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Pulmonary alveolar proteinosis

Gabriela Barcenas-Morales, Peter Jandus, Rainer Döffinger
PURPOSE OF REVIEW: Concise overview of the field of anticytokine autoantibodies with a focus on recent developments. RECENT FINDINGS: Advances in particular in the analysis of autoantibodies to IFNγ, granulocyte-macrophage colony-stimulating factor (GM-CSF) and IFN-1 are presented. The target epitope for anti-IFNγ autoantibodies has been found to have high homology to a protein from Aspergillus suggesting molecular mimicry as a mechanism of breaking self-tolerance...
October 13, 2016: Current Opinion in Allergy and Clinical Immunology
A Jézéquel, M Kerjouan, M Lederlin, C Lainé-Caroff, C Camus, P Delaval, S Jouneau
INTRODUCTION: Auto-immune pulmonary alveolar proteinosis is a rare disorder characterized by the accumulation of surfactant proteins in the alveoli. CASE REPORT: We report a case of a 41-year-old smoker, presenting initially with acute respiratory failure. Whole lung lavages were effective initially but only for a few weeks. GM-CSF subcutaneous injections were not effective, and then plasmapheresis were tried. CONCLUSION: This is the fifth report of the use of this treatment in auto-immune pulmonary alveolar proteinosis...
October 13, 2016: Revue des Maladies Respiratoires
Kamonpun Ussavarungsi, Ryan M Kern, Anja C Roden, Jay H Ryu, Eric S Edell
BACKGROUND: Diagnostic evaluation of patients with diffuse parenchymal lung disease (DPLD) is best achieved by a multidisciplinary team correlating clinical, radiologic, and pathologic features. Surgical lung biopsies remain the gold standard for histopathologic diagnosis of idiopathic interstitial pneumonias. Emerging data suggest an increasing role for transbronchial cryobiopsy (TBC) in DPLD evaluation. We describe our experience with TBC in patients with DPLD. METHODS: We retrospectively reviewed medical records of patients with radiographic features of DPLD who underwent TBC at Mayo Clinic, Rochester, Minnesota in June 2013-September 2015...
September 19, 2016: Chest
JiuWu Bai, JinFu Xu, WenLan Yang, Beilan Gao, Weijun Cao, Shuo Liang, Huiping Li
Background. Pulmonary Alveolar Proteinosis (PAP) is a syndrome characterized by pulmonary surfactant accumulation. Small proportion of PAP patients experienced spontaneous remission. Objective. The aim of this study was to assess the severity and prognosis of PAP using various indexes. Methods. Characteristics, PaO2, lung function parameters, and HRCT score of 101 patients with PAP were retrospectively analyzed. Many indexes were explored and integrated into a scale. Results. PaO2 was lower among smokers than among never-smokers...
2016: Canadian Respiratory Journal: Journal of the Canadian Thoracic Society
Masahiro Takaki, Takeshi Tanaka, Yoshihiro Komohara, Yoshiko Tsuchihashi, Daisuke Mori, Kentaro Hayashi, Junya Fukuoka, Naoya Yamasaki, Takeshi Nagayasu, Koya Ariyoshi, Konosuke Morimoto, Koh Nakata
Hereditary pulmonary alveolar proteinosis (PAP) caused by mutations in CSF2RA or CSF2RB, which encode GM-CSF receptor α and β respectively, is a rare disease. Although some experimental therapeutic strategies have been proposed, no clinical evidence has yet been reported. We herein describe the clinical course and recurrence of hereditary PAP after lung transplantation. A 36-year-old woman developed PAP of unknown etiology. She underwent bilateral lung transplantation from living donors at the age of 42 years because of severe respiratory failure complicated by pulmonary fibrosis...
2016: Respiratory Medicine Case Reports
Mirjana Trukalj, Marija Perica, Željko Ferenčić, Damir Erceg, Marta Navratil, Gzim Redžepi, Boro Nogalo
BACKGROUND Pulmonary alveolar proteinosis (PAP) is a rare condition characterized by the intra-alveolar accumulation of surfactant-derived material, which impairs gas exchange and results in respiratory insufficiency. Two major subtypes of PAP are autoimmune and non-autoimmune PAP. The diagnosis relies on clinical presentation, ground glass opacities on CT scan, bronchoscopy with PAS stain of BAL fluid (BALF), lung biopsy with PAS-positive material in the alveoli, and the presence of anti GM-CSF antibodies in serum or BALF for an autoimmune subtype...
2016: American Journal of Case Reports
Ali Berkant Avci, Eugen Feist, Gerd-Rüdiger Burmester
Granulocyte-macrophage colony-stimulating factor (GM-CSF) is well-known as a haemopoietic growth factor. However, it is also essential in regulating functions of mature myeloid cells such as macrophages. Preclinical studies and observations of flares of arthritis in patients following GM-CSF treatment supported its important contribution to the pathogenesis of rheumatoid arthritis (RA). As the most advanced compound, mavrilimumab, a monoclonal antibody against GM-CSF receptor, has already completed phase II trials with a long term of follow-up period of 74 weeks...
July 2016: Clinical and Experimental Rheumatology
Ilaria Campo, Maurizio Luisetti, Matthias Griese, Bruce C Trapnell, Francesco Bonella, Jan Grutters, Koh Nakata, Coline H M Van Moorsel, Ulrich Costabel, Vincent Cottin, Toshio Ichiwata, Yoshikazu Inoue, Antonio Braschi, Giacomo Bonizzoni, Giorgio A Iotti, Carmine Tinelli, Giuseppe Rodi
BACKGROUND: Whole lung lavage (WLL) is the current standard of care treatment for patients affected by pulmonary alveolar proteinosis (PAP). However, WLL is not standardized and international consensus documents are lacking. Our aim was to obtain a factual portrayal of WLL as currently practiced with respect to the procedure, indications for its use, evaluation of therapeutic benefit and complication rate. METHODS: A clinical practice survey was conducted globally by means of a questionnaire and included 27 centers performing WLL in pediatric and/or adult PAP patients...
2016: Orphanet Journal of Rare Diseases
F N Kaya, A S Bayram, F Kirac, E Basagan-Mogol, S Goren
No abstract text is available yet for this article.
September 2016: Anesthesia and Analgesia
Jamie Campbell, Josquin Nys, Laura Eghobamien, E Suzanne Cohen, Matthew J Robinson, Matthew A Sleeman
Pulmonary alveolar proteinosis is associated with impaired alveolar macrophage differentiation due to genetic defects in the granulocyte macrophage colony-stimulating factor (GM-CSF) axis or autoantibody blockade of GM-CSF. The anti-GM-CSFRα antibody mavrilimumab has shown clinical benefit in patients with rheumatoid arthritis, but with no accompanying pulmonary pathology observed to date. We aimed to model systemic versus pulmonary pharmacodynamics of an anti-GM-CSFRα antibody to understand the pharmacology that contributes to this therapeutic margin...
August 12, 2016: MAbs
Masanori Akira, Yoshikazu Inoue, Toru Arai, Chikatoshi Sugimoto, Sayoko Tokura, Koh Nakata, Masanori Kitaichi
OBJECTIVE: The CT findings of pulmonary fibrosis in patients with pulmonary alveolar proteinosis (PAP) are not yet well defined. The objective of this study was to evaluate the CT findings of PAP with a focus on pulmonary fibrosis secondary to PAP. MATERIALS AND METHODS: High-resolution CT (HRCT) scans of 44 patients with PAP were evaluated retrospectively with a focus on pulmonary fibrosis: 33 patients had autoimmune PAP, and 11 patients had secondary PAP. The intervals between the initial and last CT examinations ranged from 1 to 284 months (median, 60 months)...
September 2016: AJR. American Journal of Roentgenology
Zhengwang Cao, Yiliang Fang, Yonghui Lu, Fenghua Qian, Qinglong Ma, Mingdi He, Huifeng Pi, Zhengping Yu, Zhou Zhou
With recent advances in the manufacture and application of nickel oxide nanoparticles (NiONPs), concerns about their adverse effects on the respiratory system are increasing. However, the underlying cellular and molecular mechanisms of NiONP-induced pulmonary toxicity remain unclear. In this study, we focused on the impacts of NiONPs on pulmonary inflammation and investigated whether the NLRP3 inflammasome is involved in NiONP-induced pulmonary inflammation and injury. NiONP suspensions were administered by single intratracheal instillation to rats, and inflammatory responses were evaluated at 3 days, 7 days, or 28 days after treatment...
2016: International Journal of Nanomedicine
Berna Botan Yildirim, Recep Akgedik, Sukran Akgedik, Hasan Nazaroglu
Pulmonary alveolar proteinosis (PAP) is a rarely seen disease of the alveoli, characterized by accumulation of proteinous material, which stains positive with periodic acid Schiff, in the alveoli. Secondary PAP may develop as a result of occupational exposure to materials such as silica and indium. In the paper, together with a review of the relevant literature, we present an uncommon case of a 47-year old male, marble worker who was diagnosed with PAP associated with a 12-year history of exposure to marble dust...
2016: International Journal of Occupational Medicine and Environmental Health
Takuji Suzuki, Bruce C Trapnell
Pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by the accumulation of surfactant in alveoli and terminal airways resulting in respiratory failure. PAP comprises part of a spectrum of disorders of surfactant homeostasis (clearance and production). The surfactant production disorders are caused by mutations in genes required for normal surfactant production. The PAP syndrome is identified based on history, radiologic, and bronchoalveolar lavage and/or histopathologic findings. The diagnosis of PAP-causing diseases in secondary PAP requires further studies...
September 2016: Clinics in Chest Medicine
Joy Sha, David Langton
Pulmonary alveolar proteinosis (PAP) is a rare diffuse lung disease characterized by accumulation of lipoproteinacious material in alveoli, with distinct features on high resolution computed tomography and biopsy. Its association with pulmonary fibrosis is infrequently encountered, and a clear understanding of the underlying pathogenesis is yet to be established. We report the case of a 48-year-old woman with known autoimmune PAP (aPAP) first diagnosed 20 years ago, who presented with worsening hypoxemia and radiological features consistent with pulmonary fibrosis, after many years of stable disease...
July 2016: Respirology Case Reports
Yikuan Feng, Jianping Zhao, Qun Yang, Weining Xiong, Guohua Zhen, Yongjian Xu, Zhenxiang Zhang, Huilan Zhang
BACKGROUND: In the lung, melanoma is mostly arranged as patterns of multiple nodules, solitary nodules, or miliary invasions. Very rarely, it also displays a "crazy paving" pattern (also described as a "paving stone," "flagstone," or "slabstone" pattern), which is rarer still in discrete bilateral nodules. This pattern is considered to be caused by pulmonary alveolar proteinosis, but its association with various diseases is unclear. CASE PRESENTATION: A 60-year-old man was diagnosed with pulmonary melanoma...
2016: BMC Cancer
Harry Whitwell, Rose-Marie Mackay, Christine Elgy, Cliff Morgan, Mark Griffiths, Howard Clark, Paul Skipp, Jens Madsen
The formation of protein coronae on nanoparticles (NPs) has been investigated almost exclusively in serum, despite the prevailing route of exposure being inhalation of airborne particles. In addition, an increasing number of nanomedicines, that exploit the airways as the site of delivery, are undergoing medical trials. An understanding of the effects of NPs on the airways is therefore required. To further this field, we have described the corona formed on polystyrene (PS) particles with different surface modifications and on titanium dioxide particles when incubated in human bronchoalveolar lavage fluid (BALF) from patients with pulmonary alveolar proteinosis (PAP)...
November 2016: Nanotoxicology
Xiaojing Jiang, Xiuzhu Sun, Weihua Du, Haisheng Hao, Xueming Zhao, Dong Wang, Huabin Zhu, Yan Liu
Pulmonary surfactant (PS) is synthesized and secreted by alveolar epithelial type II (AEII) cells, which is a complex compound formed by proteins and lipids. Surfactant participates in a range of physiological processes such as reducing the surface tension, keeping the balance of alveolar fluid, maintaining normal alveolar morphology and conducting host defense. Genetic disorders of the surfactant homeostasis genes may result in lack of surfactant or cytotoxicity, and lead to multiple lung diseases in neonates, children and adults, including neonatal respiratory distress syndrome, interstitial pneumonia, pulmonary alveolar proteinosis, and pulmonary fibrosis...
August 2016: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
Adele Mucci, Jessica Kunkiel, Takuji Suzuki, Sebastian Brennig, Silke Glage, Mark P Kühnel, Mania Ackermann, Christine Happle, Alexandra Kuhn, Axel Schambach, Bruce C Trapnell, Gesine Hansen, Thomas Moritz, Nico Lachmann
Induced pluripotent stem cells (iPSCs) represent an innovative source for the standardized in vitro generation of macrophages (Mφ). We here describe a robust and efficient protocol to obtain mature and functional Mφ from healthy as well as disease-specific murine iPSCs. With regard to morphology, surface phenotype, and function, our iPSC-derived Mφ (iPSC-Mφ) closely resemble their counterparts generated in vitro from bone marrow cells. Moreover, when we investigated the feasibility of our differentiation system to serve as a model for rare congenital diseases associated with Mφ malfunction, we were able to faithfully recapitulate the pathognomonic defects in GM-CSF signaling and Mφ function present in hereditary pulmonary alveolar proteinosis (herPAP)...
August 9, 2016: Stem Cell Reports
Ryan R Kroll, Sameer Kumar, Ronald F Grossman, Charles Price, John R Srigley
Pulmonary alveolar proteinosis (PAP) is a rare condition characterized by dysfunctional alveolar macrophages, which ineffectively clear surfactant and typically cause mild hypoxemia. Characteristic Computed Tomography findings are septal reticulations superimposed on ground-glass opacities in a crazy paving pattern, with a clear juxtaposition between affected and unaffected parenchyma. While traditionally PAP was diagnosed via biopsy, bronchoalveolar lavage (BAL) is usually sufficient; the fluid appears milky, and on microscopic examination there are foamy macrophages with eosinophilic granules and extracellular hyaline material that is Periodic Acid-Schiff positive...
2016: Canadian Respiratory Journal: Journal of the Canadian Thoracic Society
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