Efficacy and safety of combination therapy with prednisolone and oral tacrolimus for progressive interstitial pneumonia with systemic sclerosis: A retrospective study

Konma, Junichi; Kotani, Takuya; Shoda, Takeshi; Suzuka, Takayasu; Fujiki, Youhei; Nagai, Koji; Hata, Kenichiro; Takeuchi, Tohru; Makino, Shigeki; Arawaka, Shigeki

doi:10.1080/14397595.2018.1441658

Cited by 12 publications

(12 citation statements)

References 38 publications

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“…Even in the future, to determine whether HRCT should be performed or not in an ILD patient would be based on elevated KL-6 level as a shred of clinical evidence. Meanwhile, a study also shows that the higher serum KL-6 thresholds could aid in identifying patients who are more likely to require intensification of treatment to prevent progression of ILD ( 64 ). In this case, our pooled result concluded the critical role of elevated KL-6 level reflecting poor prognosis of ILD.…”

Section: Discussionmentioning

confidence: 99%

KL-6 as an Immunological Biomarker Predicts the Severity, Progression, Acute Exacerbation, and Poor Outcomes of Interstitial Lung Disease: A Systematic Review and Meta-Analysis

et al. 2021

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ObjectInterstitial lung disease (ILD) is a specific form of chronic fibrosing interstitial pneumonia with various etiology. The severity and progression of ILD usually predict the poor outcomes of ILD. Otherwise, Krebs von den Lungen-6 (KL-6) is a potential immunological biomarker reflecting the severity and progression of ILD. This meta-analysis is to clarify the predictive value of elevated KL-6 levels in ILD.MethodEBSCO, PubMed, and Cochrane were systematically searched for articles exploring the prognosis of ILD published between January 1980 and April 2021. The Weighted Mean Difference (WMD) and 95% Confidence Interval (CI) were computed as the effect sizes for comparisons between groups. For the relationship between adverse outcome and elevated KL-6 concentration, Hazard Ratio (HR), and its 95%CI were used to estimate the risk factor of ILD.ResultOur result showed that ILD patients in severe and progressive groups had higher KL-6 levels, and the KL-6 level of patients in the severe ILD was 703.41 (U/ml) than in mild ILD. The KL-6 level in progressive ILD group was 325.98 (U/ml) higher than that in the non-progressive ILD group. Secondly, the KL-6 level of patients in acute exacerbation (AE) of ILD was 545.44 (U/ml) higher than stable ILD. Lastly, the higher KL-6 level in ILD patients predicted poor outcomes. The KL-6 level in death of ILD was 383.53 (U/ml) higher than in survivors of ILD. The pooled HR (95%CI) about elevated KL-6 level predicting the mortality of ILD was 2.05 (1.50–2.78), and the HR (95%CI) for progression of ILD was 1.98 (1.07–3.67).ConclusionThe elevated KL-6 level indicated more severe, more progressive, and predicted the higher mortality and poor outcomes of ILD.

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Section: Discussionmentioning

confidence: 99%

KL-6 as an Immunological Biomarker Predicts the Severity, Progression, Acute Exacerbation, and Poor Outcomes of Interstitial Lung Disease: A Systematic Review and Meta-Analysis

et al. 2021

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“…SSc-PAH has a worse prognosis compared with other CTD-PAH, partially because the PAH of SLE, MCTD, SS, and RA were improved with the administration of immunosuppressive drugs (cyclophosphamide, prednisolone, azathioprine, and mycophenolate mofetil) [18][19][20][21][22][23]. erefore, except for cases of progressive skin and pulmonary fibrosis, we generally do not use immunosuppressive therapies for patients with SSc [24].…”

Section: Discussionmentioning

confidence: 99%

Successful Treatment of Pulmonary Arterial Hypertension in Systemic Sclerosis with Anticentriole Antibody

Ishii

Fujii

Sugimura

et al. 2020

Case Reports in Rheumatology

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Systemic sclerosis (SSc) is characterized by skin sclerosis and multiple organ damages which may cause mortality and is usually accompanied with several specific autoantibodies, each of which is associated with characteristic complications. Among them, anticentriole antibody is recently reported to be highly associated with SSc-associated pulmonary arterial hypertension (SSc-PAH). In general, several vasodilators are used as therapeutic drugs for SSc-PAH, whereas immunosuppressive therapies are not. Here, we report the case of a 62-year-old female with anticentriole antibody-positive SSc-PAH treated with immunosuppressants and vasodilators. She presented with two-year exertional dyspnea and was diagnosed with PAH and SSc owing to the centriole staining pattern and other symptoms without digital sclerosis. Oral vasodilators were initially administered but were not sufficiently effective on dyspnea. Immunosuppressants such as prednisolone and cyclophosphamide were started. Both of them improved mean pulmonary arterial pressure and 6-minute walk distance, and the anticentriole antibody also disappeared. In this case, SSc-PAH with anticentriole antibody was properly diagnosed and immunosuppressants and vasodilators improved the hemodynamics of PAH with anticentriole antibody and stably maintained it and, in addition, reduced the titer of anticentriole antibody. This indicates that anticentriole antibody might represent a good responsive group to therapies among subgroups of patients with SSc-PAH.

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“…In recent years, the safety and efficacy of tacrolimus for treatment of lupus nephritis have been confirmed. [14][15][16] Additionally, tacrolimus is reportedly effective against rheumatoid arthritis, 17 progressive interstitial pneumonia with systemic sclerosis, 18 SS complicated with meningoencephalomyelitis, 19 and interstitial cystitis. 20 Moreover, according to the 2017 British Society for Rheumatology guidelines on the management of adult pSS, external tacrolimus can be used as a substitute for hydroxychloroquine in patients with discomfort.…”

Section: Discussionmentioning

confidence: 99%

Treatment of serologically negative Sjögren’s syndrome with tacrolimus: A case report

et al. 2019

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We herein report an unusual case of primary Sjögren’s syndrome in a 38-year-old woman with typical clinical symptoms (joint pain, dry mouth, and positive Schirmer test) and immunoglobulin G positivity but negativity for antinuclear antibody and all antinuclear antibody spectrum antibodies. Emission computed tomography demonstrated normal ingestion but impaired secretion by the submandibular and bilateral parotid glands. Labial gland biopsy revealed chronic tissue inflammatory changes and Chisholm grade 4 lymphocyte infiltration, confirming primary Sjögren’s syndrome. The patient’s condition was successfully controlled by nonsteroidal treatment with tacrolimus. Patients presenting with chronic dry mouth should be examined by a Schirmer test, lip gland biopsy, and salivary gland emission computed tomography for possible Sjögren’s syndrome, even if serological autoantibodies are negative, to facilitate early intervention. Tacrolimus is a potential treatment option in patients intolerant of steroidal drugs.

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Efficacy and safety of combination therapy with prednisolone and oral tacrolimus for progressive interstitial pneumonia with systemic sclerosis: A retrospective study

Abstract: Combination therapy with PSL and TAC appeared to be well tolerated and effective in suppressing the disease activity of SSc-PIP.

Cited by 12 publications

References 38 publications

KL-6 as an Immunological Biomarker Predicts the Severity, Progression, Acute Exacerbation, and Poor Outcomes of Interstitial Lung Disease: A Systematic Review and Meta-Analysis

KL-6 as an Immunological Biomarker Predicts the Severity, Progression, Acute Exacerbation, and Poor Outcomes of Interstitial Lung Disease: A Systematic Review and Meta-Analysis

Successful Treatment of Pulmonary Arterial Hypertension in Systemic Sclerosis with Anticentriole Antibody

Treatment of serologically negative Sjögren’s syndrome with tacrolimus: A case report

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