| Abstract|| |
Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis involving small and medium-sized blood vessels and granulomatous inflammation of upper and lower respiratory systems and/or renal system. In the limited form of GPA, there is no systemic involvement of disease with sparing of kidneys. The respiratory system is the commonly involved organ in limited GPA. Herein, we report the case of a 40-year-old male who was initially diagnosed as sarcoidosis clinically. Lung biopsy revealed necrotizing granulomatous angiitis. Diagnosis of GPA was made which was substantiated by antineutrophil cytoplasmic antibody (ANCA) positivity. This was a case of limited GPA with isolated lung involvement. The early diagnosis and initiation of treatment are critical for improved survival of patients with GPA. Tissue biopsy is necessary for the diagnosis of GPA.
Keywords: Atypical Wegener's granulomatosis, granulomatosis with polyangiitis, limited granulomatosis with polyangiitis, limited Wegener's granulomatosis
|How to cite this article:|
Begum S, Srinivasan S, Kathirvelu S, Vaithy A. Limited granulomatosis with polyangiitis presenting as an isolated lung lesion. Indian J Pathol Microbiol 2020;63:611-4
|How to cite this URL:|
Begum S, Srinivasan S, Kathirvelu S, Vaithy A. Limited granulomatosis with polyangiitis presenting as an isolated lung lesion. Indian J Pathol Microbiol [serial online] 2020 [cited 2020 Nov 25];63:611-4. Available from: https://www.ijpmonline.org/text.asp?2020/63/4/611/299327
| Introduction|| |
Granulomatosis with polyangiitis is a systemic necrotizing vasculitis involving small and medium-sized vessels and granulomatous inflammation of the upper and lower respiratory system and/or renal system. In the limited form of GPA, there is no systemic involvement of disease and the kidneys are usually spared. The respiratory system is the commonly involved organ in limited GPA. Formerly known as Wegener's granulomatosis, GPA is one of the antineutrophil cytoplasmic antibody-associated vasculitides. GPA is a potentially lethal disease with a low survival rate of only 20% if left untreated. Early detection of disease and initiation of immunosuppressive therapy improves the prognosis.
We report an atypical presentation of GPA in a middle-aged man who presented with hemoptysis and we further explore the differential diagnoses and criteria for early diagnosis of GPA. The case highlights the importance of having a suspicion for limited GPA in patients with atypical presentations, so that timely diagnosis and initiation of treatment will help in the survival of the patient.
| Case History|| |
A 40-year-old male presented to the pulmonary medicine out-patient department with a history of breathlessness on exertion for 1 year and multiple episodes of hemoptysis for 1 month, associated with cough with expectoration and chest pain. There was no history of fever, joint pain, or symptoms of the nasal and oral cavity. The patient is not a known case of pulmonary tuberculosis or bronchial asthma and he is a non-smoker.
On general physical examination, the patient's vitals were stable. There was a deviation of the nasal septum to the right side. Systemic examination did not reveal any abnormality and bilateral breath sounds were normal.
Routine blood investigations were within reference range except for a rise in ESR to 70 mm/ first hour. Sputum examination for acid-fast bacilli and sputum culture was negative. Renal function, urine examination, and liver function tests were normal. Chest radiography revealed ground-glass opacities on bilateral lower lobes and thickening of the horizontal fissure of the right lung [Figure 1]. Computed tomography (CT) of the thorax showed mild diffuse nodular thickening along fissures and also along bronchovascular bundles in both lungs. Mild diffuse interlobular septal thickening and patchy ground-glass opacities of bilateral lung fields were also noted [Figure 2]. Bilateral hilar and mediastinal lymphadenopathy was seen.
|Figure 1: Chest roentgenogram showing ground-glass opacities on bilateral lower lobes of lungs with thickening of the horizontal fissure of the right lung|
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|Figure 2: Computed tomography of thorax showing diffuse nodular thickening along fissures and bronchovascular bundles in bilateral lungs. Patchy ground-glass opacities of bilateral lung fields also seen|
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With these clinical and radiological findings, sarcoidosis was considered as one of the differential diagnoses and bronchoscopy and guided aspiration of a subcarinal mediastinal lymph node were planned. Bronchoscopy did not reveal any significant findings and transbronchoscopic FNA (fine needle aspiration) of the lymph node showed moderately cellular smear composed of clusters and singles of benign bronchial epithelial cells with reactive changes. The background was hemorrhagic with mucin and few lymphocytes. Bronchoalveolar lavage was examined. Around 1 mL of clear fluid, which was aspirated, showed few squamous cells and RBCs in the background.
Video-assisted thoracoscopy (VATS)-guided lymph node biopsy was planned. VATS revealed a highly vascular pleural surface with areas of hemorrhage and dense adhesion to the mediastinal surface. Lymph node resection was deferred due to high vascularity. Pleural biopsy and wedge resection of the left lingular lobe was done and sent for histopathological examination.
Grossly, lung tissue measuring 2 × 1.5 cm was received. The cut section showed grey white soft areas with focal areas of hemorrhage. Histological sections showed diffuse alveolar hyperinflation and focal interstitial septal thickening and necrotizing granulomatous vasculitis of the medium-sized artery with granulomas composed of epithelioid cells, giant cells, and dense lymphocytic infiltration with focal areas of necrosis [Figure 3] and [Figure 4]. No granulomas were identified in the lung parenchyma. Multiple bits of pleura were also received, largest measuring 1.3 × 0.7 cm and smallest measuring 0.2 × 0.2 cm. Histological sections from pleura showed large areas of hemorrhage and necrotizing granulomas in the pleura composed of epithelioid cells, giant cells, and lymphocytes.
|Figure 3: Photomicrograph of necrotizing granulomas surrounding an arteriole (Hematoxylin and Eosin, 10×)|
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|Figure 4: Photomicrograph of granulomas within the wall of an arteriole (Hematoxylin and Eosin, 40×)|
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Because of necrotizing angiitis in the lung and pleural granulomas, the possible differential diagnoses considered were pulmonary tuberculosis, a variant of sarcoidosis with vasculitis, and GPA. Absence of caseating granulomas in the lung parenchyma and the presence of only vasculitis were not features of tuberculosis. Ziehl Neelsen stain for acid-fast bacilli was negative. The dense lymphocytic cuff around granulomas with necrosis and pleural involvement were features against sarcoidosis. Periodic Acid Schiff stain negativity ruled out fungal infections.
The presence of necrotizing granulomas around medium-sized vessels surrounded by lymphoplasmacytic infiltrates and the absence of granulomas in the lung parenchyma was more in favor of GPA. Serological workup for confirmation was advised and c–ANCA (by immunofluorescence antibody assay) turned out to be positive.
| Discussion|| |
GPA is a disease that produces inflammation of the medium and small arteries and venules. It presents with a classical triad involving the upper respiratory system, pulmonary, and renal involvement. The prevalence of GPA is about 3 in 100,000 with a peak incidence in the age group of 50 to 60 years. The disease shows a slight male preponderance (3:2).
GPA shows a broad clinical manifestation ranging from localized disease to severe fatal disease with multiorgan involvement., It is said that GPA commences as localized respiratory tract granulomatosis, which then becomes a generalized vasculitis affecting small and medium-sized blood vessels. The upper respiratory tract is involved in nearly all patients. Nearly 90% of patients have pulmonary involvement and 80% are said to have renal involvement.
Cases of limited GPA sparing the kidneys have been reported., Sixteen cases of GPA have been reported where the disease was limited to the pulmonary organ with the absence of lesions elsewhere, particularly the kidneys. One case of limited GPA without upper respiratory tract involvement and renal involvement has also been reported.
In the present case, the patient presented with cough and hemoptysis which are the usual symptoms of several lung pathologies such as pulmonary tuberculosis, sarcoidosis, fungal infections, and lung metastasis, all of which have to be ruled out before making a diagnosis of GPA.
The ACR/EULAR (American College of Rheumatology/European League Against Rheumatism) recently proposed 2017 provisional classification criteria for GPA. Criteria include nine items, out of which five are clinical variables and four are test variables. Different scores are assigned for each item as shown in [Table 1].
|Table 1: The ACR/EULAR* 2017 Provisional Classification Criteria for GPA|
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The score for the present case is 10 (c-ANCA -5, nodules on chest imaging – 2, granuloma on biopsy – 3) making a diagnosis of GPA. A limited GPA has a good prognosis.
GPA presenting as isolated lung lesion is rare. Tuberculosis should be considered first in countries like India. As the treatment for GPA and tuberculosis is varied it is crucial to make a diagnosis of GPA without any ambiguity, so that definitive treatment can be initiated at the earliest, thereby improving the survival of patients with GPA. Tissue biopsy forms an essential component for the definitive diagnosis of GPA.
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.
We thank Dr. Pajanivel, Department of Pulmonary Medicine and Dr. Ilamaran, Department of Cardiothoracic and Vascular Surgery for their support.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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Department of Pathology, Mahatma Gandhi Medical College and Research Institute, Sri Balaji Vidyapeeth University, Pillaiyarkuppam, Puducherry - 607 402
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3], [Figure 4]