Sabato 27 ottobre 2012 - Pacini Editore

352
TESTA COLLO
Benign nasal polyposis and wegener’s
granulomatosis: an unusual association
G. Crisman1 , I. Riccardo2 , V. Corridore2 , S. Cupillari2 , V. Ciuffetelli1
, G. Calvisi4 , S. Di Rito1 , V. Ciuffetelli, P. Leocata1 1 Anatomia Patologica, Dipartimento di Scienze della Salute, Università
dell’Aquila, L’Aquila, Italia; 2 U.O.C. Otorinolaringoiatria, Università
dell’Aquila, L’Aquila, Italia; 3 U.O.C. Anatomia Patologica, P.O. “SS
Filippo e Nicola”, Avezzano (AQ), Italia; 4 U.O.C. Anatomia Patologica
Ospedale Civile “San Salvatore”, L’Aquila, Italia
Background. Benign nasal polyposis is a chronic inflammatory
disease of the nasal and paranasal sinus mucosa, occurring
in up to 4% of the population, even though its prevalence has
been reported up to 40% in autopsy series. The etiology is still
unclear, but associations with allergy, asthma, infection, cystic
fibrosis, and aspirin sensitivity have been at least suggested. Nasal
obstruction represents the main presenting symptom, even if
it can vary depending on the site and size of the polyps; however,
rhinorrhoea, post nasal drip, anosmia, and rarely facial pain have
been reported as well. Nasal endoscopy reveals single or multiple
grey polypoid masses in the nasal cavity. A combination of medical
therapy and surgery represents the mainstay of treatment and
since recurrences are not uncommon (severe disease recurring
in up to 10% of patients), nasal polyps represent a challenging
diagnosis for the physician.
Wegener’s granulomatosis (WG) represents the most common
systemic antineutrophil cytoplasmic antibodies (ANCA) necrotizing
and granulomatous vasculitis, involving the kidney and the
upper and lower respiratory tract. Clinical manifestations, histological
findings and the presence of c-ANCA in serum represent
the three-pivotal steps to achieve the diagnosis of WG. However,
the American College of Rheumatology criteria also include: oral
and nasal inflammation, abnormal chest radiography (nodules,
fixed infiltrates, or cavities), urinary sediment (hematuria), and
granulomatous inflammation on biopsy.
Material and methods. We report on a case of a 62-year-old man
who presented with one-month history of remittent fever (daily
elevated temperature > 38 C or 100.4 F), not responding to an-
Fig. 1. clinical presentation: nasal polyps and necrotizing mucosa.
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Fig. 2. histological features: a) a granulomatous vasculitis and some
foci of hemorrhage can be observed. (H&E, 4x magnification); B) a
granulomatous vasculitis, prominent lymphoplasmocytic infiltrate,
thrombosis and vascular necrosis are well-identifiable at higher
rmagnification(H&E, 10x magnification).
tibiotic therapy (levofloxacin, piperacillin and tazobactam), and
nasal obstruction. The General Practitioner suggested a severe
sinusitis within the context of a benign nasal polyposis. Nasal
endoscopy revealed a benign nasal polyposis with a necrotizing,
edematous mucosa. Due to the mucosal friability, only few fragments
resulted from the endoscopic biopsy.
Results. Histopathological findings revealed a necrotizing vasculitis
and organizing granulomas of the nasal mucosa in all
specimens. Edema and lymphoplasmocytic infiltrate with focal
lymphoid aggregate formation was observed, as well as many
blood vessels with thrombosis and vascular necrosis within the
necrotic areas. The possibility of WG was considered and serum
level of c-ANCA, and pulmonary and renal examinations have
been performed. Thus, the patient underwent a systemic corticosteroid
treatment and showed a remarkable improvement of his
general health conditions and a complete remission of the nasal
necrotizing lesions within 6 days. Nasal polyposis still persisted,
as expected.
Conclusions. The diagnosis of WG can be established at history,
since laboratory test are non-specific. As a matter of fact,
c-ANCA serum levels support the diagnosis if present but do not
exclude it. Radiology can represent a diagnostic tool as well, but
has many mimics. At the best of our knowledge, less is known
about the association between WG and benign nasal polyposis.
Nasal cavities involvement, as well as sinus, orbit, nose and ears
could also represent a further diagnostic tool in such difficult
cases. Even after complete remission with steroid, recurrences in
the targeted organs have been previously reported thus, a close
follow-up of the patient is recommended.
Our patient presented with severe systemic symptoms in the
context of a benign nasal polyposis and this seems to be a very
unusual presentation of WG. The association between WG and
Benign Nasal Polyposis requires further investigations.
In conclusion, the presence of a refractory remittent fever without
a relevant cause in the context of a common benign nasal polyposis
should lead to an accurate endoscopy and the presence of
a necrotizing mucosa should raise the suspicion of a vasculitic
process.
references
Rijuneeta MS, Panda NK, Bambery P. et al. Nasal Polyposis in Wegener’s
Granulomatosis: A rare presentation. The Internet Journal of
Otorhinolaryngology 2006;4(2).
Beltrán Rodríguez Cabo OE, Tona Acedo G. Role of the ears, nose and
throat specialist in the diagnosis and follow up of patients with primary
vasculitidies. Reumatol Clin 2011;7(Suppl. 3):S7-11.
Mujagic S, Sarihodzic S, Huseinagic H, et al. Wegener’s granulomatosis