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Year : 2014  |  Volume : 2  |  Issue : 2  |  Page : 75-77

Wegener's granulomatosis presenting as spontaneous pneumothorax in young adult

1 Department of Medicine, Jawahar Lal Nehru Medical College, DMIMSU, Sawangi, Wardha, Maharastra, India
2 Department of Ear, Nose and Throat, Jawahar Lal Nehru Medical College, DMIMSU, Sawangi, Wardha, Maharastra, India

Date of Web Publication23-Jun-2014

Correspondence Address:
Sunil Kumar
Department of Medicine, Jawahar Lal Nehru Medical College, DMIMS, Sawangi, Wardha 442 004, Maharastra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2320-8775.135117

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Pulmonary involvement in Wegener's granulomatosis (WG) usually starts with nonspecific symptoms such as cough, dyspnea, hemoptysis, and pleuritis. Spontaneous pneumothorax as initial presentation is extremely rare. Although its real incidence is unknown, according to different classic series, it ranges between 3 and 5% of the cases. In this case, a 28-year-old male presented with complaints of epistaxis and breathlessness, which was diagnosed as WG with pneumothorax on the basis of chest X-ray and computed tomography (CT) chest and pathological confirmation by high level of serum cytoplasmic antineutrophil cytoplasmic antibody (ANCA).

Keywords: Antineutrophil cytoplasmic antibody, spontaneous pneumothorax, wegener′s granulomatosis, young adult

How to cite this article:
Kumar S, Pawani N, Honmode A, Bawankule S, Diwan S K, Jain S. Wegener's granulomatosis presenting as spontaneous pneumothorax in young adult. J Assoc Chest Physicians 2014;2:75-7

How to cite this URL:
Kumar S, Pawani N, Honmode A, Bawankule S, Diwan S K, Jain S. Wegener's granulomatosis presenting as spontaneous pneumothorax in young adult. J Assoc Chest Physicians [serial online] 2014 [cited 2022 Dec 6];2:75-7. Available from: https://www.jacpjournal.org/text.asp?2014/2/2/75/135117

  Introduction Top

Wegener's granulomatosis (WG) was described in detail by Friedrich Wegener in 1936. WG a rare autoimmue disease with polyangiitis and is a distinct clinicopathologic entity characterized by granulomatous vasculitis of the upper and lower respiratory tracts together with kidney lesion. [1] It is typically associated with proteinase 3 antineutrophil cytoplasmic antibodies (PR3-ANCAs), which cause a cytoplasmic ANCA (C-ANCA) pattern of staining on immunofluorescence testing of serum. The presence of PR3-ANCA makes us suspect the diagnosis of WG rather than other vasculitic diseases. [2] Pulmonary affection in these cases is one of the main manifestations and usually starts with unspecific symptoms such as cough, dyspnea, hemoptysis, and pleuritis. At the beginning of the disease, the most common findings on a chest radiograph are nodules, infiltrations, cavities, and pleural effusion/thickening (10 to 20%). [3],[4] Spontaneous pneumothorax is extremely rare. This case report highlights the importance of radiological investigations and systematic clinical-radiological correlation, leading to diagnosis of an uncommon condition with common presentation in an unsuspecting young male patient.

  Case Report Top

A 28-year-old man from rural Maharashtra was admitted in medicine department with breathlessness, blood mixed nasal discharge, and cough with hemoptysis. He denied any history of pulmonary tuberculosis, bronchial asthma, hypertension, and diabetes. He was nonsmoker and nonalcoholic. On general physical examination, he was having malaise with fever of 39°C, tachycardia (120 beats/min), tachypnea (36 breaths/min), and absent breath sound on right side of the chest. On nasal examination, he had multiple ulcerative and granulomatous lesions on the septum [Figure 1]. In view of breathlessness he had undergone for chest X-ray, which revealed right side pneumothorax [Figure 2]. Other examination was within normal limit. His blood examination revealed hemoglobin 8.9 gm/dl, total leucocyte count 11,800/mm 3 with markedly elevated erythrocyte sedimentation rate (ESR) (114 mm in first hour). Liver and renal functions were normal and urinary sediment did not include red blood cells, casts, or proteinuria. His enzyme-linked immunosorbent assay (ELISA) for human immunodeficiency virus (HIV) was negative. His sputum for acid fast bacilli (AFB) was negative on two occasions. He was put on intercostal chest tube drainage and his breathlessness relieved. Repeat chest X-ray showed expanded lung with bilateral infiltration and subsequent computerized omography of chest revealed nodular pulmonary lesions as well as multiple cavities in both the lungs [Figure 3]. Ultrasound of the abdomen of the patient revealed normal renal size. Based on the complete radiological findings with relevant clinical and pathological data, a possibility of WG was suggested. This was supported by histopathological examination of nasal granulation tissue, which revealed granulomatous inflammation around perivascular area. His C-ANCA in serum was markedly raised and perinuclear ANCA (P-ANCA) was negative. Patient was put on oral prednisolone 40 mg/day and cyclophosphamide 80 mg/day. He showed marked improvement in clinical symptoms such as cough and breathlessness. Repeat ANCA after 2 months was not increased. He was doing well on monthly follow up.
Figure 1: Nasal granuloma on nasal septum

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Figure 2: Pneumothorax right side

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Figure 3: CT scan chest showing nodule and cavities. CT = computed tomography.

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  Discussion Top

WG is a rare autoimmune entity that may occur at any age with mean age of occurrence 40-55 years with equal predisposition to male and female. WG with involvement of the upper respiratory tract and the lungs with renal sparing is frequently seen in women, whereas the kidneys are involved in the common form frequently seen in men. [5] Most of the respiratory manifestations in WG are nonspecific symptoms, such as cough, dyspnea, hemoptysis, and thoracic pain, which are generally present in up to 80% of the patients. [3]

In our case rarity was young male with renal sparing. The four criteria of classification defined by the American College of Rheumatology (ACR) for WG are as follows: (a) Oral or nasal ulcers or purulent bloody flux, (b) an abnormal lung X-ray revealing nodules and cavities, (c) an abnormal urinary sediment, and d) granulomatous inflammation in the extravascular region at biopsy. The presence of two or more of these criteria has a sensitivity of 88% and a specificity of 99%. [6] Among them pulmonary nodules, either single or multiple, are one of the most common manifestations, with an incidence of up to 90% of the cases, generally below 10 in number. They are usually cavitated, with irregular borders in up to 50% of the cases with a size that ranges from a few millimetres up to 10 cm. [6] PR3-ANCA was positive in this patient, and we tentatively diagnosed as WG on the basis of ACR criteria. In this case, dyspnea was due to spontaneous pneumothorax as revealed by chest X-ray. The real incidence of spontaneous pneumothorax is unknown, but according to different classic series, it ranges between 3 and 5% of the cases but only few are reported. [7] We have not found any similar case reports of pneumothorax related to other vasculitides in young patients.

The explanation for spontaneous pneumothorax is still unclear. In most of the described cases it appears when a subpleural cavitary nodule breaks down, and it is followed by a bronchopleural fistula, although in some cases, no fistula was found. [8],[9] However, why does the nodule break? Whereas the probability of a nodule being cavitated depends on its size (above approximately 20 mm), the relation between size and the probability of a pneumothorax has not been defined yet. [9] There are several pathogenic mechanisms, which, by themselves or associated with others, would explain the rupture of the nodule and the appearance of the pneumothorax, hydropneumothorax, or pyopneumothorax. [10] The intrinsic activity of the disease would be another mechanism because the pneumothorax appears at the onset of the disease in most of the patients. This may be a consequence of the complications derived from treatment such as steroid therapy, immune suppressant therapy, and infections, in view of the time sequence between the beginning of the treatment and most of the pneumothorax cases. [10] Among these pathogenic mechanisms, we can find infection that would favor the creation of fistulas due to difficulties in tissue repair. However, there are low incidence of infections (1-2%) on WG lesions except patient on immunosuppressant or steroid therapy, which may act as a trigger for potential infections. [10] In conclusion, pneumothorax is a rare complication in WG cases, without a clear pathogenic mechanism involved.

Treatment of WG includes induction of remission and maintenance of remission. Induction of remission is approached as follows: (a) Cyclophosphamide with high-dose glucocorticoids, (b) rituximab with high-dose glucocorticoids, (c) methotrexate with high-dose glucocorticoids, in non-organ-threatening or non-life-threatening, and (d) plasma exchange may be considered in patients with rapidly progressive renal disease (serum creatinine level >5.65 mg/dl) in order to preserve renal function. Maintenance of remission is as follow: (a) Once induction of remission has occurred, treatment for maintenance of remission should be continued for at least 18 months, often longer, (b) azathioprine (2 mg/kg/day) is safer than, and as effective as, cyclophosphamide in maintaining remission, (c) methotrexate (20-25 mg weekly) has been used for the maintenance of remission if the serum creatinine level is less than 1.5 mg/dl, and (d) leflunomide (20-30 mg/day) is as effective as methotrexate, but it is associated with more adverse effects.

Poorer survival is associated with older age, target organ involvement, and target organ damage. Renal involvement has been consistently shown to confer a poorer prognosis. An absence of renal involvement is associated with a 100% 5-year survival rate compared with approximately 70% in individuals with renal disease. An increased risk of cardiovascular events is also noted. Overall, the 10-year survival rate ranges from 75-88%. [11] WG should be considered in cases presenting with nasal disease and associated chest signs and symptoms, and early diagnosis and prompt treatment should be performed to save from this life-threatening emergency.

  References Top

1.Carol A. Langford, anthony s fauci: The vasculitis syndrome, granulomatosis with polyangitis (wegner's). In: Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson JL, editor. Harrison's Principles of Internal Medicine, 18 th ed. New York: McGraw-Hill Inc; 2012. p. 2789.  Back to cited text no. 1
2.Seaton A. Pulmonary lymphocytic angitis and granulomatosis. In: Seaton A, editor. Crofton and Douglas's Respiratory Diseases. 5 th ed. London: Blackwell Science Inc; 2000. p. 1063.  Back to cited text no. 2
3.Delevaux I, Khellaf M, Andre M, Michel JL, Piette JC, Aumaitre O. Spontaneous pneumothorax in Wegener granulomatosis. Chest 2005;128:3074-5.  Back to cited text no. 3
4.Sezer I, Kocabas H, Melikoglu MA, Budak BS, Ozbudak IH, Butun B. Spontaneous pneumothorax in Wegener's granulomatosis: A case report. Mod Rheumatol 2008;18:76-80  Back to cited text no. 4
5.Verma SR. Case report. Wegener's Granulomatosis in a young adult patient. Webmed Central Radiology 2011;2:WMC002210.  Back to cited text no. 5
6.Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum 1990;33:1101-7.  Back to cited text no. 6
7.Belhassen-Garcia M, Velasco-Tirado V, Alvela-Suarez L, Carpio-Perez A, Lledias JP, Novoa N, et al. Spontaneous pneumothorax in Wegener's Granulomatosis: Case report and literature review. Semin Arthritis Rheum 2011;41:455-60.  Back to cited text no. 7
8.Wolffenbuttel BH, Weber RF, Kho GS. Pyopneumothorax: A rare complication of Wegener's granulomatosis. Eur J Respir Dis 1985;67:223-7.  Back to cited text no. 8
9.Epstein DM, Gefter WB, Miller WT, Gohel V, Bonavita JA. Spontaneous pneumothorax: An uncommon manifestation of Wegener granulomatosis. Radiology 1980;135:327-8.  Back to cited text no. 9
10.Storelli E, Casali C, Natali P, Rossi G, Morandi U. Unusual pathogenesis of spontaneous pneumothorax secondary to Wegener's granulomatosis. Ann Thorac Surg 2007;84:288-90.  Back to cited text no. 10
11.Phillip R, Luqmani R. Mortality in systemic vasculitis: A systematic review. Clin Exp Rheumatol 2008;26:S94-104.  Back to cited text no. 11


  [Figure 1], [Figure 2], [Figure 3]

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