difficulty in establishing an airway is anticipated, it is prudent to attempt airway
control in the operating room with surgical assistance standing by should cervical
tracheotomy is required.

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