A Case of Organizing Pneumonia in a Crohn’s Disease Patient
Corresponding Author: Vishnu Gireesh, Department of Respiratory Medicine, Ruby Hall Clinic, Pune, Maharashtra, India, Phone: +91 8667852908, e-mail: firstname.lastname@example.org
Received on: 11 May 2023; Accepted on: 25 September 2023; Published on: 30 October 2023
Introduction: Extraintestinal manifestations, including pulmonary manifestations like organizing pneumonia (OP), may be the presenting complaints in inflammatory bowel disease (IBD). In this case study, we discuss a case of OP in Crohn’s disease to understand the pulmonary manifestation of IBD and its management.
Case description: A 33-year-old female came with complaints of cough with minimal expectoration, headache, vomiting for 4 days, and dyspnea on exertion for 1 day. The patient has been a known case of Crohn’s disease for the past 5 years. The patient had no history of smoking. High-resolution computed tomography (HRCT) thorax done on admission showed ground glass opacities and peribronchovascular interstitial thickening in the left lung, predominantly the left lower lobe. Transbronchial lung biopsy (TBLB) was done, and histopathology was suggestive of OP. The patient improved clinically and radiologically with steroids.
Conclusion: Pulmonary manifestations of IBD are reported in numerous case studies. A patient with Crohn’s disease presenting with respiratory symptoms should be carefully evaluated for infective etiology since patients are on immunosuppressants. Drug-induced lung disease is the most frequent pulmonary complication of IBD. OP is a rare manifestation of Crohn’s disease, which requires careful evaluation, including bronchoscopy and biopsy, for diagnosis and management.
How to cite this article: Gireesh V, Tirpude S, Pai N, et al. A Case of Organizing Pneumonia in a Crohn’s Disease Patient. Indian J Respir Care 2023;12(3):277–280.
Source of support: Nil
Conflict of interest: None
Patient consent statement: The author(s) have obtained written informed consent from the patient for publication of the case report details and related images.
Keywords: Case report, Crohn’s disease, Cryptogenic organizing pneumonia, Inflammatory bowel disease, Pulmonary manifestations
Extraintestinal manifestations are seen in 21–40% of patients with inflammatory bowel disease (IBD).1 Pulmonary manifestations are usually rare. Drug-induced pulmonary disease is the most common and should be ruled out in all cases. Sulfasalazine, mesalamine, methotrexate, and infliximab have been found to be involved in lung disease associated with IBD.2 Other major manifestations of pulmonary disease associated with IBD are anatomical abnormalities, including fistulae, granulomatous lung disease, autoimmune disease, pulmonary function abnormalities, pleuritis, airway disease including bronchiolitis, bronchiectasis and chronic bronchitis, and interstitial fibrosis.2 Pulmonary manifestations of IBD can be broadly classified into parenchymal diseases, airway diseases, and interstitial diseases.3 Cryptogenic organizing pneumonia (COP) is the most common noninfectious IBD-related parenchymal disease.4 It is important to properly diagnose these conditions as there is a chance for recurrence and progressive worsening of lung function, and correct treatment is warranted. In this case report, we discuss a patient with OP as the pulmonary manifestations of Crohn’s disease.
A 33-year-old female came to our tertiary care center with complaints of exertional dyspnea for 1 day and high-grade fever, cough with minimal expectoration, headache, vomiting, and generalized weakness for 4 days. She is a nonsmoker and has had a known case of Crohn’s disease for the last 5 years, diagnosed by biopsy. She had been undergoing treatment with azathioprine. She underwent a left hemicolectomy 2 years back in view of ileocecal obstruction with paralytic ileus. The patient had a history of abdominal tuberculosis 10 years back, for which antitubercular drugs were taken for 9 months.
The patient was admitted, and computed tomography (CT) of the abdomen was done, which showed D1–D3 junction adhesion suspicious of ileoduodenal fistula and diffuse wall thickening in the distal descending colon, sigmoid colon, and rectum. Gastroscopy was done, which showed antral gastritis, and D2 showed nodularity. Biopsy from D3 was suggestive of mild nonspecific duodenitis with lymphoid hyperplasia. A colonoscopy revealed a deep excavating ulcer in the left colon, and the biopsy specimen had features of acute colitis with ulceration consistent with Crohn’s disease.
Additionally, lung windows revealed ground glass opacities and peribronchovascular interstitial thickening in the left lung, predominantly in the left lower lobe (Fig. 1). Coronavirus disease 2019 (COVID-19) reverse transcription polymerase chain reaction (RT-PCR), sputum bacterial culture, and tuberculosis GeneXpert were negative. Empirical broad-spectrum antibiotics were started suspecting infective etiology. For increasing breathlessness and ground glass opacities, video bronchoscopy showed visualized segments as normal. Transbronchial lung biopsy (TBLB) under fluoroscopy guidance and bronchoalveolar lavage (BAL) was done. Histopathology report showed OP with focal granulomatous inflammation (Fig. 2). Bronchoalveolar lavage yielded no infection. BAL galactomannan (GM) received after 5 days was 3.52. The patient developed pneumothorax postbiopsy (Fig. 3), so an intercostal pigtail catheter was inserted. The diagnosis of left lung COP with iatrogenic pneumothorax was made.
Prednisolone at 0.6 mg/kg/day was initiated based on the biopsy report. Tab voriconazole 200 mg was also added. The patient improved clinically. Voriconazole continued for 6 weeks. Subsequent chest X-ray and high-resolution computed tomography (HRCT) at the end of 6 weeks showed clearing of the patches on the left lower zone.
Extraintestinal manifestations are more common in Crohn’s disease than Ulcerative colitis.5 It involves nearly any organ system, including the musculoskeletal, hepatopancreatobiliary, ocular, renal, dermatologic, and pulmonary systems.6 Primary manifestations of the disease are inflammatory lesions in the skin, eye, liver, and joints, with musculoskeletal pain and joint involvement being the most common manifestations.7 If the secondary manifestations are also included, nearly 100% of the IBD patients are affected with some lesions outside the intestinal tract.7 As discussed earlier, pulmonary manifestations of IBD are infrequent. Crohn’s disease is less frequently associated with lung involvement, and those reported cases include granulomatous inflammation of the larynx, trachea and bronchi, chronic bronchitis, bronchiectasis, OP, interstitial lung disease, pulmonary edema, alveolar consolidation and lung infiltration with peripheral eosinophilia.8 So, drug-induced lung injury and infection that may be viral, fungal, or bacterial were the first differentials we considered. Her COVID-19 RT-PCR was negative, and her CT abdomen picked up lung opacities, and that’s how lung windows were taken. The accuracy of serum GM in the diagnosis of invasive pulmonary aspergillosis is being subjected to lots of research. The sensitivity varies from 42 to 80% in different studies.9 False positive GM assays are found to be associated with the administration of β-lactam antibiotics.10 In this case, GM assay is positive, which may be suggestive of probable semiinvasive aspergillosis. The possibility of a false positive GM assay cannot be ruled out. BAL GM has more sensitivity and specificity than serum GM in diagnosing Invasive pulmonary aspergillosis and chronic pulmonary aspergillosis.11,12
Organizing pneumonia (OP) is characterized by the presence of intraalveolar plugs of granulation tissue with loose fibroblastic connective tissue (OP) with or without the involvement of bronchioles (bronchiolitis obliterans). Buds extend into the adjacent alveoli, giving a characteristic “butterfly” pattern.13 Similar findings can be seen in bronchial lesions that also extend from alveolar lesions. The most common cause is infection, and the other causes are drug reaction, collagen vascular diseases, airway obstruction, toxin exposure, aspiration, organ transplantation, radiation, and a reactive change around localized lesions, which include infarcts, granulomas, abscesses, and neoplasms.14 OP can be divided into COP (without identifiable cause), secondary, and focal OP. Overall incidence around the world is 1.96 new cases per 100,000 persons per year.14 In a chest X-ray, OP is frequently seen as multiple patchy alveolar opacities, which are bilateral with peripheral distribution. In CT scan, the opacities vary from ground glass opacities to consolidations. Corticosteroids are the treatment of choice.13 The dosage of corticosteroids is not standardized. Lazor et al. suggested initial therapy with 0.75 mg/kg/day prednisolone for 4 weeks, 0.5 mg/kg/day for next 4 weeks, tapered to 20 mg/day for next 4 weeks, followed by 10 mg/day for 6 weeks and 5 mg/day for last 6 weeks.15,16 Several other authors suggested 1–1.5 mg/kg/day of prednisolone as an initial dose.15,17,18 Secondary infection should be carefully looked for since patients will be on dual immunosuppression. Histopathological findings of our patient were similar to OP (Fig. 3). Tanida et al. published a similar case study of a male patient with Crohn’s disease and bilateral lung consolidation.19 The patient was treated initially with antibiotics; later, TBLB was done, and a diagnosis of OP was made. The patient improved dramatically, and complete radiological clearance was achieved in 3 weeks with steroid therapy.
However, Basseri et al. reported the incidence of COP as 2% in a study conducted in 400 patients with pulmonary manifestation of IBD, and patients can present acutely or subacutely with dyspnea, cough, fever, and pleuritic chest pain.20
Tuberculosis is another common incidence in our endemic countries in such cases. This patient had an episode of the same 10 years ago. She was on long-term azathioprine. Azathioprine-induced lung injury is a very rare entity, even though isolated case reports linked azathioprine with interstitial lung disease.21 Findings in our patient were inconsistent with azathioprine toxicity.
In this case, the patient presented with atypical symptoms. However, the history of Crohn’s disease prompted us to look for pulmonary involvement. Although rare, patients with ulcerative colitis and Crohn’s disease should be evaluated for lung manifestations. Bronchoscopy with washings and TBLB should be carried out whenever indicated. We also conducted a literature review on this matter.
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