Antineutrophil Cytoplasmic Antibody–Associated Lung Fibrosis

Borie, R.; Crestani, Bruno

doi:10.1055/s-0038-1669914

Cited by 33 publications

(16 citation statements)

References 76 publications

(137 reference statements)

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“…However, in MPO-ANCA + patients with MPA, isolated renal disease or isolated pulmonary fibrosis is not infrequent. The recognition of pulmonary fibrosis as a feature of MPA in MPO-ANCA + patients, often as the sole manifestation of disease, has been of some interest 150 . This presentation may be more common in Japan 151,152 , although it does occur in diverse geographical locations.…”

Section: Clinical Presentationmentioning

confidence: 99%

ANCA-associated vasculitis

Kitching

Anders

Basu

et al. 2020

Nat Rev Dis Primers

626

507

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The anti-neutrophil cytoplasmic antibody (ANCA)associated vasculitides (AAVs) are diseases characterized by inflammation of blood vessels, endothelial injury and tissue damage. The three types of small-vessel vasculitis, namely granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic GPA (EGPA; previously known as Churg-Strauss syndrome), feature a loss of tolerance to neutrophil primary granule proteins, most often leukocyte proteinase 3 (PR3; also known as myeloblastin) or myeloperoxidase (MPO) (Table 1). The vessels involved in AAV are typically capillaries, arterioles and venules but small arteries and veins may also be affected. Autoimmunity is documented clinically by serum ANCAs to PR3 (PR3-ANCA) or MPO (MPO-ANCA), which are generally associated with the main syndromic AAV presentations (box 1). AAVs collectively represent one of several types of autoimmune vasculitis (Fig. 1). GPA and MPA can involve small blood vessels in any organ or tissue but commonly affect the upper and lower respiratory tract and the kidneys (box 2). Patients with AAV typically present with severe organ-threatening or life-threatening disease, although less severe presentations also occur. GPA is predominantly associated with PR3-ANCA and its clinical features typically include sinonasal disease, lower respiratory tract involvement with pulmonary haemorrhage and granulomatous inflammation, and glomerulonephritis. MPA is usually associated with MPO-ANCA and clinical features include more severe renal disease and some of the manifestations of GPA but without granulomatous inflammation. EGPA is characterized by asthma, eosinophilia and, in many (but not all) cases, vasculitis. EGPA is less common than GPA or MPA and, in some cases, is associated with ANCAs, mainly MPO-ANCA (Table 1). Although categorized as a form of AAV, EGPA has less overlap with the other AAVs than that between GPA and MPA with regard to its genetic, pathogenetic, and clinical features and its management and is typically considered a separate entity. Improvements in treatment and prognosis for patients with AAV have resulted from the translation of both preclinical and clinical research findings. Here, we provide an updated overview of the clinical and

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Section: Clinical Presentationmentioning

confidence: 99%

ANCA-associated vasculitis

Kitching

Anders

Basu

et al. 2020

Nat Rev Dis Primers

626

507

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“…Some additional items might be considered [ex: esophageal dysmotility, lymphocytic bronchiolitis on biopsy; proximal muscle weakness with myalgia (48)]. Antineutrophil cytoplasmic antibodies (ANCAs) were excluded from the IPAF classification as they are associated with systemic vasculitis, not CTD, however the combination of ILD and ANCAs and anti-myeloperoxydase specificity (49) shares with IPAF many issues related to the overlap between ILD and a chronic, systemic disease.…”

Section: Areas Of Uncertainties and Controversiesmentioning

confidence: 99%

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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“…Some patients may have overlapping features from two different ILDs, thereby not meeting defined/accepted criteria for treatment or entry into disease-specific clinical trials for either disease, for instance patients who we have recently described as having combined sarcoidosis and idiopathic pulmonary fibrosis [121]. Patients with anti-neutrophil cytoplasmic antibodies (ANCA), anti-myeloperoxidase antibody in particular, and pulmonary fibrosis (especially UIP) have been described; it is difficult to know whether to treat these patients with immunosuppressive medications versus an antifibrotic and there are no published studies to guide clinicians in this area [122][123][124][125].…”

Section: Interstitial Pneumonia With Autoimmune Featuresmentioning

confidence: 99%

Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis

2019

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Two antifibrotic medications (nintedanib and pirfenidone) were recommended (conditionally) for the treatment of patients with idiopathic pulmonary fibrosis (IPF) in the 2015 IPF evidence-based guidelines. These medications have been shown to reduce the rate of decline in forced vital capacity among patients with IPF over time and are the only two disease-modulating pharmacological agents approved by regulatory agencies and available for clinical use worldwide. With the evolved standard of care for interstitial lung disease evaluation including routine use of high-resolution computed tomography, fibrotic lung diseases other than IPF are increasingly recognised. In addition, it is becoming evident that genetic and pathophysiological mechanisms as well as disease behaviour in patients manifesting other “non-IPF progressive fibrotic interstitial lung diseases” (non-IPF-PF) may be similar to those in patients with IPF. Thus, it is biologically plausible that pharmacological agents with antifibrotic properties may be efficacious in non-IPF-PF. Indeed, studies are underway or planned to assess the safety and efficacy of nintedanib or pirfenidone among patients with several non-IPF fibrotic lung diseases. In this review, we briefly summarise the use of pirfenidone and nintedanib in IPF as well as the rationale and potential for use of these medications in non-IPF-PF that are being investigated in ongoing and upcoming clinical trials.

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Antineutrophil Cytoplasmic Antibody–Associated Lung Fibrosis

Cited by 33 publications

References 76 publications

ANCA-associated vasculitis

ANCA-associated vasculitis

Interstitial Pneumonia With Autoimmune Features (IPAF)

Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis

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