Case Report A Rare Case of Ulcerative Colitis with Diffuse Parenchymal Lung Disease, Spontaneous Pneumomediastinum and Subcutaneous Emphysema D.C. Gupta, R.K. Jenaw, S. Koolwal and N. Khippal Institute of Respiratory Diseases, S.M.S. Medical College, Jaipur (Rajasthan), India Abstract A 50-year-old male, a tobacco smoker, who was known to have ulcerative colitis presented with dry cough, chest pain, dysponea and frequent passage of blood and mucous mixed stools. Physical examination revealed clubbing, subcutaneous emphysema of upper chest and auscultatory findings of crunching sound over pre-cordial area and basal crepitations. Spirometry was suggestive of restrictive pattern. High resolution computed tomography (HRCT) of thorax revealed pneumomediastinum, subcutaneous emphysema, bilateral diffuse centrilobular nodules and ground-glass haziness with mosaic pattern along with posterior basal fibrotic changes. The present case documents the uncommon pulmonary involvement of spontaneous pneumomediastinum and subcutaneous emphysema diffuse parenchymal lung disease, in a patient with ulcerative colitis. [Indian J Chest Dis Allied Sci 2014;56:109-111] Key words: Ulcerative colitis, Diffuse parenchymal lung disease, Spontaneous pneumomediastinum, Subcutaneous emphysema. [Received: February 4, 2013; accepted: July 29, 2013] Correspondence and reprint requests: Dr D.C. Gupta, Bungalow No. 3, Institute of Respiratory Disease, Subhash Nagar, Jaipur-302 016 (Rajasthan), India; Phone: 91-941-3504535; E-mail: [email protected] Introduction Inflammatory bowel disease (IBD) is a chronic inflammatory disease that commonly involves the gastro- intestinal tract of unknown aetiology. Crohn’s disease and ulcerative colitis are the two main forms of IBD. In IBD, extra-intestinal manifestations are frequently known to occur. These include dermatological manifestations (erythema nodosum and pyoderma gangrenosum); ocular manifestations (uveitis and episcleritis); hepatobiliary manifestations (primary sclerosing cholangitis and autoimmune hepatitis); musculo-skeletal manifestations, peripheral arthritis and axial arthropathy. 1 In contrast, pulmonary involvement is rare. The patterns of involvement in IBD are 2,3 glottic/sub-glottic stenosis, chronic bronchitis, bronchiectasis, bronchiolitis obliterans, granulomatous interstitial lung disease, desquamative interstitial pneumonia, pulmonary infiltrates and eosinophilia, and sterile necrobiotic nodules, sarcoidosis, alpha-1-antitrypsin deficiency, pulmonary vasculitis, venous thromboembolism, pleural and pericardial manifestations. We report a case of ulcerative colitis with spontaneous pneumomediastinum, subcutaneous emphysema with diffuse parenchymal lung disease, that is rare. Case Report A 50-year-old male presented in July 2012 with breathlessness at rest, acute substernal chest pain, dry cough, and change in character of voice of two days duration. He also complained of loose stools for the preceding two years. The patient was a tobacco smoker, alcoholic till recently and a chef by profession. In October 2010 he consulted the department of gastroenterology with complaints of bleeding per rectum, diarrhoea, and abdominal distension. Laboratory testing revealed haemoglobin 12.3 g/dL, white cell count 6000/mm 3 , platelet count 2.8 lakhs/ mm 3 . Erythrocyte sedimentation rate (ESR) was 54 mm at the end of first hour; C-reactive protein (CRP) was 6.35 mg/dL. Sigmoidoscopy (Figure 1) revealed marked ulceration and loss of vascular pattern suggestive of grade IV ulcerative colitis. Histopathological examination of the biopsy specimen confirmed ulcerative colitis in active phase. He was treated with mesalamine, azathioprine, systemic corticosteroids with enemas (mesalamine enema). Figure 1. Sigmoidoscopy showing marked ulceration and loss of vascular pattern suggestive of grade IV ulcerative colitis.
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Case Report
A Rare Case of Ulcerative Colitis with Diffuse Parenchymal Lung Disease,Spontaneous Pneumomediastinum and Subcutaneous Emphysema
D.C. Gupta, R.K. Jenaw, S. Koolwal and N. Khippal
Institute of Respiratory Diseases, S.M.S. Medical College, Jaipur (Rajasthan), India
Abstract
A 50-year-old male, a tobacco smoker, who was known to have ulcerative colitis presented with dry cough, chest pain,dysponea and frequent passage of blood and mucous mixed stools. Physical examination revealed clubbing, subcutaneousemphysema of upper chest and auscultatory findings of crunching sound over pre-cordial area and basal crepitations.Spirometry was suggestive of restrictive pattern. High resolution computed tomography (HRCT) of thorax revealedpneumomediastinum, subcutaneous emphysema, bilateral diffuse centrilobular nodules and ground-glass haziness withmosaic pattern along with posterior basal fibrotic changes. The present case documents the uncommon pulmonaryinvolvement of spontaneous pneumomediastinum and subcutaneous emphysema diffuse parenchymal lung disease, in apatient with ulcerative colitis. [Indian J Chest Dis Allied Sci 2014;56:109-111]
Key words: Ulcerative colitis, Diffuse parenchymal lung disease, Spontaneous pneumomediastinum, Subcutaneous emphysema.
[Received: February 4, 2013; accepted: July 29, 2013]
Correspondence and reprint requests: Dr D.C. Gupta, Bungalow No. 3, Institute of Respiratory Disease, SubhashNagar, Jaipur-302 016 (Rajasthan), India; Phone: 91-941-3504535; E-mail: [email protected]
Introduction
Inflammatory bowel disease (IBD) is a chronicinflammatory disease that commonly involves the gastro-intestinal tract of unknown aetiology. Crohn’s diseaseand ulcerative colitis are the two main forms of IBD. InIBD, extra-intestinal manifestations are frequently knownto occur. These include dermatological manifestations(erythema nodosum and pyoderma gangrenosum); ocularmanifestations (uveitis and episcleritis); hepatobiliarymanifestations (primary sclerosing cholangitis andautoimmune hepatitis); musculo-skeletal manifestations,peripheral arthritis and axial arthropathy.1 In contrast,pulmonary involvement is rare. The patterns ofinvolvement in IBD are2,3 glottic/sub-glottic stenosis,chronic bronchitis, bronchiectasis, bronchiolitisobliterans, granulomatous interstitial lung disease,desquamative interstitial pneumonia, pulmonaryinfiltrates and eosinophilia, and sterile necrobioticnodules, sarcoidosis, alpha-1-antitrypsin deficiency,pulmonary vasculitis, venous thromboembolism, pleuraland pericardial manifestations.
We report a case of ulcerative colitis with spontaneouspneumomediastinum, subcutaneous emphysema withdiffuse parenchymal lung disease, that is rare.
Case Report
A 50-year-old male presented in July 2012 withbreathlessness at rest, acute substernal chest pain, drycough, and change in character of voice of two daysduration. He also complained of loose stools for thepreceding two years. The patient was a tobacco smoker,alcoholic till recently and a chef by profession. In
October 2010 he consulted the department ofgastroenterology with complaints of bleeding perrectum, diarrhoea, and abdominal distension.Laboratory testing revealed haemoglobin 12.3 g/dL,white cell count 6000/mm3, platelet count 2.8 lakhs/mm3. Erythrocyte sedimentation rate (ESR) was 54 mmat the end of first hour; C-reactive protein (CRP) was6.35 mg/dL. Sigmoidoscopy (Figure 1) revealed markedulceration and loss of vascular pattern suggestive ofgrade IV ulcerative colitis. Histopathologicalexamination of the biopsy specimen confirmedulcerative colitis in active phase. He was treated withmesalamine, azathioprine, systemic corticosteroidswith enemas (mesalamine enema).
Figure 1. Sigmoidoscopy showing marked ulcerationand loss of vascular pattern suggestive of grade IVulcerative colitis.
110 Ulcerative Colitis with Diffuse Parenchymal Lung Disease D.C. Gupta et al
Heart rate was 112 beats/min, blood pressure 114/76 mmHg, oxygen saturation 78% on room air by pulseoximetry. Physical examination revealed digitalclubbing, subcutaneous emphysema over at the anteriorchest wall extending to the supra-scapular areas andneck. Auscultation revealed a crunching sound overthe pre-cordium, synchronous with the heart beat andcrepitations over the posterior basal regions. Chestradiograph (postero-anterior view; Figure 2)demonstrated multiple black leucencies in soft tissue ofsupra-clavicular and cervical region, thin radiolucentstrip along the heart borders, bilateral haziness midand lower zones, and elevated right diaphragm.Arterial blood gas analysis revealed PH 7.35, partialpressure of oxygen in arterial blood (PaO2) 45 mm Hg,partial pressure of arterial carbon dioxide (PaCO2) 35mmHg and bicarbonate (HCO3
-) 22 mEq/L suggestive oftype I respiratory failure. Biochemical investigationsincluding liver function tests were normal.
resolution of the pneumomediastinum andsubcutaneous emphysema and bilateral mid zoneshadowing after three weeks treatment (Figure 4). Stoolfrequency decreased, serum immumoglobulin (Ig)-Elevels reduced to 450 IU/mL, ESR came down to 15mm at the end of the first hour, CRP levels droppeddown to 1.26 mg/dL. The patient was discharged onshort-acting beta-agonists (SABA), mesalamine,azathioprine with tapering dose of prednisolone.
Figure 2. Chest radiograph (postero-anterior view) atthe time of presentation demonstrate multiple blackleucencies in soft tissue of supraclavicular and cervicalregion, thin radiolucent correct strip along the heartborders, bilateral haziness mid and lower zones, andelevated right diaphragm.
Serum total immunoglobulin E (IgE) levels weregreater than 2000 IU/mL. Stool examination, skin pricktest for common allergens and autoantibody screendetected no abnormality. Ultrasonography of theabdomen revealed hepatomegaly. Spirometry wassuggestive of restrictive pattern. High resolutioncomputed tomography (HRCT) (Figure 3) of thoraxdemonstrated pneumomediastinum, subcutaneousemphysema, bilateral diffuse centrilobular nodules,ground-glass haziness with mosaic pattern along withposterior basal fibrotic changes.
The patient was managed with high flow oxygentherapy, nebulised bronchodilators, antibiotics, oralprednisolone (40 mg daily), azathioprine (50 mg daily)and mesalamine (800 mg thrice-daily). There wassymptomatic and radiological improvement with
Figure 3. HRCT thorax showing pneumomediastinum,subcutaneous emphysema, bilateral diffuse centrilobularnodules, ground-glass haziness with mosaic patternalong with posterior basal fibrotic changes.
DiscussionPulmonary involvement of inflammatory bowel diseaseis rare. Both the colonic and respiratory epithelia shareembryonic origin from the primitive foregut and bothtypes of epithelial cells include goblet cells and sub-mucosal glands. The lungs and the gastrointestinal
Figure 4. Chest radiograph (postero-anterior view)suggestive of radiological improvement with resolution ofthe pneumomediastinum and subcutaneous emphysemaand bilateral mid zone after three weeks of treatment.
2014;Vol.56 The Indian Journal of Chest Diseases & Allied Sciences 111
tract contain sub-mucosal lymphoid tissue that playscrucial role in host mucosal defense. Due to similarity inthe mucosal immune system, same pathogeneticchanges are evident in respiratory involvement in IBD.
A variety of immunologic changes have beendocumented in ulcerative colitis. Cytotoxic T-cellsaccumulate in the lamina propria of the diseasedcolonic segment. This change is accompanied by anincrease in the population of B cells and plasma cells,with increased production of IgG and IgE.
A small proportion of patients with ulcerative colitishave anti-smooth muscle and anti-cytoskeletalantibodies. Serum and mucosal auto-antibodies againstintestinal epithelial cells may be involved. The presenceof anti-neutrophil cytoplasmic antibodies (ANCA) andanti-Saccharomyces cerevisiae antibodies (ASCA) is awell-known feature of IBD.4 Subcutaneous emphysemaand pneumomediastinum occur frequently in criticallyill patients in association with blunt or penetratingtrauma, soft-tissue infections, or any condition thatcreates a gradient between intraalveolar and peri-vascular interstitial pressures. A continuum of fascialplanes connects cervical soft tissues with themediastinum and retroperitoneum, permitting aberrantair arising in any one of these areas to spreadelsewhere.5
Marten et al6 described a patient with ulcerativecolitis who developed rapidly progressive usualinterstitial pneumonia (UIP) which was non-responsive to steroid treatment.6 Bronchiolitis obliteransorganising pneumonia has also been described in apatient with ulcerative colitis.7
In another report8, occurrence of mediastinal andsubcutaneous emphysema following retroperitonealperforation in a patient with severe ulcerative colitis withouttoxic dilatation has been described. In another report,occurrence of pneumorrhachis, pneumomediastinum,pneumopericardium, pneumoretroperitoneum, andsubcutaneous emphysema has been described afterproctocolectomy for ulcerative colitis.9
Two cases of ulcerative colitis in which toxicmegacolon was complicated by the unusual occurrenceof air tracking retroperitoneally through the diaphragmand the mediastinum, without signs of freeintraperitoneal air, ultimately presenting assubcutaneous emphysema in the neck has also been
reported.10 In a young adolescent boy with ulcerativecolitis development of pneumomediastinum andsubcutaneous emphysema in the neck andsurrounding cervical soft tissues secondary to aprobable retroperitoneal perforation of the colon hasbeen described.11
Histopathological diagnosis could not be establishedin the present case as the patient did not consent for theprocedure. Thus, the present case documents the rareoccurrence of spontaneous development ofpneumomediastinum and subcutaneous emphysemawith diffuse parenchymal lung disease in a patient withulcerative colitis.
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3. Camus P, Colby TV. The lung in inflammatory boweldisease. Eur Respir J 2000;15:5-10.
4. Peeters M, Joossens S, Vermeire S, Vlietinck R, Bossuyt X,Rutgeerts P. Diagnostic value of anti-Saccharomycescerevisiae and antineutrophil cytoplasmic auto antibodies ininflammatory bowel disease. Am J Gastroenterol 2001;96:730-4.
5. Maunder RJ, Pierson DJ, Hudson LD. Subcutaneous andmediastinal emphysema. Arch Intern Med 1984;144:1447-53.
6. Marten K, Fend F, Hautmann H, Kremer M, Rummeny EJ,Engelke C. Fatal acute exacerbation of usual interstitialpneumonia in ulcerative colitis. Br J Radiol 2005;78:762-6.
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9. Holton LH, Migaly J, Rolandelli RH. Pneumorrhachis,subcutaneous emphysema, pneumomediastinum,pneumopericardium, and pneumoretroperitoneum afterproctocolectomy for ulcerative colitis: report of a case. DisColon Rectum 2002;45:567-70.
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11. Cooke AA, Deshpande AV, Shun A, O’Loughlin EV.Pneumomediastinum and subcutaneous emphysema in achild with ulcerative colitis. Pediatr Emerg Care 2010; 26:129-31.
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