Pulmonary Hypertension in Polymyositis

doi:10.1378/chest.79.1.105

Chest

Volume 79, Issue 1, January 1981, Pages 105-107

https://doi.org/10.1378/chest.79.1.105 Get rights and content

Pulmonary hypertension is an uncommon but lethal complication of polymyositis. A 69-year-old woman is described who had a 20-month history of polymyositis and cardiac failure and in whom severe pulmonary hypertension was noted terminally when she was first seen at our clinic. She died within 24 hours of admission, and autopsy revealed severe plexogenic pulmonary vascular disease.

Section snippets

Case Report

A 69-year-old woman was hospitalized in an intensive care unit on Aug 2, 1977. Previously, she had been hospitalized at another institution in March 1975 for congestive heart failure. In January 1976, she experienced dysphagia with choking on meat and fluids and described nasal changes in her voice and regurgitation of fluids through her nose on swallowing. The patient had lost weight, eventually totaling 18.2 to 22.7 kg (40 to 50 lb), and she experienced weakness in the muscles of her

Discussion

Our patient was initially considered to have polymyositis with secondary congestive cardiomyopathy. Unexpectedly, however, severe elevations of pulmonary artery pressure were noted, along with an increase in wedge pressure, which, in retrospect, remained unexplained. Systemic blood pressure decreased despite the IV administration of dopamine, and the patient died within 24 hours of admission.

At post-mortem examination, striking plexogenic vascular changes were seen in the lungs, similar to

References (4)

NGG Hepper et al.
Three types of pulmonary involvement in polymyositis
Med Clin North Am
(1964)
RH Young et al.
Pulmonary vascular changes in scleroderma
Am J Med
(1978)

There are more references available in the full text version of this article.

Cited by (77)

Pulmonary Arterial Hypertension in Connective Tissue Diseases Beyond Systemic Sclerosis
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Citation Excerpt :
There have been rare reports of patients with inflammatory myopathies, such as DM and PM, with PAH. However, other causes of PH including Group 3 PH due to chronic lung disease, as well as the presence of overlap with other CTDs, could not be completely excluded.35–37 A study involving the French PH registry suggested that PAH may be associated with dermatomyosis, although this was based on 3 of a total of 5223 patients.38
Cardiac involvement in polymyositis
2012, Annales de Cardiologie et d'Angeiologie
L’atteinte cardiaque au cours de la polymyosite constitue une cause majeure de décès. Cependant, la localisation cardiaque est rarement symptomatique et ne représente généralement pas l’essentiel du tableau au moment du diagnostic. Nous présentons une observation particulière de polymyosite où les manifestations cardiaques sont sévères et polymorphes et prédominent les signes squelettiques.
Cardiovascular involvement in polymyositis constitutes a major cause of death. However, the cardiac location is rarely symptomatic and does not usually represent the principle clinical feature at the time of the initial presentation. We present here an unusual case of polymyositis with severe and polymorph cardiac disturbances that predominant the muscular signs.
Classification of Pulmonary Hypertension
2012, Heart Failure Clinics
Citation Excerpt :
Subsequent prospective studies using screening echocardiography followed by diagnostic RHC for confirmation of PAH have found that the prevalence of PAH in patients with SSc is between 5% and 12%.17,36–39 Systemic lupus erythematosus (SLE)40–42 and mixed CTD42 are associated with PAH less frequently than SSc; however, PAH also develops in patients with other types of CTD, including Sjögren syndrome,43 rheumatoid arthritis,44 and polymyositis.45 Chronic inflammation may play a role, and macrophages, T and B lymphocytes, and dendritic cells have been identified surrounding plexiform lesions.46
Diagnosis and classifi cation of pulmonary hypertension
2010, Presse Medicale
Updated Clinical Classification of Pulmonary Hypertension
2009, Journal of the American College of Cardiology
Citation Excerpt :
In systemic lupus erythematosis (25,26) and mixed connective tissue disease (27,28), the prevalence of PAH remains unknown but likely occurs less frequently than in systemic sclerosis. In the absence of fibrotic lung disease, PAH has been reported infrequently in other connective tissue diseases such as Sjögren syndrome (29), polymyositis (30), or rheumatoid arthritis (31). Pulmonary arterial hypertension is a rare but well-established complication of HIV infection (32,33).
The aim of a clinical classification of pulmonary hypertension (PH) is to group together different manifestations of disease sharing similarities in pathophysiologic mechanisms, clinical presentation, and therapeutic approaches. In 2003, during the 3rd World Symposium on Pulmonary Hypertension, the clinical classification of PH initially adopted in 1998 during the 2nd World Symposium was slightly modified. During the 4th World Symposium held in 2008, it was decided to maintain the general architecture and philosophy of the previous clinical classifications. The modifications adopted during this meeting principally concern Group 1, pulmonary arterial hypertension (PAH). This subgroup includes patients with PAH with a family history or patients with idiopathic PAH with germline mutations (e.g., bone morphogenetic protein receptor-2, activin receptor-like kinase type 1, and endoglin). In the new classification, schistosomiasis and chronic hemolytic anemia appear as separate entities in the subgroup of PAH associated with identified diseases. Finally, it was decided to place pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis in a separate group, distinct from but very close to Group 1 (now called Group 1′). Thus, Group 1 of PAH is now more homogeneous.
Severe Pulmonary Arterial Hypertension in a Patient with Polymyositis-Dermatomyositis
2020, Open Respiratory Archives

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Selected ReportsPulmonary Hypertension in Polymyositis

Section snippets

Case Report

Discussion

Med Clin North Am

Am J Med

Selected Reports
Pulmonary Hypertension in Polymyositis