|Year : 2020 | Volume
| Issue : 1 | Page : 30-32
A rare case of granulomatosis with polyangiitis (Wegener’s) presenting with tracheo-bronchial involvement
Pragyan Rout1, Bijay Meher2, Garima Markam1, Kiran Sabat1
1 MBBS, Department of Pulmonary Medicine, S.C.B. Medical college and Hospital, Cuttack, Odisha, India
2 MBBS, MD, Department of Pulmonary Medicine, S.C.B. Medical college and Hospital, Cuttack, Odisha, India
|Date of Submission||24-Dec-2018|
|Date of Acceptance||01-Oct-2019|
|Date of Web Publication||11-Feb-2020|
Dr. Pragyan Rout
Room No-82, Senior Resident’s Hostel, S.C.B. Medical college, Cuttack, Odisha, 753007
Source of Support: None, Conflict of Interest: None
Granulomatosis with polyangiitis (Wegener’s) (GPA) is a form of systemic vasculitis that involves primarily the upper and lower airways and the kidneys. Although the pulmonary involvement of GPA is well described, the lower airway findings are rarely involved, and only various descriptions have been reported. The most frequent airway manifestations include subglottic stenosis and inflammation, and tracheal and bronchial stenoses. We, hereby, report a case of GPA presenting with fever, cough and haemoptysis with bronchoscopy showing markedly inflamed bronchial mucosa.
Keywords: Granulomatosis with polyangiitis, granulomatous vasculitis, haemoptysis
|How to cite this article:|
Rout P, Meher B, Markam G, Sabat K. A rare case of granulomatosis with polyangiitis (Wegener’s) presenting with tracheo-bronchial involvement. J Assoc Chest Physicians 2020;8:30-2
|How to cite this URL:|
Rout P, Meher B, Markam G, Sabat K. A rare case of granulomatosis with polyangiitis (Wegener’s) presenting with tracheo-bronchial involvement. J Assoc Chest Physicians [serial online] 2020 [cited 2020 May 27];8:30-2. Available from: http://www.jacpjournal.org/text.asp?2020/8/1/30/278120
| Introduction|| |
Granulomatosis with polyangiitis (Wegener’s) (GPA) is a small-vessel necrotizing granulomatous vasculitis, affecting mainly small arteries, arterioles, capillaries and venules of upper and lower airways and of kidneys. Ear, nose and throat (ENT), lung and kidney are the most frequently involved organs (90%, 90% and 80% patients, respectively). The manifestations of GPA in the respiratory tract include nasal stenosis, nasal cartilage necrosis, subglottic stenosis, tracheal and bronchial stenosis, granulomatous nodules and masses, alveolar infiltrates and cavities. Although the pulmonary involvement of GPA is well described, the lower airway findings are not frequently seen, and only various cases have been reported. Lower airway involvement in Wegener’s granulomatosis though rare can be seen in some patients.
| Case history|| |
A 48-year old female presented with complaints of fever, cough with expectoration, dyspnea, intermittent haemoptysis and epistaxis for six months. She was a known diabetic and non-smoker. There was no history of bronchial asthma or any other chronic diseases. On examination, her blood pressure was 110/80 mmHg, the pulse was 100 per minute, respiratory rate was 27 per minute and the oxygen saturation was 93% while she was breathing ambient air. There was presence of pallor with bilateral pitting pedal oedema. There were no skin eruptions, clubbing or saddle-nose deformity. On auscultation, there was bilateral vesicular breath sound with expiratory wheeze.
On investigation, complete hemogram showed normocytic normochromic anaemia (Hb-6.0 g/dl), elevated erythrocyte sedimentation rate (ESR) of 126 mm/1st Hour. C-reactive protein level was elevated (76.3 mg/L). Cytoplasmic Anti-Neutrophil Cytoplasmic Antibody (C-ANCA) was positive (20.36 U/ml). Serum Creatinine was elevated (2.5 mg/dl). Serum electrolytes and liver function tests were normal.
Chest X-ray showed right middle lobe consolidation ([Figure 1]). CECT chest showed minimally enhancing cavitating right lower lobe mass encasing right lower bronchus ([Figure 2]). Bronchoscopy was performed, which revealed diffuse erythema and oedema of the tracheobronchial mucosa with few nodules ([Figure 3]a). There was presence of a nodule over the vocal cord ([Figure 3]b). Broncho-alveolar Lavage (BAL) fluid and bronchial biopsy were taken from apical segment of right lower lobe. BAL showed no growth in culture media. Bronchial biopsy showed presence of inflammatory cells, multinucleated giant cells and granuloma along with necrotic foci. Catridge-Based Nucleic Acid Amplification Test (CBNAAT) of BAL fluid did not detect mycobacterium tuberculosis. Ultrasonography of abdomen showed features of chronic renal parenchymal disease. Renal biopsy could not be performed due to financial limitations.
|Figure 2 Right lower lobe cavitating mass encasing right lower lobe bronchus (arrow)|
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|Figure 3 (A) Vocal cord nodule (arrow) (B) Erythematous tracheo-bronchial mucosa (arrow)|
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The diagnosis of GPA was made on the basis of these histopathological findings and positive C-ANCA. She was treated with intravenous methylprednisolone pulse therapy (1 g/day) for three days followed by oral steroid. Injection Cyclophosphamide was given at a dose of 0.5kg/m2 every two weeks for three months. The patient is currently doing well and is on regular follow-up.
| Discussion|| |
GPA is characterized by necrotizing granulomatous inflammation of upper and lower respiratory tracts, glomerulonephritis and necrotizing vasculitis of the lungs and a variety of systemic organs and tissues. Lung parenchymal disease is the most frequent manifestation which produces multiple nodules and masses. Airway involvement in GPA is uncommon and does not show typical radiographic pattern. In large airways, these findings may consist of focal or elongated segments of bronchial stenosis and intraluminal soft tissue mass or bronchial thickening with or without lobar or segmental atelectasis. Bronchiectasis and peribronchial thickening in small airways were reported in approximately 40% cases of GPA.
Bronchoscopic examination is thought to be useful in detecting the abnormal bronchial findings of GPA compared with CT scan. It is reported that airway involvement is observed in 15–55% of patients with GPA. Tracheobronchial involvement with GPA has several manifestations, including tracheal and bronchial stenosis, mass lesions, tracheobronchial malacia and tracheoesophageal fistulae. In the acute lesions of GPA, the prominent neutrophil inflammation has the appearance of an abscess more than a granuloma. In the chronic phase, the pattern of injury shows an irregular central zone of necrosis containing varying numbers of degenerating neutrophils and necrotic debris surrounded by poorly defined granulomatous inflammation with palisades of elongated macrophages and scattered multinucleated giant cells. C-ANCA is found in 70–90% of patients with active GPA, and P-ANCA is observed in only 5–10% of patients with GPA. This case was unique, because there were features of both lower respiratory tract (trachea bronchial) involvement along with renal involvement, making it a severe variety of GPA.
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There are no conflicts of interest.
| References|| |
Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med 1997;337:1512-23.
Polychronopouls VS, Prakash UBS, Golbin JM et al.
Airway involvement in Wegener’s granulomatosis. Rheumatic Disease Clinics of North America 2007;33:755-75.
Lee KS, Kim TS, Fujimoto K et al.
Thoracic manifestation of Wegener’s granulomatosis: CT findings in 30 patients. European Radiology 2003;13:43-51.
Screaton NJ, Sivasothy P, Flower CDR, Lockwood CM. Tracheal involvement in Wegener’s granulomatosis: evaluation using spiral CT. Clinical Radiology 1998;53:809-15.
Mukhtyar C, Guillevin L, Cid MC et al.
EULAR recommendations for the management of primary small and medium vessel vasculitis. Annals of the Rheumatic Diseases 2009;68:310-17.
Schonermarck U, Lamprecht P, Csernok E, Gross WL. Prevalence and spectrum of rheumatic diseases associated with proteinase 3-antineutrophil cytoplasmic antibodies (ANCA) and myeloperoxidase-ANCA. Rheumatology 2001;40:178-84.
[Figure 1], [Figure 2], [Figure 3]