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ARP Rheumatology 2024We aim to study the prevalence and epidemiology of pulmonary arterial hypertension in SS, and the impact of PAH on SSc hospitalizations in the United States population.
OBJECTIVES
We aim to study the prevalence and epidemiology of pulmonary arterial hypertension in SS, and the impact of PAH on SSc hospitalizations in the United States population.
METHODS
We utilized the National Inpatient Sample (NIS) from 2016-2019 to obtain adult hospitalizations with the primary/secondary diagnosis of SSc and coexistent PAH (SSc-PAH). Epidemiological variables, mortality rates, and secondary outcomes were studied including pulmonary embolism, atrial flutter, atrial and ventricular fibrillation, pneumonia, sepsis, cardiac arrest and cardiac & renal failure, and ventilator requirement. Healthcare burden was estimated from total hospital charges (THC) and length of stay (LOS). Statistical analysis was performed on STATA 16.1, using linear and logistic regression analyses.
RESULTS
Out of 126,685 adult systemic sclerosis hospitalizations, 16.89% had PAH (SSc-PAH). The SSc-PAH group had significantly more females (85.4 % vs. 83.8%) and higher mean age (64.85±13.29 vs. 62.56±14.51). More African Americans were in this group than in the control group (19.5% vs. 14.6, p-value<0.001) while Whites (61.3% vs. 65.6%, p<0.001) and Asians (18.0 % vs. 2.8%, p<0.001) were less common. Charlson comorbidity index was higher for the SSc-PAH population (3.42 vs. 2.94, p-value<0.001). SSc-PAH group had a higher adjusted odds ratio (aOR) for mortality (aOR: 1.39, p<0.001), increased LOS (6.64 vs. 6.0 days, p<0.001) increased THC ($83,813 vs. $71,016, p <0.001). For the SSc-PAH group, there were also significantly higher odds of cardiac failure (aOR 3.13), ventilator requirement (aOR 2.15), cardiac arrest (aOR 1.39), kidney failure (aOR 1.63), pulmonary embolism (aOR 1.84), atrial flutter (aOR 1.86) atrial fibrillation (aOR1.56) and pneumonia (aOR 1.22). No significant difference in ventricular fibrillation, sepsis, or respiratory failure was noted.
CONCLUSION
Pulmonary arterial hypertension in SSc is associated with worse outcomes in terms of mortality and morbidity, and higher healthcare burden compared to SSc without PAH. Also, PAH disproportionately affects White, African American & Asian populations. There remains a pressing need to continue efforts for early diagnosis and management of PAH in SSc patients.
Topics: Humans; Scleroderma, Systemic; Female; Male; Middle Aged; United States; Aged; Pulmonary Arterial Hypertension; Hospitalization; Prevalence; Adult; Length of Stay; Inpatients
PubMed: 38956992
DOI: No ID Found -
ARP Rheumatology 2024To develop evidence-based recommendations for the non-pharmacological and pharmacological management of Raynaud's phenomenon (RP) and digital ulcers (DUs) in patients...
OBJECTIVE
To develop evidence-based recommendations for the non-pharmacological and pharmacological management of Raynaud's phenomenon (RP) and digital ulcers (DUs) in patients with systemic sclerosis and other immune-mediated connective tissue diseases (CTDs).
METHODS
A task force comprising 21 rheumatologists, two surgeons (vascular and plastic), two nurses, and one patient representative was established. Following a systematic literature review performed to inform the recommendations, statements were formulated and discussed during two meetings (one online and one in-person). Levels of evidence, grades of recommendation (GoR), and level of agreement (LoA) were determined.
RESULTS
Five overarching principles and 13 recommendations were developed. GoR ranged from A to D. The mean ± standard difference (SD) LoA with the overarching principles and recommendations ranged from 7.8±2.1 to 9.8±0.4. Briefly, the management of RP and DUs in patients with CTDs should be coordinated by a multidisciplinary team and based on shared decisions with patients. Nifedipine should be used as first-line therapy for RP and/or DUs. Sildenafil, tadalafil, and/or iloprost IV are second-line options for severe and/or refractory patients with RP and/or DUs. Sildenafil, tadalafil and/or Iloprost IV, should be prescribed for healing and prevention (also including bosentan) of DUs. In patients with RP and/or DUs, non-pharmacological interventions might be considered as add-ons, but there is limited quality and quantity of scientific evidence supporting their use.
CONCLUSIONS
These recommendations will inform rheumatologists, specialist nurses, other healthcare professionals, and patients about a comprehensive and personalized management of RP and DUs. A research agenda was developed to address unmet needs, particularly for non-pharmacologic interventions.
Topics: Humans; Raynaud Disease; Scleroderma, Systemic; Skin Ulcer; Fingers; Connective Tissue Diseases; Portugal
PubMed: 38956991
DOI: No ID Found -
The Journal of Rheumatology Jul 2024Scleroderma renal crisis (SRC) is a rare, life-threatening complication of systemic sclerosis (SSc) and can sometimes be the first manifestation of the disease. A...
Scleroderma renal crisis (SRC) is a rare, life-threatening complication of systemic sclerosis (SSc) and can sometimes be the first manifestation of the disease. A 56-year-old female presented with acute encephalopathy requiring intubation and a systolic blood pressure of 230 mmHg; no information was available about her medical history.
PubMed: 38950955
DOI: 10.3899/jrheum.2024-0173 -
The Journal of Rheumatology Jul 2024Systemic sclerosis sine scleroderma (ssSSc), formally described in 1962, is a subset of SSc which, unlike limited (lcSSc) and diffuse cutaneous (dcSSc) forms, lacks skin...
OBJECTIVE
Systemic sclerosis sine scleroderma (ssSSc), formally described in 1962, is a subset of SSc which, unlike limited (lcSSc) and diffuse cutaneous (dcSSc) forms, lacks skin fibrosis. According to the 2013 ACR/EULAR criteria, SSc can be diagnosed in the absence of skin thickening, even if this is expected to develop later in disease course. Driven by a fatal case of ssSSc with cardiac involvement, we analysed published data on ssSSc prevalence and severity.
METHODS
A systematic literature review and qualitative synthesis of SSc cohorts with data on ssSSc was performed.
RESULTS
Thirty-five studies on a total of 25,455 SSc patients published between 1976 and 2023 were identified. The mean prevalence of ssSSc, albeit using different definitions, was almost 10% (range 0-23%), with the largest study reporting a cross-sectional prevalence of 13%. In 5 studies with a follow-up period of up to 9 years, reclassification of ssSSc into lcSSc or dcSSc ranged from 0% to 28%. Interstitial lung disease, pulmonary arterial hypertension, scleroderma renal crisis, and cardiac diastolic dysfunction were present in 46% (range 9.3-59.1%), 15% (range 5.9-24.6%), 5% (range 1.6-24.6%), and 26.5% (range 1.8-40.7%) of ssSSc patients, respectively. Survival across studies was comparable to lcSSc and better than dcSSc.
CONCLUSION
Published data on ssSSc vary widely regarding prevalence, clinical expression and prognosis partly due to underdiagnosis and misclassification. Although classification criteria should not impact appropriate management of patients, updated ssSSc subclassification criteria, which will take into account time from disease onset, should be considered.
PubMed: 38950948
DOI: 10.3899/jrheum.2023-1113 -
Postepy Psychiatrii Neurologii Mar 2024Scleroderma is a multisystemic disorder characterised by inflammatory and vascular anomalies, and excess fibrosis. Progressive systemic sclerosis (PSS) mainly progresses...
PURPOSE
Scleroderma is a multisystemic disorder characterised by inflammatory and vascular anomalies, and excess fibrosis. Progressive systemic sclerosis (PSS) mainly progresses with skin, joint, lung, heart, and kidney involvement. Involvement of cerebral vessels is rare in both localised scleroderma and PSS. Transient ischemic attack and stroke are rare complications of scleroderma.
CASE DESCRIPTION
We present a 60-year-old stroke patient with localised scleroderma presenting with impaired speech, forgetting words, and occasional temporary memory loss.
COMMENT
In the case we present, no pathology was found in the clinical and laboratory tests performed in terms of ischemic risk factors. Skin findings included contracture, skin biopsy results, and antibody positivity related to scleroderma. Given the current pathogenesis of scleroderma, the patient was suspected of having a stroke.
PubMed: 38948684
DOI: 10.5114/ppn.2023.134450 -
The Lancet. Rheumatology Jun 2024
PubMed: 38944052
DOI: 10.1016/S2665-9913(24)00166-8 -
Expert Review of Respiratory Medicine Jun 2024Clinical guidance on the identification and management of connective tissue disease-associated interstitial lung disease (CTD-ILD) is needed for optimal clinical...
Identification and management of interstitial lung disease associated with systemic sclerosis (SSc-ILD), rheumatoid arthritis (RA-ILD), and polymyositis/dermatomyositis (PM/DM-ILD): development of expert consensus-based clinical algorithms.
BACKGROUND
Clinical guidance on the identification and management of connective tissue disease-associated interstitial lung disease (CTD-ILD) is needed for optimal clinical practice. We aimed to develop clinical algorithms for identifying and managing three common CTD-ILDs: those associated with systemic sclerosis (SSc-ILD), rheumatoid arthritis (RA-ILD), and polymyositis/dermatomyositis (PM/DM-ILD).
RESEARCH DESIGN AND METHODS
Meetings were held October - November 2023 to create consensus-based algorithms for identifying and managing SSc-ILD, RA-ILD, and PM/DM-ILD in clinical practice, based on expert consensus statements for identification and management of CTD-ILD previously derived from a Delphi process.
RESULTS
We developed clinical algorithms for SSc-ILD, RA-ILD, and PM/DM-ILD that highlight both commonalities and differences in the identification and management of these CTD-ILDs. Importantly, ILD should be suspected in patients with SSc, RA, or PM/DM who have respiratory symptoms. Chest high-resolution computed tomography has utility for screening, diagnosis and assessment of severity. Furthermore, regular follow-up and multidisciplinary management are important. Disease-specific considerations include unique risk factors such as anti-topoisomerase I antibodies in SSc-ILD, high-titer cyclic citrullinated peptide antibodies in RA, anti-aminoacyl tRNA synthetase antibodies in PM/DM, and anti-melanoma differentiation-associated gene 5 antibody in DM.
CONCLUSIONS
These algorithms may help physicians to identify and manage patients with SSc-ILD, RA-ILD, or PM/DM-ILD.
PubMed: 38943279
DOI: 10.1080/17476348.2024.2374910 -
Rheumatic Diseases Clinics of North... Aug 2024Connective tissue disease associated interstitial lung disease (CTD-ILD) is a heterogenous collection of conditions with a diverse spectrum of interstitial lung disease... (Review)
Review
Connective tissue disease associated interstitial lung disease (CTD-ILD) is a heterogenous collection of conditions with a diverse spectrum of interstitial lung disease (ILD) manifestations. Currently, clinical practice of lung-directed immunosuppression in CTD-ILD is supported by several randomized, placebo-controlled trials (RCTs) in patients with scleroderma and several observational, retrospective studies in other autoimmune conditions. However, given the harm of immunosuppression in idiopathic pulmonary fibrosis, there is an urgent need for RCTs of immunosuppression and antifibrotic agents in fibrotic CTD-ILD populations as well as the study of intervention in patients with subclinical CTD-ILD.
Topics: Humans; Lung Diseases, Interstitial; Connective Tissue Diseases; Immunosuppressive Agents; Scleroderma, Systemic
PubMed: 38942578
DOI: 10.1016/j.rdc.2024.03.001 -
Rheumatic Diseases Clinics of North... Aug 2024The majority of connective tissue diseases (CTDs) are multisystem disorders that are often heterogeneous in their presentation and do not have a single laboratory,... (Review)
Review
The majority of connective tissue diseases (CTDs) are multisystem disorders that are often heterogeneous in their presentation and do not have a single laboratory, histologic, or radiologic feature that is defined as the gold standard to support a specific diagnosis. Given this challenging situation, the diagnosis of CTD is a process that requires the synthesis of multidisciplinary data which may include patient clinical symptoms, serologic evaluation, laboratory testing, and imaging. Pulmonary manifestations of connective tissue disease include interstitial lung disease as well as multicompartmental manifestations. These CT imaging patterns and features of specific diseases will be discussed in this article.
Topics: Humans; Connective Tissue Diseases; Tomography, X-Ray Computed; Lung Diseases, Interstitial; Lung; Lung Diseases; Lupus Erythematosus, Systemic; Scleroderma, Systemic
PubMed: 38942577
DOI: 10.1016/j.rdc.2024.03.002 -
Rheumatic Diseases Clinics of North... Aug 2024Pulmonary hypertension (PH), a syndrome characterized by elevated pulmonary pressures, commonly complicates connective tissue disease (CTD) and is associated with... (Review)
Review
Pulmonary hypertension (PH), a syndrome characterized by elevated pulmonary pressures, commonly complicates connective tissue disease (CTD) and is associated with increased morbidity and mortality. The incidence of PH varies widely between CTDs; patients with systemic sclerosis are most likely to develop PH. Several different types of PH can present in CTD, including PH related to left heart disease and respiratory disease. Importantly, CTD patients are at risk for developing pulmonary arterial hypertension, a rare form of PH that is associated with high morbidity and mortality. Future therapies targeting pulmonary vascular remodeling may improve outcomes for patients with this devastating disease.
Topics: Humans; Connective Tissue Diseases; Hypertension, Pulmonary; Scleroderma, Systemic
PubMed: 38942575
DOI: 10.1016/j.rdc.2024.03.005