Cyclophosphamide in steroid refractory unclassifiable idiopathic interstitial pneumonia and interstitial pneumonia with autoimmune features (IPAF)

Wiertz, Ivo A.; Moorsel, Coline H.M. van; Vorselaars, Adriane D.M.; Quanjel, Marian J.R.; Grutters, Jan C.

doi:10.1183/13993003.02519-2017

Cited by 30 publications

(20 citation statements)

References 15 publications

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“…There have been no randomized controlled trials supporting immunomodulation in IPAF, and the proposed treatment strategies are extrapolated from CTD-ILD studies (25, 26). In one study of patients with unclassifiable ILD, intravenous pulse cyclophosphamide was suggested to stabilize lung function (27); a subset of patients in this study had IPAF and these seemed to benefit more from the treatment regimen, although none of them had a UIP pattern. This suggest that patients with IPAF and a non-UIP pattern may benefit from immunomodulation, however this needs confirmation.…”

Section: Managementmentioning

confidence: 85%

Interstitial Pneumonia With Autoimmune Features (IPAF)

et al. 2019

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A significant proportion of patients with interstitial lung disease (ILD) manifest autoimmune features, but do not fulfill the diagnostic criteria for a definite connective tissue disease (CTD). In 2015, the European Respiratory Society (ERS) and American Thoracic Society (ATS) “Task Force on undifferentiated Forms of connective tissue disease-associated interstitial lung disease” proposed classification criteria for a so-called research category of Interstitial Pneumonia with Autoimmune Features (IPAF). These classification criteria were based on a combination of features from three domains: a clinical domain consisting of extra-thoracic features; a serologic domain with specific autoantibodies; and a morphologic domain with imaging patterns, histopathological findings or multi-compartment involvement. Patients meeting IPAF criteria tend to have a history of smoking similar to patients with idiopathic pulmonary fibrosis. The most frequent clinical and serological markers of autoimmune features are Raynaud' phenomenon and positive antinuclear antibodies, respectively. Non-specific interstitial pneumonia is the predominant radiologic and histopathologic pattern, although patients meeting IPAF criteria through the clinical and serologic domains may also have a usual interstitial pneumonia pattern. Management should be carefully individualized on a case-by-case basis in keeping with the wide heterogeneity of IPAF and lack of evidence in this particular subgroup of patients. Prognosis is generally intermediate between that of idiopathic pulmonary fibrosis and connective tissue disease-associated interstitial lung disease, but substantially variable according to the predominant histologic and radiologic patterns. As acknowledged by the Task Force, the proposed classification scheme of IPAF is a research concept that will need revision and refinement based on data to better inform prognostication and patient care.

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Section: Managementmentioning

confidence: 85%

Interstitial Pneumonia With Autoimmune Features (IPAF)

et al. 2019

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“…Depending on the relative likelihoods of different diagnoses in a patient with unclassifiable ILD, either antifibrotic or immunosuppressive therapy may be warranted. The use of immunosuppressive therapies in this situation is based on very limited retrospective data suggesting potential benefit from cyclophosphamide in the subgroup of unclassifiable ILD patients who have autoimmune features [63,64]. Additional clinical trials are ongoing that might provide further evidence on the efficacy and safety of immunosuppressive therapies in patients with non-IPF ILDs (Table 3).…”

Section: Immunosuppressive Therapiesmentioning

confidence: 99%

“…In the absence of robust head-to-head data, it will likely be most appropriate to consider antifibrotic medication in patients with an IPF-like phenotype, particularly those with a UIP pattern on chest imaging or lung biopsy, as well as patients for whom immunosuppression might be associated with greater potential adverse effects. Using immunsuppressive therapy as a firstline option is likely to be most beneficial in patients with a more inflammatory phenotype, and particularly those with an organising pneumonia pattern on chest imaging and other features of active autoimmunity [63,64]. There is a substantial "grey zone" of patients with fibrotic ILD who fall between these two extremes.…”

Section: How Should Clinicians Choose Between Antifibrotic or Immunosmentioning

confidence: 99%

Progression of fibrosing interstitial lung disease

2020

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Fibrotic interstitial lung diseases (ILDs) are often challenging to diagnose and classify, but an accurate diagnosis has significant implications for both treatment and prognosis. A subset of patients with fibrotic ILD experience progressive deterioration in lung function, physical performance, and quality of life. Several risk factors for ILD progression have been reported, such as male sex, older age, lower baseline pulmonary function, and a radiological or pathological pattern of usual interstitial pneumonia. Morphological similarities, common underlying pathobiologic mechanisms, and the consistently progressive worsening of these patients support the concept of a progressive fibrosing (PF)-ILD phenotype that can be applied to a variety of ILD subtypes. The conventional approach has been to use antifibrotic medications in patients with idiopathic pulmonary fibrosis (IPF) and immunosuppressive medications in patients with other fibrotic ILD subtypes; however, recent clinical trials have suggested a favourable treatment response to antifibrotic therapy in a wider variety of fibrotic ILDs. This review summarizes the literature on the evaluation and management of patients with PF-ILD, and discusses questions relevant to applying recent clinicial trial findings to real-world practice.

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“…Therefore, the treatment for IPAF would cover both of the two sides. Wiertz et al [29] have reported that IPAF patients may bene t from cyclophosphamide treatment. Besides, McCoy et al [30] have shown that mycophenolate therapy can attenuate disease progression in IPAF patients.…”

Section: Discussionmentioning

confidence: 99%

The Role of Pirfenidone in the Treatment of Interstitial Pneumonia With Autoimmune Features

Chen

Zhang

et al. 2020

Preprint

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Rationale: No approved pharmacotherapies are available for patients with interstitial pneumonia with autoimmune features (IPAF). Objective: In the present work, we aimed to evaluate the efficacy and safety of pirfenidone for the treatment of IPAF.Methods: A retrospective cohort study consisting of patients who met diagnostic criteria for IPAF was performed after a multidisciplinary review, and the patients receiving pirfenidone were compared with those in the non-pirfenidone group. The baseline data and diagnostic characteristics of patients were assessed. Pulmonary function and prednisone dose were analyzed by a mix-effects model.Results: A total of 184 patients, who met the diagnostic criteria of IPAF, were divided into two groups: pirfenidone group (n=81) and non-pirfenidone group (n=103). Patients in the pirfenidone group had a lower forced vital capacity (FVC%, P< 0.001) and a lower diffusion capacity for carbon monoxide (DLCO%, P=0.003). The pirfenidone group exhibited a greater increase of FVC% at 6 (P=0.003), 12 (P=0.013), and 24 (P=0.003) months. After adjustment for sex, age, UIP pattern, baseline FVC% and DLCO%, patients in the pirfenidone group continued to show a greater improvement in FVC% (χ2 (1) =4.59, P=0.032). Subgroup analysis identified superior therapeutic effects of pirfenidone in patients with dosage > 600 mg/day (P = 0.010) and medication course > 12 months (P = 0.007). Besides, the pirfenidone group had a lower prednisone dose than the non-pirfenidone group after 12 months of treatment (P=0.002). Moreover, 17 patients (19.32%) experienced side effects after taking pirfenidone, including one case of anaphylactic shock.Conclusions: Pirfenidone (600-1,800 mg/day) might help improve FVC, with an acceptable safety and tolerability profile in IPAF patients.

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Cyclophosphamide in steroid refractory unclassifiable idiopathic interstitial pneumonia and interstitial pneumonia with autoimmune features (IPAF)

Cited by 30 publications

References 15 publications

Interstitial Pneumonia With Autoimmune Features (IPAF)

Interstitial Pneumonia With Autoimmune Features (IPAF)

Progression of fibrosing interstitial lung disease

The Role of Pirfenidone in the Treatment of Interstitial Pneumonia With Autoimmune Features

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