|Year : 2020 | Volume
| Issue : 2 | Page : 146-149
Eosinophilic Granulomatosis with Polyangiitis Presenting as Acute Nasal Obstruction
Mansoor C Abdulla
Department of General Medicine, M.E.S. Medical College, Malappuram, Kerala, India
|Date of Submission||01-Apr-2020|
|Date of Decision||13-Jun-2020|
|Date of Acceptance||21-Jun-2020|
|Date of Web Publication||8-Dec-2020|
Mansoor C Abdulla
Department of General Medicine, M.E.S. Medical College, Perinthalmanna, Malappuram - 679 338, Kerala
Source of Support: None, Conflict of Interest: None
A 55-year-old man presented with high-grade fever, nasal obstruction, and erythematous skin rash for 1 week. He had hypertension for 5 years, uncontrolled diabetes mellitus for 6 years, and bronchial asthma for the past 8 years. He was diagnosed to have eosinophilic granulomatosis with polyangiitis (EGPA) based on the clinical, laboratory, and histopathological findings. He was treated with prednisolone 1 mg/kg which was tapered over months. He had immediate relief for his nasal obstruction following steroids. Fever and skin rash subsided later. We present a patient with acute nasal obstruction during the vasculitic phase of EGPA. Nasal manifestations are usually seen during the early phase along with asthma long before the onset of vasculitic phase. Invasive fungal infections of the paranasal sinuses which can have similar presentations also should be ruled out by histopathology in such patients, especially when they are at risk. Awareness regarding such rare acute presentations of an uncommon vasculitis can avoid diagnostic dilemmas.
Keywords: Acute nasal obstruction, eosinophilic granulomatosis with polyangiitis, vasculitis
|How to cite this article:|
Abdulla MC. Eosinophilic Granulomatosis with Polyangiitis Presenting as Acute Nasal Obstruction. J Head Neck Physicians Surg 2020;8:146-9
|How to cite this URL:|
Abdulla MC. Eosinophilic Granulomatosis with Polyangiitis Presenting as Acute Nasal Obstruction. J Head Neck Physicians Surg [serial online] 2020 [cited 2021 Apr 21];8:146-9. Available from: https://www.jhnps.org/text.asp?2020/8/2/146/302702
| Introduction|| |
Eosinophilic granulomatosis with polyangiitis (EGPA) is an eosinophil-rich and necrotizing granulomatous vasculitis predominantly affecting small to medium vessels involving the respiratory tract. Referred to as Churg–Strauss syndrome for many years, it is now recognized as EGPA after the 2012-revised nomenclature. Upper airway involvements such as sinonasal and paranasal sinus abnormalities are seen during the initial phase of the disease with asthma and precede the onset of the systemic disease by several years.
| Case Report|| |
A 55-year-old man presented with high-grade fever, nasal obstruction, and erythematous skin rash for 1 week. He had no running nose or postnasal drip. His sleep was disturbed significantly due to the nasal obstruction. He had bronchial asthma for the past 8 years (on steroid inhalation), diabetes mellitus for 6 years (on glimepiride and metformin), and hypertension for 5 years (on amlodipine). He denied a history of weight loss, had no sick contacts, and had no history of addictions.
He had high-grade fever of 101°F, tachypnea, and normal blood pressure. He had multiple elevated erythematous lesions over the upper chest, both ears, and forearms. Respiratory system examination showed deviated nasal septum to the left, bilateral inferior turbinate hypertrophy, fine inspiratory crepitations over bilateral infrascapular areas, and ronchi over bilateral infra-axillary and infrascapular areas.
Hemoglobin was 11.7 g/dl, total leukocyte count: 15,550/ml (with 69% neutrophils and 14% eosinophils), platelet count: 2, 40,000/μL, erythrocyte sedimentation rate: 64 mm in 1 h, and C-reactive protein was high. Peripheral smear showed normocytic normochromic red blood cells with eosinophilia. Blood chemistries were normal except for high blood sugars and glycosylated hemoglobin. Chest X-ray showed that bilateral heterogeneous opacities. HIV, and hepatitis B and hepatitis C serologies were negative. Contrast-enhanced computed tomogram of the thorax showed multiple enhancing parenchymal- and pleural-based nodules bilaterally, mild bronchiectasis in the left lower lobe, and bilateral pulmonary infiltrates [[Figure 1] upper panel]. Contrast-enhanced computed tomogram of the paranasal sinuses showed inflammatory sinonasal mucosal disease [[Figure 1] lower panel]. Nasal endoscopy showed deviated nasal septum to left and hypertrophied right uncinate process, and it was not possible to pass the scope further. P antineutrophil cytoplasmic antibody (ANCA, immunofluorescence method) was positive. Antinuclear antibody was negative. Blood culture was sterile. Skin biopsy showed infiltration of eosinophils, neutrophils, and lymphocytes in the vasculature as well as interstitial spaces of the dermal layer with focal fibrinoid necrosis in the reticular dermis [Figure 2]a,[Figure 2]b,[Figure 2]c,[Figure 2]d. Biopsy of the polyp from the right uncinate process showed inflammatory changes [[Figure 3] upper panel]. Fungal stain was negative (Periodic acid–Schiff and Grocott’s methenamine silver) [[Figure 3] lower panel]. Aspergillus fumigatus‐specific IgE antibody level was normal (0.25 kUA/L). He was diagnosed to have EGPA based on the clinical, laboratory, and histopathological findings. He was treated with prednisolone 1 mg/kg for 2 weeks following which the dose was tapered over the next 3 months and stopped. He had immediate relief for his nasal obstruction following three days of steroids. Fever and skin rash subsided later.
|Figure 1: Contrast-enhanced computed tomogram of the thorax showing multiple enhancing parenchymal- and pleural-based nodules bilaterally, mild bronchiectasis in the left lower lobe, and bilateral pulmonary infiltrates (upper panel). Contrast-enhanced computed tomogram of the paranasal sinuses showing inflammatory sinonasal mucosal disease (lower panel)|
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|Figure 2: Skin biopsy showing infiltration of eosinophils, neutrophils, and lymphocytes in the vasculature and interstitial spaces of the dermal layer and focal fibrinoid necrosis in the reticular dermis (a-d)|
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|Figure 3: Biopsy of the polyp from the right uncinate process showing inflammatory changes (upper panel). Fungal stain negative with Periodic acid–Schiff and Grocott’s methenamine silver (lower panel)|
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| Discussion|| |
The clinical manifestations of EGPA have three stages (1) asthma and rhinitis, (2) tissue eosinophilia, and (3) extra-pulmonary eosinophilic disease with vasculitis. Asthma in EGPA usually precedes the onset of the vasculitis stage by several years and is usually corticosteroid dependent. The nasal manifestations of EGPA are usually seen during the early phase along with asthma long before the onset of vasculitic phase.
The nasal manifestations of EGPA are common and the prevalence is 70%–85% in various studies., Nasal polyposis and chronic rhinitis (allergic rhinitis and nonallergic rhinitis) are the common nasal manifestations.,, Skin lesions occur in 40%–70% of patients and are seen usually during the vasculitic phase accompanied by constitutional symptoms. Skin lesions can manifest as palpable purpura, nodules, urticaria, livedo racemosa, or vesicles with necrosis. Our patient had acute nasal obstruction along with skin rash manifesting together during the vasculitic phase. He did not have any nasal complaints in the previous years.
The most common radiological in EGPA is bilateral areas of ground-glass opacity or consolidation with a bilateral symmetric distribution and a peripheral predominance which is seen in up to 90% of patients. Other radiological findings are bronchial dilatation, bronchial wall thickening, and small peribronchial and centrilobular nodules.,
Invasive fungal infections of the paranasal sinuses may be considered as a possibility in patients with acute-onset nasal obstruction and fever having uncontrolled diabetes mellitus. Imaging findings in such patients may be nonspecific, as seen in our patient (inflammatory sinonasal mucosal disease). It is usually seen in patients with poorly controlled diabetes mellitus, receiving chemotherapy, and on chronic oral corticosteroids. Histopathological specimen in our patient was negative for fungus (Periodic acid–Schiff and Grocott’s methenamine silver stains). Thus we were able to exclude invasive fungal infections in our patient which can worsen with steroids.
Other differential diagnoses of EGPA include hypereosinophilic syndromes (HES) and other vasculitides. HES is characterized by marked and persistent eosinophilia (>1500 cells/μL), organ involvement, and the absence of “reactive” forms of eosinophilia (particularly parasitic and viral infections, medications, allergies, tumors, autoimmune or immunological diseases). Idiopathic HES can overlap with EGPA since cardiac, and lung manifestations may be similar in both the diseases, while patients with idiopathic HES rarely present either asthma or nasal polyps or vasculitic complications (e.g., purpura, glomerulonephritis, and neuropathy). In idiopathic HES tissue biopsies do not show vasculitis, and ANCA are typically negative. EGPA must be differentiated from other small-vessel vasculitis. Granulomatosis with polyangiitis (GPA) or Wegner’s granulamatosis is to be differentiated from EGPA, especially in cases with eosinophilia. The distinguishing characteristics include ANCA specificity (c-ANCA/proteinase-3 ANCA being more frequent in GPA) and the presence of cavitated pulmonary nodules, nasal crusts, and sinus bone erosions in GPA. Microscopic polyangiitis (MPA) is usually associated with p-ANCA/MPO-ANCA as in EGPA but rarely shows high eosinophilia and involvement of the upper airway. The renal complications of MPA are often more severe when compared to EGPA.
Patient’s prognosis mainly determines the choice of initial therapy. Although not internationally validated, the “Five Factors Score” is used as a prognostic index for EGPA (cardiomyopathy, gastrointestinal involvement, central nervous system involvement, proteinuria [>1 g/24 h], and serum creatinine [>150 mmol/L]). The treatment for EGPA, according to the EGPA Consensus Task Force, is based on the presence of poor-prognosis manifestations, for example, kidney, heart (the leading cause of EGPA-related deaths), central nervous system, and gastrointestinal involvement. Such patients require a remission-induction regimen with glucocorticoids (the cornerstone of EGPA therapy) and another immunosuppressant (e.g., oral cyclophosphamide or intravenous pulses). Life-threatening manifestations require initial methylprednisolone pulses. However, glucocorticoids alone can be used to manage patients without life-and/or organ-threatening disease manifestations.
| Conclusion|| |
We present a patient with acute nasal obstruction during the vasculitic phase of EGPA. Upper airway involvement is usually seen during the early phase along with asthma long before the onset of vasculitic phase. Invasive fungal infections of the paranasal sinuses which can have similar presentations also should be ruled out by histopathology in such patients, especially when they are at risk. Awareness regarding such rare acute presentations of an uncommon vasculitis can avoid diagnostic dilemmas.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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There are no conflicts of interest.
This material has never been published and is not currently under evaluation in any other peer reviewed publication.
The permission was taken from Institutional Ethics Committee prior to starting the project. All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
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[Figure 1], [Figure 2], [Figure 3]