Diffuse Panbronchiolitis Preceding Ulcerative Colitis: Discussion

13 Nov
2014

Diffuse Panbronchiolitis Preceding Ulcerative Colitis: DiscussionAlthough the spectrum of pulmonary abnormalities associated with inflammatory bowel disease is broad, associated lung disease is unusual. For example, in reviewing the extraintestinal manifestations of inflammatory bowel disease in 700 patients with either Crohn’s disease or ulcerative colitis, Greenstein and colleagues recognized no patients with bowel-associated lung disease. Rogers et al2 cited a similarly low prevalence (0.21 percent). The current report presents a patient with both panbronchiolitis and ulcerative colitis whose lung illness antedated symptoms of bowel disease, was steroid-responsive, and improved after proctocolectomy. We speculate that his panbronchiolitis represents another, but previously unreported, manifestation of ulcerative colitis-associated lung disease (Table 2).

As presented in Table 2, the described spectrum of ulcerative colitis-associated bowel disease includes airway-related diseases (eg, sclerosing tracheobronchitis, bronchiolitis, bronchitis, and bronchiectasis), pulmonary vascular disease (eg, pulmonary vasculitis, pulmonary embolus), parenchymal/interstitial disease (eg, apical fibrosis, pulmonary fibrosis), and pleural disease (eg, pleural eflusions). Most common among the 47 available patient reports are airway-associated diseases, which occurred in 42.5 percent of these patients. Bronchitis and bronchiectasis were most common (17.1 percent and 19.1 percent, respectively).
The current patients panbronchiolitis satisfies both the clinical and pathologic diagnostic criteria outlined in the series by Homma et al, but has not been recognized previously, to our knowledge, as a pulmonary manifestation of ulcerative colitis. read

Other unusual features of this patients lung illness are that (1) its onset preceded the symptoms of bowel disease, and (2) the pulmonary symptoms improved for several months after proctocolectomy, which was performed for intractable bowel symptoms. Of the 47 patients described with ulcerative colitis-associated lung disease, only six, or 12.8 percent (present case included), experienced lung disease before the colonic presentation of ulcerative colitis. Only nine of the 47 reported patients (40 percent) have undergone a colectomy, and in only eight was colectomy performed after the onset of the associated pulmonary disease. Among these eight patients, the pulmonary disease (five airways-associated diseases, two pulmonary vasculitis, one pleural disease) worsened or was unchanged after colectomy in five but improved after colectomy in three patients. In the case reported here, pulmonary improvement after colectomy permitted a four-month cessation of systemic corticosteroid therapy, which had previously been frustrated by the return of cough and copious phlegm production during attempted steroid tapering.
In proposing this new clinical association between panbronchiolitis and ulcerative colitis, we can only speculate that a common pathophysiologic mechanism links the pulmonary and bowel illnesses. However, this association seems tenable in that other recognized causes of bronchiolitis, such as chronic toxic gas inhalation and viral infection, were not evident in this patient. We describe a unique patient with diffuse panbronchiolitis presenting as an extraintestinal complication of ulcerative colitis. This disease preceded the onset of bowel symptoms, responded to steroid therapy, and showed marked resolution following a colectomy that was performed for intractable bowel symptoms.
Table 2—Types of Pulmonary Diseases Associated with Ulcerative Colitis

Type of Pulmonary Disease* ReferenceNo.Total No. of CasesNo. of Cases Per CategoryFrequency Distribution, % of all CasesFrequency Distribution, % of Major Categories
Airway-associated diseases2042.5
Bronchiectasis11,12,17,22919.1
Bronchitis8,12817.1
Sclerosingtracheobronchitis2112.1
Sclerosingbronchiolitis2112.1
Difluse panbronchiolitisPresent case12.1
Pulmonary vascular diseases510.7
Pulmonary vasculitis7,9,1948.6
Pulmonary embolus312.1
Parenchymal/interstitial disease612.8
Interstitial fibrosis5,14,18,2048.6
Apical pulmonary fibrosis612.1
Bullous lung disease1312.1
Pleural Diseases714.9
Pleural effusion1624.3
Pleural effusion associated with pericardial effusion8,10,15510.6
Nonspecific919.1
Dyspnea on exertion productive cough11612.7
Abnormal bronchial biopsy, otherwise unspecified1136.4
Total4747100100
top