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QJM: An International Journal of Medicine, 2017, 1
doi: 10.1093/qjmed/hcx182
Advance Access Publication Date: 22 September 2017
Clinical picture
Saddle nose deformity and granulomatosis with
A 34-year-old Caucasian woman presented with recurrent episodes of acute otitis media. Despite multiple rounds of antibiotics,
her symptoms remained unresolved, so she was referred to ENT
where she underwent myringotomy and eventually mastoidectomy. Within the first 6 months, she developed fatigue, fever and
polyarthritis in her hips, knees and hands. By 7 months, she developed facial pain, purulent sinusitis, epistaxis and saddle nose.
Within a week, her saddle nose rapidly progressed to complete collapse of the nasal bridge (Figure 1A and B). Labs revealed elevated
erythrocyte sedimentation rate (79 mm/h) and C-reactive protein
(39.6 mg/l), positive cytoplasmic antineutrophil cytoplasmic antibodies (1:40) and proteinase-3 antibody (PR-3; 28.3 U/ml) consistent
with granulomatosis with polyangiitis (GPA). She was initiated
on prednisone followed by rituximab. Clinically, she is improving
with near-resolution of symptoms and normalization of PR-3.
GPA is a rare autoimmune small- and medium-vessel vasculitis that typically affects the upper respiratory tract, lungs and
kidneys. Even though 63% of patients initially present with isolated ENT manifestations, most of these patients faced a delayed
diagnosis due to the perceived absence of symptoms involving
other organs.1 Saddle nose deformity is a well-recognized
hallmark of GPA but more commonly occurs in trauma, cocaineinduced necrosis and sarcoidosis.2 Treatment consists of immunosuppressants and is tailored to the severity of disease:
methotrexate for mild disease, glucocorticoids plus rituximab or
cyclophosphamide for organ and life-threatening manifestations
and rarely plasma exchange in hemoptysis and renal failure.
Photographs and text from: Byung Hoon Ban, Department of
Internal Medicine, University of Tennessee Chattanooga, 975
East 3rd St., Chattanooga, TN, USA; Jayne Littlejohn Crowe,
Department of Rheumatology, University of Tennessee
Chattanooga, Chattanooga, Tennessee, USA; Maria Tudor,
Department of Internal Medicine, University of Tennessee
Chattanooga, Chattanooga, TN, USA. email:
Conflict of interest: None declared.
1. Srouji IA, Andrews P, Edwards C, Lund VJ. Patterns of presentation and diagnosis of patients with Wegeners granulomatosis:
ENT aspects. J Laryngol Otol 2006; 121:653–8.
2. Pakalniskis MG, Berg AD, Policeni BA, Gentry LR, Sato Y,
Moritani T, et al. The many faces of Granulomatosis with
polyangiitis: a review of the head and neck imaging manifestations. Am J Roentgenol 2015; 205:W619–29.
Figure 1. Front (Panel A) and lateral (Panel B) views of the patient’s saddle nose deformity after the nasal bridge has completely collapsed from immune-mediated
inflammation and destruction.
C The Author 2017. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved.
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