SAGES Magazine

THE SOUTH AFRICAN GASTROENTEROLOGY REVIEW 2022 | VOLUME 20 | ISSUE 1 | 32 CASE REPORT 28.7mmo/L and lactate 0.9mmol/L. A small right-sided pleural effusion was aspirated under ultrasound guidance for microbiological and cytochemical analysis as shown in table 2. Although the nucleated cell count was elevated with a mononuclear predominance, the chemical analysis was consistent with a transudative process. Table 2. Pleural fluid analysis results Pleural fluid parameter Value Reference range Protein (g/L) 14 < 30 = transudate; > 30 = exudate Pleural fluid-to-serum protein = 0.27 Albumin (g/L) 6 White cell count (x10⁹) 2.068 <0.002 Polymorphonuclear cells (%) 13 Mononuclear cells (%) 87 Adenosine deaminase (U/L) 4.6 > 30 suggestive of tuberculosis Lactate dehydrogenase (U/L) 141 Pleural fluid-to-serum LDH = 0.52 In addition, the pleural fluid adenosine deaminase level was not suggestive of tuberculosis; and PCR and mycobacterial culture on the fluid was negative for tuberculosis. Further invasive pulmonary evaluation such as broncho-alveolar lavage and lung biopsy was deemed to be high risk and unlikely to influence short term management. There was particular concern for the risk of an iatrogenic pneumothorax with invasive procedures. The patient was given a diagnosis of severe ulcerative colitis with accompanying pulmonary fibrosis as an extra-intestinal manifestation. According to imaging features, there was ‘extensive’ colonic involvement. No other extra-intestinal manifestations were evident at the time. Specific treatment was initiated with systemic corticosteroids, initially intravenous hydrocortisone followed by weight-based prednisone orally, with good symptom response. Oral and rectal (suppository) mesalamine was also started. Due to the severity of the IBD, the patient was a candidate for top-down medical treatment with early initiation of biologic therapy. However, because of limited resources in our hospital, biologic treatment is restricted and patients are usually treated in a step-up manner. Although it was planned to initiate azathioprine as maintenance treatment, this was deferred to the outpatient setting so as to ascertain optimal and uneventful response to corticosteroid induction therapy. The patient received a transfusion of 2 units of packed cells and nutritional supplements for protein-energy malnutrition. Bone prophylaxis in the form of vitamin D and calcium supplements was given. Although the patient was not screened for latent tuberculosis (by interferon gamma release assay) there was a perceived significant risk for tuberculosis reactivation with immunosuppression treatment; or for primary infection since tuberculosis is prevalent in our setting. Therefore, isoniazid prophylaxis was prescribed. Initially no specific or topical treatment was prescribed for the pulmonary ventilatory defects since the patient had minimal chest symptoms and there was need to manage the severe colitis first. The patient was eventually discharged home after 3 weeks in hospital and scheduled for follow up with IBD and respiratory clinics. Diarrhoea had resolved and she had started to gain weight. Discussion Clinically evident pulmonary EIMs are uncommon but are increasingly recognised. Interestingly subclinical abnormalities in pulmonary function testing (PFT) and chest imaging (with high resolution CT scan) have been reported in 25% to 50% of patients with IBD compared to controls; and pulmonary involvement correlates with active bowel disease. 2,3,4 Pulmonary manifestations are more common in ulcerative colitis (UC) compared to Crohn’s disease (CD). However, UC activity does not appear to correlate with values of pulmonary function testing; 3 and cases of post-colectomy pulmonary disease are well recognised. Our patient presented with a severe form of parenchymal lung disease (pulmonary fibrosis) and concomitant severe ulcerative colitis. Pulmonary extraintestinal manifestations can involve any area of the bronchopulmonary tree – airways (large and small), lung parenchyma, serosal surfaces and pulmonary vasculature; resulting in widely varied clinical presentations. However large airways inflammation (bronchiectasis > chronic bronchitis) is reported as the most prevalent manifestation and accounted for 39% of all pulmonary EIMs in one series. 5 Considered less common, small airways disease (commonly bronchiolitis) and parenchymal disease (such as pneumonitis) is increasingly recognised with high resolution chest imaging and other more sensitive diagnostic tests. Severe parenchymal disease, such as our patient had, is comparatively much less common. However, a population-based study in Korea has reported an increased incidence of idiopathic pulmonary fibrosis (IPF) in IBD patients compared to non-IBD controls. 6 The pathogenesis for pulmonary EIMs is not well understood. However as for other (non-pulmonary) EIMs, an interplay of multiple factors exists and includes shared genetic markers, environmental risk factors such as smoking, the role of gut microbiota and adaptive immune responses due to molecular mimicry. 7 Both the colonic and respiratory epithelia share an embryonic origin from the primitive foregut and the lungs and gastrointestinal tract contain submucosal lymphoid tissue and play crucial roles in host mucosal defence. Risk factors for lung parenchymal disease have not been well established but a slight female predominance may exist. In a study of pulmonary function abnormalities in patients with UC, abnormal PFT could not be predicted by current or past smoking status, family history of respiratory diseases or occupational history. 8 Many patients with pulmonary function or chest