Diagnosis: Crohn's disease of unspecified site with associated lung disease characterized by bronchitis (by bronchoscopy), lymphocytic bronchiolitis with focal purulent exudate, cellular interstitial pneumonia with focal bronchiolitis obliterans organizing pneumonia-like (cryptogenic organizing pneumonia-like) pattern, and necrobiotic nodules (sterile neutrophilic foci)


Postoperatively, a CT scan showed new opacities in the upper lungs, with centrilobular densities consistent with the patient's diagnosis of airway disease. Sulfasalazine was discontinued, and the dose of prednisone was increased. Cough, dyspnea, and fever disappeared, the chest radiographs cleared, and he resumed playing soccer. Spirometry two weeks following discharge showed mild to moderate restrictive lung disease without airflow limitation. Seven months later, PFTs showed normal lung volumes and diffusing capacity with minimal peripheral airway obstruction.
Pulmonary Disease Associated with Inflammatory Bowel Disease

Introduction: Inflammatory bowel disease (Crohn's disease and chronic ulcerative colitis) may be associated with extraintestinal manifestations including lung disease. The various manifestations, which are listed in the Table, may be coincident with, follow, or even precede the intestinal disease. Some of the extraintestinal manifestations have been attributed to therapy for the bowel disease [1].

Table: Extraintestinal manifestations of IBD [1] (multiple features in some patients)

Pulmonary Manifestations

Tracheobronchial stenosis: Symptoms include cough, hoarseness, stridor, and dyspnea. Endoscopy may show luminal narrowing up to 80%. Histology shows eroded epithelium and granulation tissue rich in plasma cells, lymphocytes, and PMNs. Treatment with corticosteroids (via aerosol or intravenously) ± laser therapy results in cure. Differential diagnosis includes: tuberculosis; sarcoidosis; amyloidosis; Wegener granulomatosis; lymphoma; cutaneous pemphigus; relapsing polychondritis; SLE; tracheobronchopathia osteochondroplastica; CMV, herpes, or aspergillus infection [1]; and crack cocaine smoking [2].

Bronchitis

Definition of terms

Chronic bronchitis is characterized by episodes of productive cough that are poorly responsive to antibiotics. Radiographs of CBS and bronchiectasis may have tubular opacities ± "tram lines" in lower lobes. PFTs demonstrate airflow insufficiency. Bronchoscopy shows redness and swelling of mucosa (trachea sometimes involved), and BAL fluid has increased numbers of PMNs. Cultures are usually negative. Histology shows a lymphocytic bronchitis, focal squamous metaplasia, and luminal PMNs. Inhaled steroids are beneficial in chronic bronchitis, but less so with CBS and bronchiectasis. Lavage with steroids has been advocated [1].

Bronchiolitis: Symptoms include productive cough and wheeze. Radiographs show diffuse small, irregular opacities. PFTs show obstruction and decreased FVC. Histology resembles diffuse panbronchiolitis or sclerosing, constrictive bronchiolitis with little inflammation. Prednisone is of little benefit [1].

COP-like pattern: Symptoms include acute to subacute onset of fever, dyspnea, dry cough, and pleurisy. Radiographs may have focal, unilateral, or diffuse opacities, often pleural-based with air bronchograms. PFTs show restriction. Histologically, granulation tissue in bronchioles and adjacent alveoli, and interstitial inflammation ± scattered eosinophils may be present. Oral or intravenous steroids, if necessary, reverse it [1].

Pulmonary infiltrates with eosinophilia: Wheeze, chest tightness, mild fever, night sweats, or malaise occur. BAL fluid may show eosinophilia. Radiographs may have bilateral, symmetric and/or peripheral opacities. Histology shows marked interstitial and some airspace infiltrates with eosinophils. The entity usually responds to oral steroid therapy [1].

Interstitial lung disease: Dyspnea, rales, clubbing, radiographic interstitial opacities, and focal honeycombing occur. PFTs show restriction. Histology shows a cellular interstitial pneumonia. One patient improved on oral steroids [1].

Necrobiotic nodules (= "sterile abscesses"): These have only been described previously in 2 patients with ulcerative colitis diagnosed 11 & 25 y earlier, respectively. Bowel disease was inactive. High fever and constitutional symptoms were present. There was no response to antibiotics. Radiographically, there were multiple nodules, some cavitated. In one case, p-ANCA (anti-lactoferrin IgG antibody) was present. Early lesions showed PMNs and fibrin with necrosis. Cavities were surrounded by granulation tissue. Giant cells and granulomas were absent. Steroids ± cyclophosphamide produced remission, without relapse, in both cases [1].

Other pulmonary conditions associated with Crohn's disease: Granulomatous involvement of larynx, trachea, bronchi, and alveolar parenchyma [1,3]; and subclinical lymphocytic alveolitis [4] have been described. Also, sarcoidosis has been described in association with both Crohn's disease and ulcerative colitis [1]. The role of the associated p-ANCA and immune complexes in the development of lung disease is unknown [5,6]. Pleuritis ± pericarditis, sometimes with effusion, is frequent [1].

Support for relation of lung disease to IBD: Evidence includes 1) coincidental exacerbations, 2) bronchiolitis immediately following colectomy, 3) inflammation in bronchi resembling that in ulcerative colitis, and 4) response to steroids [1].

Drug-induced disease with IBD: Drugs used to treat IBD, including sulfasalazine, 5-aminosalicylate, and methotrexate, can produce lung disease that resembles IBD-related disease [1].

Summary of Respiratory Manifestations of IBD (N = 89 patients) [1]

Airway disease

Parenchymal disease

Tracheobronchial stenosis 6.7%

COP-like pattern 12.4%

Chronic bronchitis 10.2%

Interstitial lung disease 18%

Chronic bronchitis + suppuration 7.9%

Pulmonary infiltrates & eosinophilia 3.4%

Bronchiectasis 22.5%

Necrobiotic nodules 5.6%

Chronic bronchiolitis 3.4%

Airway and parenchymal disease may occur separately or together.


Conclusions: Clinically-apparent pulmonary disease is rare with IBD. The pulmonologist may see the patient years after IBD is diagnosed, sometimes after bowel surgery, or when disease is inactive, but, nevertheless, should consider the possibility that a new-onset pulmonary disease is related to the IBD.

References

1. Camus P, Piard F, Ashcroft T, Gal A, Colby T. The lung in inflammatory bowel disease. Medicine 1993; 72:151-183.

2. Taylor R, Bernard G. Airway complications from free-basing cocaine. Chest 1989; 95:476-477.

3. Puntis J, Tarlow M, Raafat F, Booth I. Crohn's disease of the lung. Arch Dis Child 1990; 65:1270-1271.

4. Wallaert B, Colombel J, Tonnel A, Bonniere P, Cortot A, Paris J, Voisin C. Evidence of lymphocyte alveolitis in Crohn's disease. Chest 1985; 87:363-367.

5. Hardarson S, LaBrecque D, Mitros F, Neil G, Goeken J. Antineutrophil cytoplasmic antibody in inflammatory bowel and hepatobiliary diseases. High prevalence in ulcerative colitis, primary sclerosing cholangitis, and autoimmune hepatitis. Am J Clin Pathol 1993; 99:277-281.

6. Boberg K, Lundin K, Schrumpf E. Etiology and pathogenesis in primary sclerosing cholangitis. Scand J Gastroenterol 1994; 29 Suppl 204:47-58.

Case 10--Clinical summary

Comments: mw6825@itsa.ucsf.edu

Copyright 1997 by Martha L. Warnock

Last Revised 9/29/97

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