INTRODUCTION:Pulmonary hypertension (PH) and Wegener’s Granulomatosis (WG) are both uncommon diseases and have not been reported together. We report a case in which WG is suspected as the cause of PH.
CASE PRESENTATION:A forty-two year old woman with a known diagnosis of WG presented with complaints of worsening dyspnea on exertion, fatigue and generalized malaise. She was on cyclophosphamide and prednisone and had been transitioned to azathioprine. She had new room air hypoxia, peripheral edema and bibasilar rales. An echocardiogram demostrated severe pulmonary hypertension. The left ventricle was normal and there was no mitral or aortic valve disease. Right heart catheterization demonstrated mean pulmonary artery pressure of 66 mm Hg, pulmonary capillary wedge pressure of 18 mm Hg, a cardiac index of 1.3 liters/minute/ meter2 and pulmonary vascular resistance of 1264 dyne·s-1·cm-5. Left heart catheterization was negative for coronary artery disease and demonstrated normal left ventricular end diastolic pressures. A vasodilator challenge was not performed. She was only able to complete 250 meters during a six minute walk, desaturating to 83% on room air. She denied anorexigen use, history of pulmonary embolism (PE) or deep venous thrombosis (DVT). She also denied a family history of PH.An arterial blood gas showed a room air partial pressure of oxygen of 53 mm Hg and carbon dioxide of 30 mm Hg. V/Q scan was low probability for PE. A computed tomographic scan of chest showed scattered areas of ground glass opacities and enlarged pulmonary artery but no PE. Lower extremity duplexes were negative for DVT. A polysomnogram was negative. Rheumatological screen and human immunodeficiency viral screen were negative. Pulmonary function testing revealed a total lung capacity of 60% of predicted and a diffusion capacity for carbon monoxide of 38% of predicted. An open lung biopsy done previously had demonstrated changes consistent with WG.The patient was classified as World Health Organization Functional Status Class IV and started on oxygen, bosentan, sildenafil, warfarin, digoxin, and diuretics. Inhaled prostacyclin was added later. During a repeat six minute walk (6MW), there was an improvement to 350 meters. She required 3 liters of oxygen to maintain her resting oxygen saturation in the 90s. Her symptoms improved significantly and she was able resume activities of daily living.
DISCUSSIONS:PH and WG are both uncommon diseases. WG is a systemic vasculitis of the medium and small arteries, as well as the venules, arterioles, and occasionally large arteries. Vasculitis is not known to lead to PH. Other autoimmune diseases, such as systemic lupus erythematous and rheumatoid arthritis have been associated with a risk of development of PH. There is not a known association between WG and PH except for one report in the literature which involved granulomatous stenosis of the main pulmonary artery. (1) There was no evidence of pulmonary artery stenosis in our patient. We postulate that PH in our patient is secondary to her underlying vasculitis. Injury to the vascular endothelium activates endothelin expression leading to proliferative changes in the vascular wall and ultimate development of PH. This form of PH may be indistinguishable from an idiopathic form of PH.
CONCLUSION:We describe a patient with WG who had a clinical worsening and was discovered to have severe PH. We suspect that the development of PH in patients with vasculitis may be an underrecognized cause of clinical deteriorations. Physician awareness and appropriate treatment may contribute to improved quality of life in these patients.
DISCLOSURE:Ashish Mahindra, No Financial Disclosure Information; No Product/Research Disclosure Information