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Seminars in Respiratory and Critical Care Medicine

Benjamin Terrier, Loïc Guillevin
Pulmonary vasculitis can be heterogeneous and have some pathophysiological, clinical, and biological specificities that might influence treatment choices. Main vasculitides associated with pulmonary vasculitis include antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, immune complex small vessel vasculitis, and antiglomerular basement membrane disease. Patient characteristics and association of lung involvement with other manifestations affecting disease severity may also influence treatment strategy...
August 2018: Seminars in Respiratory and Critical Care Medicine
Stephen P McAdoo, Charles D Pusey
Antiglomerular basement membrane (anti-GBM) disease is a rare but life-threatening autoimmune vasculitis that is characterized by the development of pathogenic autoantibodies to type IV collagen antigens expressed in the glomerular and alveolar basement membranes. Once deposited in tissue, these autoantibodies incite a local capillaritis which manifests as rapidly progressive glomerulonephritis (GN) in 80 to 90% of patients, and with concurrent alveolar hemorrhage in ∼50%. A small proportion of cases may present with pulmonary disease in isolation...
August 2018: Seminars in Respiratory and Critical Care Medicine
Mouhamad Nasser, Vincent Cottin
Defined by the accumulation of red blood cells into the alveolar space, diffuse alveolar hemorrhage (DAH) is a severe and potentially fatal medical condition requiring careful attention. In contrast to simple extravasation of erythrocytes facilitated by impaired hemostasis or hemodynamic causes, DAH in vasculitis is due to capillaritis, that is, inflammation of capillaries. Dyspnea, hemoptysis, chest infiltrates, and abrupt fall of blood hemoglobin level represent the cardinal features of DAH; yet, hemoptysis is lacking in one-third of cases...
August 2018: Seminars in Respiratory and Critical Care Medicine
Yann Nguyen, Loïc Guillevin
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg-Strauss syndrome, is a systemic necrotizing vasculitis of small- and medium-size vessels, characterized by asthma and blood eosinophilia. EGPA typically occurs in patients with preexisting asthma, and involves the skin, lungs, and peripheral nerves. Poor-prognosis factors (i.e., involvement(s) of the gastrointestinal tract, heart, and/or kidney) have been assessed with the Five-Factor Score (FFS). One-third of the patients have anti-myeloperoxidase antineutrophil cytoplasm antibodies, and their presence seems to differentiate between two phenotypes, with different clinical characteristics and prognoses...
August 2018: Seminars in Respiratory and Critical Care Medicine
Raphael Borie, Bruno Crestani
Pulmonary fibrosis is observed in a substantial number of patients with ANCA-associated vasculitis (AAV), 15% in a recent German series, and may be more frequent in Asian populations. ANCA are usually of anti-MPO specificity and microscopic polyangiitis is the most frequent vasculitis. Pulmonary fibrosis may increase the risk of death in patients with AAV. Treatment for AAV in patients with lung fibrosis should follow the international guidelines for vasculitis. The role of anti-fibrotic drugs (pirfenidone, nintedanib) in this condition is still unknown...
August 2018: Seminars in Respiratory and Critical Care Medicine
Alexandre Karras
Microscopic polyangiitis (MPA) is one of the main clinical presentations of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Although the disease is defined by clinical and pathological criteria, the anti-myeloperoxidase (MPO) specificity of ANCAs is observed in almost 80% of MPA patients. The direct pathogenic role of anti-MPO antibodies has been proven in animal models, in which the disease was transmitted by transfer of anti-MPO antibodies or anti-MPO-specific splenocytes. The most frequently affected organs in this disease are the kidneys and the lungs...
August 2018: Seminars in Respiratory and Critical Care Medicine
Joseph P Lynch, Ariis Derhovanessian, Henry Tazelaar, John A Belperio
Granulomatosis with polyangiitis (GPA), formerly termed Wegener's granulomatosis , is the most common of the pulmonary vasculitides. GPA typically involves the upper respiratory tract, lower respiratory tract (bronchi and lung), and kidney, with varying degrees of disseminated vasculitis. Cardinal histologic features include a necrotizing vasculitis involving small vessels, extensive "geographic" necrosis, and granulomatous inflammation. The spectrum and severity of the disease is heterogeneous, ranging from indolent disease involving only one site to fulminant, multiorgan vasculitis...
August 2018: Seminars in Respiratory and Critical Care Medicine
Jason V Scapa, Gregory A Fishbein, W Dean Wallace, Michael C Fishbein
Vasculitides are a heterogeneous group of disorders in which inflammation of blood vessel walls is present at least some time during the course of the disease. Vasculitides can affect any caliber or type of vessel in many anatomic sites; however, the disease can alter more than just vasculature. Given the diversity of vasculitides, in 2012, a revised classification system was proposed to categorize vasculitides by the type of vessel involved including size, function, and structural attributes. In the lung, vasculitis impacts both the pulmonary vessels and parenchyma...
August 2018: Seminars in Respiratory and Critical Care Medicine
Marco A Alba, J Charles Jennette, Ronald J Falk
Antineutrophil cytoplasmic antibodies (ANCAs) are autoantibodies specific for antigens located in the cytoplasmic granules of neutrophils and lysosomes of monocytes. ANCAs are associated with a spectrum of necrotizing vasculitis that includes granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis. Pulmonary vasculitis and related extravascular inflammation and fibrosis are frequent components of ANCA vasculitis. In this review, we detail the factors that have been associated with the origin of the ANCA autoimmune response and summarize the most relevant clinical observations, in vitro evidence, and animal studies strongly indicating the pathogenic potential of ANCA...
August 2018: Seminars in Respiratory and Critical Care Medicine
Loïc Guillevin
No abstract text is available yet for this article.
August 2018: Seminars in Respiratory and Critical Care Medicine
Michael R Holt, Shannon Kasperbauer
Extrapulmonary disease occurs in a minority of nontuberculous mycobacterial (NTM) infections. The pattern of disease tends to be multifocal in immunocompromised individuals and localized in the immunocompetent. There is increasing recognition of disseminated Mycobacterium chimaera infection, as a complication of cardiac surgery, and focal infections due to rapidly growing mycobacteria. Microbiologic diagnosis requires detection of NTM in blood or tissue samples by microscopy, culture, or molecular methods. Management of extrapulmonary NTM infection requires prolonged, targeted multiple-drug therapy and, in some cases, aggressive surgical intervention...
June 2018: Seminars in Respiratory and Critical Care Medicine
John D Mitchell
There is renewed interest in the use of adjuvant surgical resection in the treatment of pulmonary mycobacterial disease. For pulmonary Mycobacterium tuberculosis , the emergence of significant drug resistance has led clinicians to reconsider surgery in select cases, where a clear benefit in bacterial conversion and cure has been noted. Less data exist for the use of anatomic resection in the setting of pulmonary nontuberculous mycobacterial disease, although multiple reports have supported the use of surgery in select cases...
June 2018: Seminars in Respiratory and Critical Care Medicine
Brian S Furukawa, Patrick A Flume
Nontuberculous mycobacteria (NTM) can cause chronic pulmonary infection in susceptible hosts. Individuals with cystic fibrosis (CF), a multisystem disease predominated by progressive structural lung disease, are particularly vulnerable. Only recently have NTM been recognized for their potential to cause lung deterioration in CF patients. The reported prevalence varies widely from 4 to 40%, significantly more common than in the general population, but this varies because of multiple factors including inconsistent screening practices...
June 2018: Seminars in Respiratory and Critical Care Medicine
Damien Basille, Vincent Jounieaux, Claire Andréjak
Nontuberculous mycobacteria (NTM) are numerous, and for the vast majority of them, randomized studies are lacking and data regarding optimal treatment are limited. When Mycobacterium avium complex (MAC) and M. abscessus are excluded, the main NTM are M. xenopi, M. kansasii, M. malmoense, M. szulgai , and M. simiae . Treatment is long (at least 12 months after culture conversion according to recommendations by scientific societies) and difficult (at least three drugs are required, each of which have potential adverse events)...
June 2018: Seminars in Respiratory and Critical Care Medicine
Luke Strnad, Kevin L Winthrop
Of the nontuberculous mycobacteria (NTMs) causing lung disease, members of the Mycobacterium abscessus complex (MABc) present a formidable obstacle to successful management. This challenge starts from a poorly understood pathogenesis, continues with complicated subspecies variation in treatment response, and extends to the multidrug-resistant nature of these organisms. The disease often necessitates the use of intravenous therapy, toxic drug combinations, and, in some cases, lung resection. Like many NTMs, MABc treatment requires prolonged therapy with multiple medications, and pulmonary disease in some subspecies can be impossible to eradicate or cure...
June 2018: Seminars in Respiratory and Critical Care Medicine
David E Griffith
Mycobacterium avium complex (MAC) is the most commonly isolated nontuberculous mycobacterial respiratory pathogen worldwide. MAC lung disease is manifested either by fibrocavitary radiographic changes similar to pulmonary tuberculosis or by bronchiectasis with nodular and reticulonodular radiographic changes. This latter form of MAC lung disease, termed "nodular bronchiectatic (NB) MAC lung disease" is the most common form of MAC lung disease in the United States. Treatment at the time of diagnosis is always indicated for fibrocavitary MAC lung disease because it is always progressive and associated with increased morbidity and mortality compared with NB MAC lung disease...
June 2018: Seminars in Respiratory and Critical Care Medicine
Steven A Cowman, Michael R Loebinger
The diagnosis of pulmonary nontuberculous mycobacteria (NTM) disease may be challenging, as their presence alone does not necessarily indicate disease and diagnosis requires the integration of clinical, radiological, and microbiological findings. The first step is to suspect NTM disease; however, clinical manifestations of NTM are nonspecific and it may not be possible to separate them from those caused by underlying respiratory disease. The radiological appearance generally falls into two patterns, fibrocavitary disease and nodular-bronchiectatic disease; consolidation, infiltrates, and solitary nodules are also described...
June 2018: Seminars in Respiratory and Critical Care Medicine
Sanne M H Zweijpfenning, Jakko van Ingen, Wouter Hoefsloot
Isolation frequency of nontuberculous mycobacterial (NTM) differs per region. Differences in isolation frequency as well as frequencies in clinical relevance are relevant for daily clinical practice. We conducted a systematic review, searching PubMed to assess these differences. Mycobacterium avium complex (MAC) is the most frequently isolated species and the majority of MAC isolates are causative agents of clinically relevant disease, that is, the patient ultimately meets American Thoracic Society/Infectious Disease Society of America diagnostic criteria for NTM pulmonary disease...
June 2018: Seminars in Respiratory and Critical Care Medicine
Jennifer Adjemian, Shelby Daniel-Wayman, Emily Ricotta, D Rebecca Prevots
Annual prevalence estimates for pulmonary nontuberculous mycobacterial (PNTM) disease in the contiguous United States range from 1.4 to 13.9 per 100,000 persons, while one study found an annual prevalence of up to 44 per 100,000 persons in Hawaii. PNTM prevalence varies by region, sex, and race/ethnicity, with higher prevalence among women and persons of Asian ancestry, as well as in the Southern United States and Hawaii. Studies consistently indicate that PNTM prevalence is increasing, with estimates ranging from 2...
June 2018: Seminars in Respiratory and Critical Care Medicine
Charles L Daley, Jose A Caminero
Drug-resistant strains of Mycobacterium tuberculosis pose a major threat to global tuberculosis control. Despite the availability of curative antituberculosis therapy for nearly half a century, inappropriate and inadequate treatment of tuberculosis, as well as unchecked transmission of M. tuberculosis , has resulted in alarming levels of drug-resistant tuberculosis. The World Health Organization (WHO) estimates that there were 600,000 cases of multidrug-resistant tuberculosis (MDR-TB)/rifampin-resistant (RR) tuberculosis in 2016, defined as strains that are resistant to at least isoniazid and rifampicin...
June 2018: Seminars in Respiratory and Critical Care Medicine
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