Pulmonary Hypertension in a Patient with Rheumatoid Arthritis

Fatal pulmonary hypertension and rheumatoid vasculitis

“…In this latter group, the disease is usually secondary to interstitial fibrosis or due to associated primary pulmonary hypertension. Very few cases of pulmonary hypertension have been documented as secondary to histologically proven pulmonary vasculitis [5][6][7], and the distinction between vasculitis and changes secondary to pulmonary hypertension of any cause can be problematic [8]. In the case reported here, rheumatoid arthritis was confirmed by a positive rheumatoid factor and synovial biopsy, although the rheumatoid disease was mild as judged by current articular involvement.…”

Lung Disease in Rheumatoid Arthritis

“…It is more commonly seen in other CTDs such as scleroderma and systemic lupus erythematosis. 58 The most common form of vascular involvement in RA is rheumatoid vasculitis, which is characterized pathologically by the presence of a destructive inflammatory infiltrate within small and medium-sized blood vessel walls. This condition carries significant morbidity and mortality, but primary involvement of the lung is rare.…”

Survival in rheumatoid arthritis‐associated pulmonary arterial hypertension compared with idiopathic pulmonary arterial hypertension

“…In comparison to other CTD, our understanding of rheumatoid arthritis‐associated pulmonary arterial hypertension (RA‐PAH) comes from only a few observational studies and case reports, summarized in the http://onlinelibrary.wiley.com/doi/10.1111/resp.12464/suppinfo. One observational study screened 146 rheumatoid arthritis patients with echocardiography, and reported that 21% had a pulmonary artery pressure of >30 mm Hg .…”

“…Additionally pulmonary hypertension may be the consequence of rheumatoid lung disease (interstitial lung disease, or vasculitis of the pulmonary tree). There have been six reports that have focused on the histological features in these patients and concluded that the pulmonary arterial hypertension was a consequence of rheumatoid vasculitis . In contrast, a case report suggests that the pulmonary hypertension was related to a pulmonary vasculopathy of intimal and medial thickening resulting in luminal narrowing but without plexogenic lesions .…”

“…It has been reported that RA patients with underlying lung pathology develop pulmonary hypertension earlier; however, their mean survival time is greater than patients with idiopathic pulmonary arterial hypertension (IPAH) . Many reports indicate that RA‐PAH is fatal, yet one cohort study reported a 1‐year survival of 96% . Given this survival rate, it has been speculated that RA‐PAH may be more like IPAH than SSc‐PAH or SLE‐PAH .…”