Dominating Cause of Pulmonary Hypertension May Change Over Time—Diagnostic and Therapeutic Considerations in a Patient with Pulmonary Hypertension Due to Rheumatoid Arthritis with Lung Involvement

Szturmowicz, Monika; Franczuk, Monika; Jędrych, Małgorzata Ewa; Wyrostkiewicz, Dorota; Oniszh, Karina; Darocha, Szymon; Kasperowicz, Krzysztof; Kurzyna, Marcin

doi:10.3390/diagnostics11101931

Cited by 5 publications

(10 citation statements)

References 27 publications

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“…One of the main causes of PH is a disease of the connective tissue, and RA is the most common disease of the connective tissue. However, compared with connective tissue diseases such as scleroderma, mixed connective tissue disease, and dermatomyositis/polymyositis, the prevalence of PAH in RA patients is lower 7 . According to Fayed et al's analysis of the various forms of PAH associated with connective tissue diseases, patients with RA often develop postcapillary PAH 8 …”

Section: Discussionmentioning

confidence: 99%

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The importance of pulmonary pulse transit time in indicating right ventricular dysfunction and pulmonary arterial stiffness in rheumatoid arthritis

Ulutas,

Tasolar,

Bayramoglu

et al. 2023

J of Clinical Ultrasound

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SubjectRheumatoid arthritis patients are at risk of developing cardiovascular disease such as right heart failure and pulmonary hypertension (PH). Arterial stiffness can be used to assess pulmonary hemodynamics. Noninvasive approaches can also be used to assess pulmonary hemodynamics. Recently, there have been reports that pulmonary pulse transit time (PPTT) may also be a useful measure. This study aims to examine the effects of pulmonary hemodynamic alterations on PPTT in RA patients.MethodsForty RA patients and 40 healthy controls were included in the study. Sociodemographic characteristics, laboratory data, and echocardiographic examinations were performed in both groups. Conventional echocardiographic examination included left and right ventricular systolic and diastolic diameters, right ventricular myocardial performance index (RVMPI), right ventricular diastolic function, estimated pulmonary artery systolic pressure (sPAP), tricuspid annular plane systolic excursion (TAPSE), pulmonary artery stiffness (PAS), and PPTT. Right ventricular diastolic and systolic volumes, right ventricular ejection fraction (RVEF), and right ventricular fractional area change (RVFAC) were determined by four‐dimensional echocardiography (4DE).ResultsThere was no difference between the sPAP values of the patients. RVMPI and PAS were increased in RA patients compared with controls. The PPTT was shortened in RA patients and correlated with RVEF, RVFAC, RVMPI, TAPSE/sPAP, disease duration, and C‐reactive protein (CRP). In univariate linear regression analysis, PPTT (p < .001) was thought to be an independent predictor of PAS. RVFAC, disease duration, and PAS were also independent predictors of PPTT.ConclusionIn RA patients, PPTT may be the first evidence of early abnormalities in pulmonary vascular hemodynamics. PPTT and PAS are the values that may predict each other in RA patients. Due to its more practical application, PPTT can be used instead of PAS to assess pulmonary hemodynamics.

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Section: Discussionmentioning

confidence: 99%

“…Even a single example, RA‐associated PH, illustrates the complexity of disease development. At the onset of the disease, interstitial lung disease predominated PH, whereas PAH occurred later in the course of the disease 7 . Even a small increase in pulmonary artery pressure can lead to a marked increase in PAS 12 …”

Section: Discussionmentioning

confidence: 99%

The importance of pulmonary pulse transit time in indicating right ventricular dysfunction and pulmonary arterial stiffness in rheumatoid arthritis

Ulutas,

Tasolar,

Bayramoglu

et al. 2023

J of Clinical Ultrasound

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“…PAH is a life-threatening complication associated with CTD, particularly in systemic sclerosis and systemic lupus erythematosus. RA is the most common CTD, but the prevalence of PAH in patients with RA is lower than that in patients with CTDs such as scleroderma, mixed collagen tissue disease, and dermatomyositis/polymyositis[ 17 ]. Fayed et al [ 18 ] provided an overview of CTD-associated PAH types and reported that post-capillary PAH is most common in patients with RA, while lung disease-associated PAH is commonly seen in patients with IIM and sarcoidosis.…”

Section: Discussionmentioning

confidence: 99%

“…Fayed et al [ 18 ] provided an overview of CTD-associated PAH types and reported that post-capillary PAH is most common in patients with RA, while lung disease-associated PAH is commonly seen in patients with IIM and sarcoidosis. The case of RA-associated PAH presented by Szturmowicz et al [ 17 ] illustrates the complexity of PAH in a single individual, with ILD-PAH dominating the early stage and CTD-PAH developing later.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary hypertension secondary to seronegative rheumatoid arthritis overlapping antisynthetase syndrome: A case report

Huang¹,

Lu²,

Tian³

et al. 2022

WJCC

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BACKGROUND Polyarthritis is the most frequent clinical manifestation in antisynthetase syndrome (ASS) forms of idiopathic inflammatory myositis and may be misdiagnosed as rheumatoid arthritis (RA), particularly in patients with seronegative RA (SNRA). It is unclear whether there is an overlap between ASS and RA, or if ASS sometimes mimics RA. Pulmonary hypertension (PAH) is common in connective tissue diseases (CTDs). However, published reports on CTD-PAH do not include overlapping CTDs, and its incidence and impact on patient prognosis are unclear. CASE SUMMARY We report the case of a 63-year-old woman who presented with a 3-mo history of symptom aggravation of recurrent symmetrical joint swelling and pain that had persisted for over 10 years. The patient was diagnosed with RA and interstitial lung disease. The patient repeatedly presented to the hospital’s respiratory and rheumatology departments with arthralgia, plus shortness of breath after activity. Relevant tests indicated that anti-CCP and RF remained negative, while anti-J0-1 and anti-Ro-52 were strongly positive. It was not until recently that we recognized that this could be an unusual case of SNRA with concurrent ASS. Joint pain was relieved after regular anti-rheumatic treatment. Chest computed tomography scans showed that pulmonary interstitial changes did not progress significantly over several years; however, they showed gradual widening of the pulmonary artery, and cardiac ultrasound indicated elevated pulmonary artery systolic pressure. The prescribed treatment of PAH was not effective in improving shortness of breath. CONCLUSION Overlap of RA and ASS may be missed. Further research is necessary to facilitate early diagnosis, effective evaluation, and prognosis.

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“…Also, the prothrombotic effect associated to chronic inflammation increases the risk of venous thromboembolism ( 62 ), and chronic thromboembolic pulmonary hypertension should be taken into account when approaching to the differential diagnosis of pulmonary hypertension in patients with RA and RA-ILD. Interestingly, the dominating cause of pulmonary hypertension may change over time, making the diagnostic and therapeutic process more challenging ( 63 ).…”

Section: Introductionmentioning

confidence: 99%

Unmet needs and perspectives in rheumatoid arthritis-associated interstitial lung disease: A critical review

et al. 2023

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Rheumatoid arthritis (RA) is a chronic systemic autoimmune disease characterized by synovitis as the most common clinical manifestation, and interstitial lung disease (RA-ILD) represents one of the most common and potentially severe extra-articular features. Our current understanding of the mechanisms and predictors of RA-ILD is limited despite the demonstration that an early identification of progressive fibrosing forms is crucial to provide timely treatment with antifibrotic therapies. While high resolution computed tomography is the gold standard technique for the diagnosis and follow-up of RA-ILD, it has been hypothesized that serum biomarkers (including novel and rare autoantibodies), new imaging techniques such as ultrasound of the lung, or the application of innovative radiologic algorithms may help towards predicting and detecting early forms of diseases. Further, while new treatments are becoming available for idiopathic and connective tissue disease-associated forms of lung fibrosis, the treatment of RA-ILD remains anecdotal and largely unexplored. We are convinced that a better understanding of the mechanisms connecting RA with ILD in a subgroup of patients as well as the creation of adequate diagnostic pathways will be mandatory steps for a more effective management of this clinically challenging entity.

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Dominating Cause of Pulmonary Hypertension May Change Over Time—Diagnostic and Therapeutic Considerations in a Patient with Pulmonary Hypertension Due to Rheumatoid Arthritis with Lung Involvement

Cited by 5 publications

References 27 publications

The importance of pulmonary pulse transit time in indicating right ventricular dysfunction and pulmonary arterial stiffness in rheumatoid arthritis

The importance of pulmonary pulse transit time in indicating right ventricular dysfunction and pulmonary arterial stiffness in rheumatoid arthritis

Pulmonary hypertension secondary to seronegative rheumatoid arthritis overlapping antisynthetase syndrome: A case report

Unmet needs and perspectives in rheumatoid arthritis-associated interstitial lung disease: A critical review

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