Severe Pulmonary Hypertension as Initial Presentation of SLE: A Case Report and Literature Review

Kiani, Rabia; Siddiqui, Muhammad Danial; Tantoush, Hamza

doi:10.1155/2020/6014572

Cited by 6 publications

(12 citation statements)

References 17 publications

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“…In the present study, the prevalence of PAH in newly diagnosed drug-naive patients with SLE was 13.3%. PAH can be an initial presentation of SLE, including severe PAH ( 28 ). Moreover, in the present study sPAP could reach 156 mmHg.…”

Section: Discussionmentioning

confidence: 99%

Decreased low‑density lipoprotein and the presence of pulmonary arterial hypertension among newly diagnosed drug‑naïve patients with systemic lupus erythematosus: D‑dimer as a mediator

Huang

Zhang

et al. 2022

Exp Ther Med

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Pulmonary arterial hypertension (PAH) is commonly associated with systemic lupus erythematosus (SLE). The present study investigated the relationship between coagulation and changes in lipid parameters in newly-diagnosed patients with SLE in the presence of PAH and whether the coagulation parameters were mediators between lipids and PAH presence. A total of 301 subjects scheduled for new-onset drug-naïve SLE were consecutively enrolled. Baseline data for patients without PAH and with PAH were gathered and compared. Coagulation and lipid parameters were compared across patients without lipid regulating and anticoagulation medications. Multivariable logistic regression model was applied to examine potential predictors of PAH in SLE. The relationships between them were examined using Spearman's correlation analysis. The relationship between coagulation index and lipids with SLE-PAH was evaluated using mediation analysis. Female patients accounted for 88.0% of the 301 subjects, and the average age was 32 years (range, 25-45 years). A total of 40 patients (13.3%) had PAH, and the average pulmonary artery systolic pressure (sPAP) was 55.825±26.67 mmHg. Patients with PAH were older and had higher levels of fibrin/fibrinogen degradation products (FDP), D-dimer, C-reactive protein, lower levels of complement 3, complement 4 and 25-hydroxy vitamin D3 compared with the non-PAH group. Multivariable logistic regression analysis showed that age and D-dimer were independent predictor factors for PAH. Among patients without lipid regulating and anticoagulation medications, patients in the PAH group had higher levels of D-dimer and FDP, and lower low-density lipoprotein (LDL) levels compared with patients without PAH. There was also a positive relationship between sPAP and D-dimer and FDP, and a negative relationship between sPAP and total cholesterol and LDL. Mediation analysis indicated that 25.61% of the effect of low LDL on PAH presence in systemic lupus erythematosus was mediated by D-dimer. Overall, the effect of low LDL on SLE-PAH appeared to be mediated by D-dimer, which mediated 25.61% of this effect.

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Section: Discussionmentioning

confidence: 99%

Decreased low‑density lipoprotein and the presence of pulmonary arterial hypertension among newly diagnosed drug‑naïve patients with systemic lupus erythematosus: D‑dimer as a mediator

Huang

Zhang

et al. 2022

Exp Ther Med

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“…As the development of PAH in SLE carries a worse prognosis, prompt recognition and early treatment initiation are crucial [ 6 ]. TTE is recommended for the initial screening of patients with suspected PAH as well as for the evaluation of response to treatment [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

“…Rituximab, mycophenolate mofetil, and cyclosporine have been used effectively in refractory cases of SLE-a PAH [ 12 , 14 ]. Similarly, among the vasodilators, for functional class III-IV with rapid progression of the disease and other markers of poor prognosis, initial therapy should be instituted with parenteral prostanoid agents, and then additional vasodilators are added as tolerated along with immunosuppression [ 6 ].…”

Section: Discussionmentioning

confidence: 99%

“…As the symptoms of PAH in SLE can be mild and non-specific in the early stages, increasing awareness of this devastating complication is essential for early diagnosis [ 5 ]. The pathophysiology of PAH involves multiple mechanisms including vasoconstriction, vasculitis, in situ thrombosis, and endothelial dysfunction to interstitial lung disease leading to vasospasm [ 6 ]. Patients with unrecognized PAH or those who are not yet treated progress to right ventricular dilatation and failure, which can ultimately lead to death [ 6 ].…”

Section: Introductionmentioning

confidence: 99%

“…The pathophysiology of PAH involves multiple mechanisms including vasoconstriction, vasculitis, in situ thrombosis, and endothelial dysfunction to interstitial lung disease leading to vasospasm [ 6 ]. Patients with unrecognized PAH or those who are not yet treated progress to right ventricular dilatation and failure, which can ultimately lead to death [ 6 ]. Here, we describe an interesting case of PAH in a patient with no established diagnosis of SLE and how our unique approach with hybrid therapy can be particularly helpful in severe SLE-associated PAH (SLE-a PAH) before referral to a tertiary care center.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Severe Pulmonary Artery Hypertension in Otherwise Silent Lupus: A Unique Hybrid Treatment Approach Using Hydroxychloroquine and Sildenafil

Saleem¹,

Kola²,

Shah³

2022

Cureus

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Pulmonary hypertension (PH) is a rare manifestation of systemic lupus erythematosus (SLE). Even more rare is pulmonary artery hypertension (PAH) presenting as the initial manifestation of SLE and may be a cause of diagnostic delay. As symptoms of PAH are very mild in the early stages, prompt diagnosis is crucial to prevent the progression of the disease. Echocardiographic evaluation involving the measurement of different right-sided heart variables in addition to estimated pulmonary artery pressure helps in reducing the false-positive rates of detection of PAH. The role of immunosuppression in addition to PAH-specific vasodilator therapy is one of the key aspects of management to minimize flares and improve hemodynamics. Equally important is the choice of a regimen best suited to minimize complications. We present a case of PAH in newly diagnosed SLE and the diagnostic and treatment challenges as a safety net hospital.

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Agranulocytosis in systemic lupus erythematosus: A case report

Frikha

Salah

Mefteh

et al. 2022

Clinical Case Reports

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Agranulocytosis is a rare acute condition characterized by severe a < gft (neutropenia in which the neutrophils count is less than 100/mm 3 . It can be classified into two categories, inherited, and acquired. Acquired agranulocytosis is not commonly caused by auto‐immune diseases such as systemic lupus erythematosus (SLE). We report a case of a patient suffering from agranulocytosis related to SLE at disease onset, associated with other rare disease involvements.

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Severe Pulmonary Hypertension as Initial Presentation of SLE: A Case Report and Literature Review

Cited by 6 publications

References 17 publications

Decreased low‑density lipoprotein and the presence of pulmonary arterial hypertension among newly diagnosed drug‑naïve patients with systemic lupus erythematosus: D‑dimer as a mediator

Decreased low‑density lipoprotein and the presence of pulmonary arterial hypertension among newly diagnosed drug‑naïve patients with systemic lupus erythematosus: D‑dimer as a mediator

Severe Pulmonary Artery Hypertension in Otherwise Silent Lupus: A Unique Hybrid Treatment Approach Using Hydroxychloroquine and Sildenafil

Agranulocytosis in systemic lupus erythematosus: A case report

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