J Rheum Dis.  2016 Jun;23(3):174-178. 10.4078/jrd.2016.23.3.174.

Outcome of Localized Granulomatosis with Polyangiitis: A Case Study

• Choe AR
• Kim IJ
• Lee J
• Sung SH

• KMID: 2326732
• DOI: http://doi.org/10.4078/jrd.2016.23.3.174

Abstract

OBJECTIVE
A substantial portion of granulomatosis with polyangiitis (GPA) patients present with localized disease limited to the upper respiratory tract, however; disease spectrum and prognosis of these patients are unclear. The aim of this study is to describe the clinical characteristics and outcome of patients with localized GPA.
METHODS
This was a retrospective descriptive case series of patients with a biopsy proven localized GPA presenting to a single tertiary rheumatology service between January 1995 and September 2015.
RESULTS
A total of 5 patients, median age 56 years (range 48 to 59 years) at diagnosis and 80% female, were identified. The median follow-up period was 42 months (range 15 to 62 months). Diagnosis was delayed with median time to diagnosis of 12 months (range 3 to 36 months), and patients underwent 1-3 ear, nose, and throat surgeries during the period of diagnostic delay. Sinusitis was the most frequent symptom in all patients, followed by otomastoiditis with cranial nerve palsies (n=2) and orbital mass (n=1). Antineutrophil cytoplasmic antibody (ANCA) was positive initially in 2/5 patients (40%). Two patients with otomastoiditis and cranial nerve palsies progressed to systemic disease with ANCA positive conversion. These two cases along with a case with orbital mass were refractory to standard treatment of cyclophosphamide with glucocorticoids requiring rituximab treatment.
CONCLUSION
Patients with localized GPA may progress to systemic disease over the disease course, and may have aggressive disease refractory to standard treatment. Close monitoring for systemic symptoms and repeated ANCA testing is required in patients with localized GPA.

Keyword

Granulomatosis with polyangiitis; Localized; Outcome

MeSH Terms

Antibodies, Antineutrophil Cytoplasmic
Biopsy
Cranial Nerve Diseases
Cyclophosphamide
Diagnosis
Ear
Female
Follow-Up Studies
Glucocorticoids
Granulomatosis with Polyangiitis*
Humans
Nose
Orbit
Pharynx
Prognosis
Respiratory System
Retrospective Studies
Rheumatology
Rituximab
Sinusitis
Antibodies, Antineutrophil Cytoplasmic
Cyclophosphamide
Glucocorticoids
Rituximab

Figure

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  Figure 1. Case 1 in 2012. Computed tomography of paranasal sinus image showed mass extends to the intraorbital portin of the left oribt (arrow). Soft tiss densities along the both maxillary and frontoethmoidal sinuses.

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  Figure 2. Case 1 in 2013. Naso-pharyngeal biopsy revealing granulomatous inflammation with palisading necrosis (A), and vasculitis (B) (H&E, ×200).

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  Figure 3. Case 3 in 2014. Gadolinium enhanced T1-weighted sagittal magnetic resonance image showed diffuse enlargement of pituitary gland (11.5 mm in height) with thickened enhanced pituitary stalk (arrow).

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