Distinctive Clinical, Radiographic, and Functional Characteristics of Patients With Sarcoidosis-Related Pulmonary Hypertension

Sulica, Roxana; Teirstein, Alvin S.; Kakarla, S; Nemani, Nimish; Behnegar, A.; Padilla, María L.

doi:10.1378/chest.128.3.1483

Cited by 174 publications

(195 citation statements)

References 23 publications

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“…In one cohort of 54 patients with sarcoidosis and elevated pulmonary pressures by www.intechopen.com echocardiography, 60% of the patients had radiographic stage IV disease, while 10% had radiologically normal lung parenchyma (stages 0 and I). (Sulica et al, 2005) When right heart catheterization (RHC) was used to measure mPAP, pulmonary hypertension was found in 31.8% of 22 patients without lung fibrosis. (Nunes et al, 2006) …”

Section: Epidemiologymentioning

confidence: 99%

“…(Handa et al, 2006;Nunes et al, 2006;Sulica et al, 2005) Similarly, appearance or worsening of dyspnea in a patient with stable or improved pulmonary function or despite optimization of immunosuppressive therapy may suggest SAPH. (Baughman et al, 2010) Other symptoms such as cough, chest pain, palpitations, and pre-syncope or syncope are suggestive, but not specific of SAPH.…”

Section: Clinical Presentationmentioning

confidence: 99%

“…SAPH should be considered in patients presenting with syncope, although this symptom could also represent sarcoid cardiac involvement. (Sulica et al, 2005) Specific signs of pulmonary hypertension, such as a loud pulmonary component of the second heart sound, tricuspid or pulmonary regurgitant murmurs, and right-sided gallop may be present in SAPH. Signs of right ventricular failure, like lower extremity edema, right ventricular heave, and elevated jugular venous pressure, are present in 21% of the patients with SAPH.…”

Section: Clinical Presentationmentioning

confidence: 99%

“…Signs of right ventricular failure, like lower extremity edema, right ventricular heave, and elevated jugular venous pressure, are present in 21% of the patients with SAPH. (Sulica et al, 2005) Hypoxemia and decreased functional status, measured by the 6-minute-walk distance, exercise desaturation and decreased distance-saturation product (DSP), tend to be more pronounced in patients with SAPH compared to sarcoidosis patients without PH. (Alhamad et al, 2010;Baughman et al, 2007;Bourbonnais & Samavati, 2008) …”

Section: Clinical Presentationmentioning

confidence: 99%

See 3 more Smart Citations

Sarcoidosis Associated Pulmonary Hypertension

Palmero¹,

Factor²,

Sulica³

2011

Pulmonary Hypertension - From Bench Research to Clinical Challenges

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Section: Epidemiologymentioning

confidence: 99%

Section: Clinical Presentationmentioning

confidence: 99%

Section: Clinical Presentationmentioning

confidence: 99%

Section: Clinical Presentationmentioning

confidence: 99%

See 2 more Smart Citations

Sarcoidosis Associated Pulmonary Hypertension

Palmero¹,

Factor²,

Sulica³

2011

Pulmonary Hypertension - From Bench Research to Clinical Challenges

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“…This higher frequency of SAPH is noted particularly in patients who are being evaluated for or awaiting lung transplantation, and increased mortality is noted in this population as well. [2][3][4][5] The optimal treatment approach to SAPH remains unclear.Conventional corticosteroid and immunomodulator therapies (such as methotrexate) utilized to treat sarcoidosis do not clearly improve SAPH. Furthermore, there is a relative paucity of data with regard to the utility of treating SAPH with pulmonary arterial hypertension (PAH)-specific therapies.…”

mentioning

confidence: 99%

Tadalafil Therapy for Sarcoidosis‐Associated Pulmonary Hypertension

et al. 2016

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Sarcoidosis-associated pulmonary hypertension (SAPH) is estimated to occur in at least 5% or more of sarcoidosis patients, and it contributes to significant morbidity and mortality. Optimal therapy for SAPH is not well established. We performed a 24-week open-label trial of tadalafil for SAPH at 2 academic medical centers. Subjects were required to have confirmed sarcoidosis plus a right heart catheterization within 12 months of enrollment showing a mean pulmonary artery pressure ≥ 25 mmHg, a pulmonary artery wedge pressure ≤ 15 mmHg, and a calculated pulmonary vascular resistance ≥ 3 Wood units. Subjects received 20 mg/day of tadalafil for the first 4 weeks and then 40 mg/day for the subsequent 20 weeks. Sixteen patients were screened, 12 of whom met criteria for enrollment. At 24 weeks, there was no overall improvement in 6-minute walk distance (6MWD). Five of the 12 subjects dropped out of the study early (2 for social reasons, 3 for medical reasons). There was no significant change in short form 36, St. George's respiratory questionnaire, or maximum Borg dyspnea scores over the 24 weeks. There were no significant adverse events or laboratory abnormalities clearly related to tadalafil in the cohort. The study did not meet the primary end point of change in 6MWD because of the small sample size. Tadalafil was generally safely administered in this cohort of SAPH patients. There was a relatively high dropout rate but no major adverse events and no clinical worsening. Larger studies are needed to explore this question further. (Trial registration: ClinicalTrials.gov identifier: NCT01324999) Keywords: sarcoidosis, pulmonary hypertension, tadalafil. Sarcoidosis is a multiorgan system disease of unknown etiology, characterized by noncaseating granulomatous tissue infiltration and inflammation. The most commonly affected organ system is the lung, most often in the form of alveolitis and/or parenchymal fibrosis and associated sequelae (such as bronchiectasis and cavity formation). Sarcoidosis-associated pulmonary hypertension (SAPH) is seen in at least 5% of patients with sarcoidosis 1 and more often in patients with advanced, end-stage disease. This higher frequency of SAPH is noted particularly in patients who are being evaluated for or awaiting lung transplantation, and increased mortality is noted in this population as well. [2][3][4][5] The optimal treatment approach to SAPH remains unclear.Conventional corticosteroid and immunomodulator therapies (such as methotrexate) utilized to treat sarcoidosis do not clearly improve SAPH. Furthermore, there is a relative paucity of data with regard to the utility of treating SAPH with pulmonary arterial hypertension (PAH)-specific therapies. We report the results of a prospective, openlabel trial of tadalafil therapy in a defined cohort of SAPH patients. The study was conducted at 2 academic medical centers in the United States. METHODSThis was an investigator-initiated study, approved by the institutional review boards of the University of Cincinnati and the Univ...

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Cystic bronchiectasis in sarcoidosis

Jadav,

Dadana,

Avula

2023

Clinical Case Reports

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Key Clinical MessagePulmonary sarcoidosis can manifest in different radiologic patterns. Typical manifestations in high‐resolution computed tomography are bilateral perihilar lymphadenopathy, micronodules, and fibrotic changes. Atypical manifestations are mass‐like or alveolar opacities, honeycomb‐like cysts, miliary opacities, tracheobronchial involvement, and pleural disease. Cystic bronchiectasis in pulmonary sarcoidosis is rare, with only a few reported cases in the literature. We present another case of cystic bronchiectasis with a honeycomb‐like pattern in pulmonary sarcoidosis and with cardiac involvement. This case was presented as an abstract poster at the American Thoracic Society conference in 2022.

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Distinctive Clinical, Radiographic, and Functional Characteristics of Patients With Sarcoidosis-Related Pulmonary Hypertension

Cited by 174 publications

References 23 publications

Sarcoidosis Associated Pulmonary Hypertension

Sarcoidosis Associated Pulmonary Hypertension

Tadalafil Therapy for Sarcoidosis‐Associated Pulmonary Hypertension

Cystic bronchiectasis in sarcoidosis

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