|
Wegener's
granulomatosis: current trends in diagnosis and management. Curr
Opin Otolaryngol Head Neck Surg.2007;15(3):170-6.
PURPOSE OF
REVIEW: To provide an update on diagnostic methods and treatment
options for Wegener's granulomatosis and to review common head and
neck manifestations of the disease. RECENT FINDINGS: Recent advances
have been made in the systemic treatment of Wegener's granulomatosis,
including the introduction of investigational immunosuppressive
agents such as etanercept, leflunomide and deoxyspergualin. Surgical
options remain indicated in selected complications of Wegener's
granulomatosis such as saddle nose deformity and subglottic stenosis.
SUMMARY: Wegener's granulomatosis is an idiopathic, systemic
vasculitis characterized by the formation of necrotizing granulomas
of the respiratory tract in addition to focal or proliferative
glomerulonephritis. Diagnosis is made by a combination of physical
examination, laboratory studies and tissue biopsy. Head and neck
manifestations are abundant and varied; common sites of involvement
include the middle ear, nose and sinuses and subglottis. The
mainstay of treatment remains systemic therapy using a combination
of glucocorticoids and immunosuppressants. The otolaryngologist
plays a key role in the diagnosis and treatment of head and neck
complications of the disease. A surgical role exists for the
management of nasal and sinus disease as well as laryngeal and
tracheal disease.
Head and neck manifestations of Wegener's
granulomatosis. Rhinology. 2006 Dec;44(4):227-33.
Wegener's
Granulomatosis (WG) is a necrotizing granulomatous angiitis that
presents the classic ELK triad of ear, nose, throat (E), lung (L),
and kidney (K) involvement. Its potential rapid and fatal outcome
makes the early recognition--before irreversible organ involvement
occurs--mandatory. The aetiology is still unknown. Today,
immunosuppressive therapy makes WG a treatable disease with a
chronically relapsing course. The otorhinolaryngologist plays an
important role in early diagnosis of WG, because in up to 95% of the
patients initial WG symptoms are observed in the head and neck
region. The majority of these patients show nasal or sinunasal
involvement. Common manifestations are sinusitis, crusting of the
nose, and development of saddle nose deformity. Other head and neck
problems are middle and inner ear symptoms and subglottic stenosis.
Follow up and activity assessment of the disease are also important
roles to play for the otorhinolarygologist.
Wegener's
granulomatosis presenting as otomastoiditis. A case report.
B-ENT. 2006;2(1):7-12.
A
55-year-old male presented with left-sided otorrhoea, hearing loss
and tinnitus of 3 months duration. On clinical examination polypoid
tissue was seen prolapsing in the external ear canal. A CT scan of
the mastoid cells and middle ear showed otomastoiditis with
osteolysis. Oral antibiotic therapy and eardrops were started. When
a facial nerve paresis appeared one month later, a mastoidectomy was
performed. The mastoid cells and middle ear were filled with a
connective tissue-like substance. Postoperative corticosteroids were
administered. Despite the therapy the facial nerve problem
aggravated and the patient developed severe parietotemporal
headache, meningeal irritation and somnolence. The diagnosis of
neurosarcoidosis was hypothesised. Blood analysis, including c-ANCA's,
culture of the otorrhoea and biopsies of the connective tissue were
inconclusive. A CT scan of the brain showed thickening of the left
tentorium. A biopsy of the dura indicated a diagnosis of Wegener's
granulomatosis. The patient was treated with immunosuppressive
medication with satisfactory results.
An unusual
case of otitis media. J Am Acad Audiol.2005 ;16(8): 596-9.
This case
report of bilateral otitis media in a 39-year-old woman secondary to
Wegener's Granulomatosis highlights, for the audiologist, the
importance of being aware of some of the less common etiologies of
middle ear disease. Bilateral otitis media that resists usual forms
of medical treatment may represent one of the earliest signs of
Wegener's Granulomatosis, a potentially life-threatening disease.
According to recent literature, involvement of the ears, nose, and
throat can in many instances be the only early manifestation of the
disease. It is important that audiologists be aware of the early
signs in order to make appropriate referral for treatment,
particularly since they are usually among the first to see these
patients. Early diagnosis and referral is critical since the mean
survival of untreated WG is five months, with 82% of patients dying
within one year, and more than 90% dying within two years. The
disease involves a systemic vasculitis that may involve any organ
system: however, pulmonary or renal disease appear to be among the
later signs, while both middle ear and upper respiratory involvement
are frequently among the earliest.
How can the
diagnostic value of head and neck biopsies be increased in Wegener's
granulomatosis: a clinicopathologic study of 49 biopsies in 21
patients. Ann Pathol. 2005 Apr;25(2):87-93.
Head and
neck biopsies usually have a low diagnostic value in Wegener's
granulomatosis (WG). On the basis of 49 biopsies obtained from 21 WG
patients at diagnosis from various sites, i.e. nose (29), paranasal
sinus (7), oral cavity (4), larynx (4), conjunctiva (3) and external
ear (2), we described the suggestive histological features and
studied the diagnostic potential of the biopsy size, anaesthesia
method (general (GA) or local (LA)), anatomic region of the biopsy,
number of sections, and presenting macroscopic manifestations.
Associated granulomatous inflammation (scattered giant cells, 28.5%
of biopsies; poorly-formed granulomas, 28.5%), necrosis (neutrophilic
microabscesses, 16.3%; geographic necrosis, 18.3%), angiitis (leukocytoclastic,
10%; necrotizing, 12%; and granulomatous, 6%) which confirmed the
diagnosis were only present in 18.3% of the biopsies (28.5% of the
patients). We think it is possible to propose a "WG-compatible"
diagnosis when at least one of these histological features is
present (24.5% of biopsies, 26% of patients in our study). We found
that it was always better to perform biopsies targeted on
macroscopic lesions. When there was no lesion, samples from
paranasal sinuses obtained under GA had the highest diagnostic value
in the head and neck region, whereas 90% of nasal systematic
biopsies performed under LA were nonspecific. Moreover, we
demonstrated that performing two further sections increased the
sensitivity of histological examination by 7%.
Wegener's
granulomatosis in the head and neck region. HNO. 2004
Oct;52(10):935-45; quiz 946-7.
Wegener's
granulomatosis (WG) is defined as a granulomatous inflammation of
the upper and lower respiratory tract and systemic vasculitis of
small and medium sized vessels which is often accompanied by a
necrotizing glomerulonephritis. The etiology of the disease is still
unknown. In former times untreated WG usually ended deadly.
Immunosuppressive therapy made WG a treatable disease with a
chronically relapsing course. Therefore an early diagnosis of WG is
of utmost importance. WG usually starts as a limited and localized
organ manifestation in the upper respiratory tract and it
generalizes, if untreated, with pulmonary and renal involvement.
Symptoms in the head and neck region are observed in up to 95% of
the patients with WG. Sinusitis, crusting of the nose, development
of a saddle nose, middle and inner ear symptoms and subglottic
stenosis are common manifestations. Due to the early and common
manifestation of WG in the head and neck region the
otorhinolaryngologist plays an important role for the early
diagnosis and the fast initiation of immunosuppressive therapy but
also during follow-up for activity assessment.
Wegener's
granulomatosis (WG) presented with hearing loss and without positive
serologic ANCA. Laryngorhinootologie. 2004 Mar;83(3):180-4.
BACKGROUND:
Wegener's granulomatosis (WG) is a granulomatous inflammation
involving the upper and lower respiratory tract and necrotizing
vasculitis affecting small to medium-sized vessels. In contrast to a
generalised WG with glomerulonephritis initial or isolated forms of
the upper respiratory tract may be a diagnostic challenge. PATIENT:
We report the case of a 33 year old man with clinical signs of a
limited WG exhibiting an imminent irreversible hearing loss,
negative PR3-ANCA (anti neutrophil cytoplasmic antibodies with
proteinase 3 as target) in serum and an ambiguous histology.
CONCLUSION: In case of a chronic otitis media and rhinitis as well
as signs of a labyrinthine deafness a limited form of a WG has to be
taken into account, even with an ambiguous histology and negative
PR3-ANCA. This diagnosis is supported by high inflammation
parameters, e. g. ESR and CRP, exclusion of infectious cause and
response to corticosteroids. A quick therapeutic intervention with
corticosteroids and cyclophosphamide is required in order to
interrupt the vasculitis of the inner ear with consequential
deafness.
Protracted
superficial Wegener's granulomatosis. Australas J Dermatol.2003
Aug;44(3):207-14.
A
27-year-old woman presented with a right infra-auricular
noduloulcerative lesion progressing to a peri-auricular pyoderma
gangrenosum-like ulcer with destruction of her right earlobe over an
8-month period. Similar nodules appeared on the right malar and left
infra-auricular regions. The cutaneous manifestations were
associated with nasal congestion, rhinorrhoea and serosanguineous
nasal crusting. Skin biopsy demonstrated suppurative granulomatous
inflammation. Investigation of both renal and pulmonary function
showed no abnormality. Serological testing revealed a positive
cytoplasmic pattern antineutrophil cytoplasmic antibody with high
proteinase-3 specificity, which in conjunction with the clinical
findings is consistent with a diagnosis of protracted superficial
Wegener's granulomatosis. Initial treatment with prednisone 1
mg/kg/day and azathioprine 100 mg/day resulted in complete
resolution of her lesions. Reduction of the corticosteroid dose
below 0.3 mg/kg/day led to recrudescence of cutaneous and upper
respiratory tract symptoms, at which stage methotrexate was
substituted for azathioprine with rapid induction of remission and
further prednisone withdrawal. Thirty-two months after the initial
diagnosis the patient remains well with no other organ involvement.
Cutaneous
Wegener's granulomatosis: a variant or atypical localized form?
Australas J Dermatol.2003 May;44(2):129-35.
A
74-year-old woman presented with an antineutrophil cytoplasmic
antibody titre-negative, treatment-responsive Wegener's
granulomatosis confined to the integument. She initially presented
with a painful left postauricular nodulo-ulcerative lesion with
chronically discharging sinuses. This lesion was effectively treated
with a short, 3-month course of cyclophosphamide and 24 months of
oral prednisone. After 5 months in remission, she developed further
similar ulcers, in addition to painless nodules on her ankles and
feet bilaterally. These lesions resolved with an extra 32 months of
high-dose oral prednisone therapy before complete remission. At most
recent review, there was no evidence of disease recurrence 21 months
after ceasing all active treatment. Histology demonstrated a
granulomatous inflammation. No systemic disease progression to the
upper respiratory tract, lung or kidney was detected. This case
highlights the importance of being aware of atypical or partial
presentations of Wegener's granulomatosis. This diagnosis needs to
be considered with patients presenting with a culture-negative
chronic ulcer, where malignancy and trauma have been excluded. This
will avoid unnecessary surgery and ensure early diagnosis and
effective treatment of a disease that is disfiguring and usually
fatal if inappropriately treated.
Early
otorhinolaryngological manifestations of Wegener's granulomatosis.
Analysis of 21 patients. Ann Otolaryngol Chir Cervicofac. 2002
Dec;119(6):330-6.
Wegener's
Granulomatosis (WG) is a necrotizing granulomatous vasculitis that
has a strong affinity for the upper respiratory tract. OBJECTIVE: To
retrospectively study the clinical features of
otorhinolaryngological manifestations from 21 WG patients. PATIENTS:
Eleven men and 10 women were studied with respectively a mean age of
62.7 (23-79) and 63.9 (53-73). METHODS: Otorhinolaryngological
manifestations were recorded before and during the course of WG.
RESULTS: Upper respiratory tract involvement occurred in 81% of
cases (17/21 patients) and was isolated in 42.8% of cases (9/21
patients). Nasosinusal manifestations occurred in 71.4% of cases
(15/21) before and at the time of GW diagnosis. They included
bilateral sinusitis (7 cases), nasal crusting (6 cases), purulent
rhinorrhea (5 cases), epistaxis (4 cases), nasal ulcers (2 cases),
nasal congestion with obstruction (3 cases) and 4 cases of nose
deformity (saddle nose or oedema). Otologic manifestations occurred
in 28.5% of cases and were never isolated. They included otitis
media (3 cases), sudden hearing loss (3 cases), tinnitus (1 case),
facial palsy (1 case) and 2 cases of chondritis.
Pharyngolaryngotracheal manifestations occurred in 33.3% of cases
(7/21). Diagnosis of GW was based on positive ANCA test (95.2% of
cases), presence of biologic inflammatory parameters (76% of cases)
and histological features. 29 biopsies from nasosinusal lesions on
17 patients were made. 44.8% of the biopsies were contributive with
at least one histologic feature of the combination including
vasculitis, necrosis and granulomatous inflammation. The best
contributive site of biopsy was the paranasal sinus. CONCLUSION: We
report that otorhinolaryngological manifestations occurred in 81% of
cases before and at the time of GW diagnosis. These findings
indicate that otorhinolaryngologists have a central role to play in
early diagnosis of the disease.
Otologic
manifestations of Wegener's granulomatosis. Laryngoscope. 2002
Sep;112(9):1684-90.
OBJECTIVE/HYPOTHESIS:
To evaluate the clinical features, treatment, and outcomes of
otologic manifestations in Wegener's granulomatosis (WG) treated at
Hokkaido University Graduate School of Medicine, Sapporo, Japan.
STUDY DESIGN: We retrospectively reviewed 15 cases of WG with ear
involvement. METHODS: Twenty-five patients with WG were treated at
Hokkaido University Graduate School of Medicine between 1992 and
2001. Fifteen of these patients had otologic symptoms. We evaluated
the clinical course, method of therapy, and outcomes in all cases.
Diagnosis of WG was made when the patients had clinical findings and
a positive titer of cytoplasmic pattern antineutrophil cytoplasmic
antibodies (c-ANCA), or when there were clear histologic findings.
We also present three case reports. RESULTS: In 15 cases, the most
frequent finding was chronic otitis media. Sensorineural hearing
loss was present in 2 patients. In 7 patients whose otologic
manifestations were the primary involvement of WG, all were
confirmed positive for c-ANCA and were treated with glucocorticoids
and immunosuppressive drugs. Three patients who could be treated
within 1 month of symptom onset showed marked improvement.
CONCLUSIONS: In localized cases, biopsy specimens are often small,
and it is frequently difficult to make a histologic diagnosis. The
prognosis for hearing was poor when appropriate treatment was not
given in the early stages of the disease. Therefore, WG should be
included in the differential diagnosis in cases of atypical
inflammatory states of the ear. Early diagnosis and appropriate
treatment are important to prevent irreversible changes in the
middle ear and inner ear.
Otologic
manifestations presenting and predominating in necrotizing angiitis.
Rev Laryngol Otol Rhinol (Bord). 1989;110(3):249-52.
The authors
report on 5 observations of necrotising angiitis with a specifically
otological onset, namely 4 Wegener's syndromes and one periarteritis
nodosa. In all 5 cases, the initial symptomatology consisted of
otological signs in a feverish context, producing pictures of serous
or sero-purulent otitis. Its resistance to usual forms of therapy
led in 3 cases to the installation of transtympanic aerators, and in
2 cases to the performance of an antro-attico-mastoidectomy. One of
the observations led to the discovery in the middle ear of a
histological aspect specific to the disease, from which it can be
considered that the otologic impairment could be a specific seat of
the disease. The 5th observation is particular in that it was the
otological signs which revealed each time a new progression of the
disease, enabling the immediate implementation of an adapted
treatment prior to the occurrence of the systemic signs. 3
observations proved the difficulty of diagnosis at the initial
stage, when at times only the otological signs are present, together
with the importance of its discovery prior to the occurrence of
systemic lesions that are occasionally irreversible. Any isolated
otological symptomatology in a feverish context, resisting usual
therapies, should evoke the diagnosis, and lead to the performance
of further duly adapted examinations (sedimentation rate, pulmonary
X-ray, proteinuria, hematuria).
Ear, nose, and
throat symptoms in subacute Wegener's granulomatosis. BMJ.1989 Aug
12;299(6696):419-22.
The standard
description of Wegener's granulomatosis emphasises renal failure and
thus a distorted impression may be given. Subacute and even chronic
cases occur, and in these patients the presentation is varied and
often insidious, leading to delay in diagnosis. Twenty two such
patients (13 women and nine men) with a mean age of 44 years were
seen in our connective tissue disease clinic. The mean duration of
symptoms before diagnosis was 3.6 years and the mean duration of
disease 5.9 years (19 years in one patient). All patients had
malaise and ear, nose, and throat symptoms, and most had joint
pains. Impaired renal function was seen in seven patients only.
Tissue biopsy was diagnostic in half of the patients, and
appreciably high titres of antineutrophil cytoplasmic antibodies
were detected in only nine of 18 patients in whom these were
measured. The most useful investigations were neutrophil counts,
chest radiographs, and computed tomography of the sinuses and
orbits. The most effective treatment was with intravenous pulses of
cyclophosphamide. No deaths occurred. At the time of writing two
patients were in remission and no longer being treated and 18
patients were in partial remission on continued treatment. Patients
with subacute forms of Wegener's granulomatosis present with a
variety of clinical features and the insidious presentation often
leads to delay in diagnosis. A history of ear, nose, and throat
symptoms was universal in our patients.
|
