Pulmonary Vasculitis and Ulcerative Colitis John A.H. Forrest, BSc, MB, ChB, MRCP, and David J.C. Shearman, PhD, FRCPE



extraintestinal manifestations

o f u l c e r a t i v e colitis i n c l u d e uveitis, a r t h r i t i s , s k i n l e s i o n s a n d liver disease. L u n g


s u c h as c h r o n i c f i b r o s i n g alveolitis (1) o r p u l m o n a r y v a s c u l i t i s (2), h a v e o n l y r a r e l y b e e n rep o r t e d in a s s o c i a t i o n w i t h u l c e r a t i v e colitis. T h i s a r t i c l e d e s c r i b e s a p a t i e n t w i t h clinical a n d p a t h o l o g i c e v i d e n c e o f m i l d u l c e r a t i v e colitis, w h o , in a 2 - y e a r p e r i o d , d e v e l o p e d t w o d i s c r e t e episodes of a lesion closely resembling that of p u l m o n a r y vasculitis. T h e l u n g l e s i o n s c o i n c i d e d w i t h t w o r e l a p s e s o f u l c e r a t i v e colitis a n d r e s p o n d e d to c o r t i c o s t e r o i d t h e r a p y .

C A S E REPORT A 37-year-old male with no significant past medical history was admitted to hospital in February 1971, with a 6week history of intermittent bloody diarrhea, tenesmus, malaise, anorexia, and a weight loss of 21 lb. At that time there were no symptoms referrable to the respiratory or other systems. On examination, he was febrile (temperature 37.4 ~ C), his chest was clear, and there were no abnormalities on abdominal examination. Investigations revealed the following: Hematocrit 36%, WBC 14,000 mm 3 with 73% neutrophils, ESR 120 mm in the first hour (Westergren), bilirubin 2.0 mg/100 ml, SGPT 136 units/ml with alkaline phosphatase 120 KA units/100 ml; the alkaline phosphatase was of liver origin. Total proteins 7.9 g/100 ml, albumin 3.1 g/100 ml, "y-globulin elevated at 2.4 rag/ 100 ml. Australia antigen negative. A chest x-ray was normal. Sigmoidoscopy to 12 cm revealed a granular proctitis, biopsy of which was compatible with ulcerative colitis. The provisional diagnosis was one of ulcerative colitis and chotangitis. Two days after admission to hospital the patient became dyspneic at rest and developed a cough productive of bloodstained sputum, which was sterile on repeated culture. His From the Gastrointestinal Service, University Department of Therapeutics, Royal Infirmary, Edinburgh. Address for reprint requests: Dr. D.J.C. Shearman, Gastrointestinal Service, University Department of Therapeutics, Royal Infirmary, Edinburgh, EH3 9YW, Scotland.


temperature rose to 39 ~ C. He was on no drugs at this time. Auscultation of the chest revealed fine respiratory crackling erepitations. There was no finger clubbing. A chest x-ray showed bilateral pulmonary shadowing (Figure 1), which failed to clear with intravenous Furosemide or with the antibiotics ampicillin, penicillin, cephaloridine, or cloxacillin. The patient became increasingly more breathless and was started on prednisolone (60 mg/day). Within 24 hours his fever abated, his dyspnea had disappeared, and his chest xray had improved considerably (Figure 2). His chest x-ray had returned to normal 7 days later (Figure 3). In an attempt to find a causative agent for his clinical condition, the following investigations were performed and all were negative: viral and other studies for influenza A and B, adenovirus, Psittacosis, respiratory syncytial virus, Mycoplasma pneumoniae, Q fever, Leptospirosis, Brucellosis, mumps, herpes simplex, measles, and varicella-zoster, specimens for tuberculosis, ANF, and LE cells. Repeated stool cultures for amebae were negative and the amebae precipitin test was also negative. Mitochondrial and smooth muscle antibodies and cyclomegalovirus titers were normal. Lung biopsy was not performed. At the onset of the chest signs, the IgG was 2750 rag/100 ml (normal 700-1675) and this reverted to normal when his lungs became clear. His liver function tests remained abnormal. Biliary radiology followed by laparotomy revealed no extrahepatic cause for this. Three liver biopsies performed over a 6month period showed the features of a pericholangitis (Figure 4). Prednisolone was gradually reduced and then withdrawn after 1 month and his chest signs did not recur. The patient's proctitis resolved with the prednisolone given for his chest condition. From May 1971 to July 1973 his liver function tests remained abnormal, with the SGPT ranging from 80 to 216 units/ml and the alkaline phosphatase from 50 to 260 KA units/100 ml. These indices did not improve with intermittent courses of antibiotics. Although his bowel was clinically quiescent and a barium enema examination was normal, biopsies taken at colonoscopy in January 1972 showed the features of ulcerative colitis (Figure 5). In July 1973 the patient was readmitted with a mild attack of diarrhea. A chest x-ray was normal and he was receiving no drug therapy at that time. A few days lster he became febrile, with an unproductive cough. His fever continued, he became dyspneic, and the chest x-ray again showed bilateral pulmonary shadowing. As previously, all

Digestive Diseases, Vol. 20, No. 5 (May 1975)

Fig 1. X-ray of the chest, demonstrating extensive bilateral shadowing.

Fig 2. Chest x-ray 24 hours after prednisolone was started, showing considerable clearing.

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Fig 3. Normal chest x-ray 8 days after prednisolone was started.

Fig 4. Photomicrograph of liver section demonstrating portal tract, with pericholangitis and direct intralobular bile duct involvement (H&E, x140). 484

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Fig 5. Photomicrograph of colon section demonstrating gross infiltration with inflammatory cells, crypt abscess and reduction in number of glands (H&E, •

investigations revealed no cause for his chest condition, which again resolved with prednisolone within 72 hours. At this time, sigmoidoscopyshowed a moderate proctitis. Prednisolone was continued for 4 weeks, and 6 weeks after it had been withdrawn his chest x-ray and lung volume studies were normal, although his single breath CO transfer factor was slightly reduced at 21 (range 23-24).


Several types of pulmonary lesion have been described in association with ulcerative colitis. Turner-Warwick (1) described a patient with coexisting chronic fibrosing alveolitis, while Isenberg et at (2) documented a patient with a pulmonary vasculitis, which resolved following colectomy. Davies and MacFarlane (3) reported a patient with ulcerative colitis who developed subacute fibrosing alveolitis (HammanRich syndrome) while receiving salazopyrine. In addition, apical pulmonary fibrosis has been described (4). In our patient the prompt response of his Digestive Diseases, Vol. 20, No. 5 (May 1975)

lung lesion to corticosteroids and the fact that the lesion recurred rule out the diagnosis of subacute fibrosing alveolitis. Although drugs, such as salazopyrine (5) and chemicals, are known to cause lung lesions similar to that observed in this patient, he had not been receiving drug therapy and there had been no exposure to chemicals prior to his two bouts of illness. There is no evidence that he had an extrinsic allergic alveolitis and, indeed, both episodes developed while he was hospitalized. Repeated tests for a collagen disorder were negative. Although cytomegalovirus infection may precipitate acute ulcerative colitis (6), this virus is more usually seen as a coincidental finding in those with that disease (7). Cytomegalovirus may also cause pulmonary shadowing. In this patient repeated cytomegalovirus titers were normal over a 2-year period, and rectal and liver biopsies showed none of the pathologic lesions characteristically produced by that virus. In addition, the pulmonary lesions secondary to 485


cytomegalovirus do not respond to corticosteroids. It is unlikely therefore that his condition was due to cytomegalovirus infection. Although the barium enema examination was normal, biopsies taken at colonoscopy showed evidence of ulcerative colitis. In this situation the two techniques tend to be complementary, the barium study demonstrating such features as abnormal haustrations and severe mucosal abnormalities, whereas colonoscopy elicits less severe mucosal changes (8). Although fibrosing alveolitis has been reported in association with active chronic hepatitis (1), this patient's liver pathology is one of a pericholangitis secondary to ulcerative colitis. Although a lung biopsy was not performed, the most likely explanation for the patient's lung lesion is that he suffered two episodes of pulmonary vasculitis which, on both occasions, coincided with a relapse of his ulcerative colitis. There should now be greater awareness that patients with ulcerative colitis may develop a number of different forms of pulmonary disease, either associated with their colitis per se or secondary to salazopyrine (9). SUMMARY

A patient with ulcerative colitis who developed two separate episodes of a lung lesion compatible with pulmonary vasculitis is described. These episodes responded only to corti-


costeroid therapy and on each occasion they were associated with a relapse in the patient's ulcerative colitis. REFERENCES

1. Turner-Warwick M: Fibrosing alveolitis and chronic liver disease. Q J Med 145:133-149, 1968 2. Isenberg JI, Goldstein H, Korn AR, Ozeran RS, Rosen V: Pulmonary vasculitis--an uncommon complication of ulcerative colitis. N Engl J Med 279:1376-1377, 1968 3. Davies D, MacFarlane A: Fibrosing alveolitis and treatment with sulphasalazine. Gut 15 : 185188, 1974 4. Meadway J: Ulcerative colitis, colitic spondylitis and associated apical pulmonary fibrosis. Proc Roy Soc Med67:324-325, 1974 5. Jones GR, Malone DNS: Sulphasalazine induced lung disease. Thorax 27:713-717, 1972 6. Tamura H: Acute ulcerative colitis associated with cytomegalic inclusion virus. Arch Pathol 96:164-167, 1973 7. Farmer GW, Vincent MM, Fucillo DA, HortaBarbosa L, Ritman S, Sever JL, Gitnick GL: Viral investigations in ulcerative colitis and regional enteritis. Gastroenterology 65:8-18, 1973 8. Warwick RRG, Sumerling MD, Gilmour HM, Shearman DJC: Colonoscopy and double contrast barium enema examination in chronic ulcerative colitis. Am J Roentgenol 117, 292-296, 1973 9. Sulphasalazine-induced lung disease. Editorial. Lancet 2:504-505, 1974

Digestive Diseases, Vol. 20, No. 5 (May 1975)

Pulmonary vasculitis and ulcerative colitis.

CASE REPORT Pulmonary Vasculitis and Ulcerative Colitis John A.H. Forrest, BSc, MB, ChB, MRCP, and David J.C. Shearman, PhD, FRCPE The common extr...
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