Dyspnea and Interstitial Lung Disease

Today's case was of a 46-year-old man presenting with a 4-6 week history of disabling progressive dyspnea, pleuritic chest discomfort, weight loss, night sweats, and dry cough.  He had a medical history of asthma which seemed to be well-controlled, and possible ulcerative colitis investigated, but without any follow up or established diagnosis.  His physical examination features dry “Velcro” sounding crackles consistent with interstitial lung disease.  His chest x-ray was abnormal which led to a CT scan demonstrating ground glass opacification.

There were multiple learning points today:

-Night sweats (in contrast to the type of sweating that many people experience) are characteristically drenching and require changing of clothes/sheets overnight.  It is helpful to phrase the question this way when taking a history to increase the specificity of the question.

-Drenching night sweats are often associated with tuberculosis but have a differential diagnosis that includes malignant processes (Hodgkin’s and Non-Hodgkin’s lymphomas can both produce this) and autoimmune/inflammatory causes.

-We talked about congestive heart failure as being a possible etiology for this man’s dyspnea.  In addition, if he had pulmonary venous thromboembolic disease for a long enough duration he’d be able to develop pulmonary hypertension which could lead to cor pulmonale and right-sided decompensated heart failure.  We also talked about pleuritis and pericarditis as manifestations of his presumed ulcerative colitis which could lead to pleural effusions and pericardial effusions +/- tamponade.

-We talked about pheochromocytoma, a rare tumour of the adrenal medulla which secretes catecholamines and can lead to hypertensive emergencies.  These tumors are classically associated with the “3 P’s” of Pounding (headache), Palpitations (from hypertension/tachycardia), and Pallor (from excessive vasoconstriction due to catecholamines).  They are also a secondary cause of hypertension and can be diagnosed by finding abnormal amounts metanephrines and catecholamine byproducts in the urine.  Like people intoxicated with cocaine, you should never give these people beta-blockers because it leads to an “unopposed alpha” phenomenon with a paradoxical increase in blood pressure as a result of the beta blockade.

-We discussed that this may be intrinsic lung disease related ulcerative colitis as well as treatment of inflammatory bowel disease.

-We discussed briefly what sounds like alphabet soup – Cryptogenic Organizing Pneumonia (COP) and Broncioloitis Obliterans with Organizing Pneumonia (BOOP).  These are similar clinical entities with the main difference being that there is an identifiable cause in BOOP (secondary organizing pneumonia).  They are both organizing pneumonias with a variety of possible causes.

I have included two reviews of the extra-intestinal manifestations of IBD, as well as one focusing on the pulmonary manifestations of IBD.  I have also included a review of cryptogenic and secondary organizing pneumonias.

Further Reading:

Geddes, D. M. (1991). BOOP and COP. Thorax, 46(8), 545-547.

Drakopanagiotakis, F., Paschalaki, M. K., Abu-Hijleh, M. M., Braman, S. S., & Polychronopoulos, V. (2011). Cryptogenic and Secondary Organizing Pneumonia. Chest, 139(4).

Greenstein, A. J., Janowitz, H. D., & Sachar, D. B. (1976). The extra-intestinal complications of Crohn's disease and ulcerative colitis: a study of 700 patients. Medicine, 55(5), 401-412.

Ardizzone, S., Puttini, P. S., Cassinotti, A., & Porro, G. B. (2008). Extraintestinal manifestations of inflammatory bowel disease. Digestive and Liver Disease, 40, S253-S259.

Storch, I., Sachar, D., & Katz, S. (2003). Pulmonary manifestations of inflammatory bowel disease. Inflammatory bowel diseases, 9(2), 104-115.