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https://www.readbyqxmd.com/read/27896807/fdg-pet-ct-imaging-of-therapeutic-response-in-granulomatous-lymphocytic-interstitial-lung-disease-glild-in-common-variable-immunodeficiency-cvid
#1
S Jolles, E Carne, M Brouns, T El-Shanawany, P Williams, C Marshall, P Fielding
Common variable immunodeficiency (CVID) is the most common severe adult primary immunodeficiency and is characterized by a failure to produce antibodies leading to recurrent predominantly sinopulmonary infections. Improvements in the prevention and treatment of infection with immunoglobulin replacement and antibiotics have resulted in malignancy, autoimmune, inflammatory and lymphoproliferative disorders emerging as major clinical challenges in the management of patients who have CVID. In a proportion of CVID patients, inflammation manifests as granulomas that frequently involve the lungs, lymph nodes, spleen and liver and may affect almost any organ...
November 28, 2016: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/27492372/x-linked-inhibitor-of-apoptosis-complicated-by-granulomatous-lymphocytic-interstitial-lung-disease-glild-and-granulomatous-hepatitis
#2
Cathal L Steele, Matthew Doré, Sandra Ammann, Maurice Loughrey, Angeles Montero, Siobhan O Burns, Emma C Morris, Bobby Gaspar, Kimberly Gilmour, Shahnaz Bibi, Hiba Shendi, Lisa Devlin, Carsten Speckmann, David M Edgar
The X-linked inhibitor of apoptosis (XIAP) deficiency is a primary immunodeficiency characterized by Epstein-Barr virus (EBV)-driven hemophagocytic lymphohistiocytosis (HLH), splenomegaly, and colitis. Here, we present, for the first time, granulomatous hepatitis and granulomatous and lymphocytic interstitial lung disease (GLILD) as manifestations of XIAP deficiency. We report successful treatment of GLILD in XIAP deficiency with rituximab and azathioprine and discuss the role of XIAP deficiency in immune dysregulation...
October 2016: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/27335365/management-of-granulomatous-lymphocytic-interstitial-lung-disease-in-a-patient-with-common-variable-immune-deficiency
#3
Mohini Pathria, Daniel Urbine, Marc Stuart Zumberg, Juan Guarderas
A 61-year-old woman presented with longstanding cough and progressive dyspnoea. She underwent an extensive evaluation and was diagnosed with common variable immunodeficiency (CVID) with granulomatous lymphocytic interstitial lung disease (GLILD). She was initially treated with subcutaneous immunoglobulin therapy, having declined intravenous immunoglobulin (IVIG) therapy. She also declined treatment with oral glucocorticoids. Over several months, she became increasingly symptomatic and developed increased pulmonary infiltrates, pleural effusions, mediastinal adenopathy, splenomegaly, pancytopenia and ascites...
2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27243233/granulomatous-lymphocytic-interstitial-lung-disease-as-the-first-manifestation-of-common-variable-immunodeficiency
#4
Basheer Tashtoush, Roya Memarpour, Jose Ramirez, Pablo Bejarano, Jinesh Mehta
Common variable immunodeficiency (CVID) is one of the most common primary immunodeficiencies, which is characterized by reduced serum immunoglobulin levels and B-lymphocyte dysfunction. There are many clinical manifestations of this disease, the most common of which are recurrent respiratory tract infections. Among the most recently recognized autoimmune manifestation of CVID is a disease described as granulomatous-lymphocytic interstitial lung disease (GLILD), where CVID coexists with a small airway lymphoproliferative disorder, mimicking follicular bronchiolitis, or lymphocytic interstitial pneumonitis (LIP) on histology specimens...
May 31, 2016: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/27064856/clinical-predictors-of-a-diagnosis-of-common-variable-immunodeficiency-related-granulomatous-lymphocytic-interstitial-lung-disease
#5
Amar Mannina, Jonathan H Chung, Jeffrey J Swigris, Joshua J Solomon, Tristan J Huie, Zulma X Yunt, Tho Q Truong, Kevin K Brown, Rosane Duarte Achcar, Amy L Olson, Christian W Cox, Seth J Kligerman, Douglas Curran-Everett, Evans R Fernández Pérez
RATIONALE: Granulomatous-lymphocytic interstitial lung disease (GLILD) has emerged as a major cause of morbidity in patients with common variable immunodeficiency (CVID). While GLILD is among the most serious noninfectious pulmonary complications of CVID, risk factors for this condition have not been reported. OBJECTIVES: To identify clinical, physiologic, and serologic risk factors for GLILD in adults with CVID. METHODS: Of 345 consecutive adult patients with CVID, we identified 34 in the National Jewish Health research database who had a radiographic-pathologic diagnosis of GLILD evaluated between 2002 and 2014...
July 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/26071128/-lung-disease-in-adult-common-variable-immunodeficiency
#6
REVIEW
J Hadjadj, M Malphettes, C Fieschi, E Oksenhendler, A Tazi, A Bergeron
INTRODUCTION: Common variable immunodeficiency (CVID) is characterized by a defect in antibody production and may be complicated by infectious or non-infectious respiratory disease. BACKGROUND: In addition to recurrent infectious complications, mainly due to encapsulated bacteria, CVID may be complicated by diffuse infiltrative, non-infectious lung disease. The latter may be related to granulomatosis, lymphoid interstitial pneumonia, follicular bronchiolitis, follicular nodular hyperplasia, organizing pneumonia or lymphoma...
December 2015: Revue des Maladies Respiratoires
https://www.readbyqxmd.com/read/25007085/sarcoidosis-and-common-variable-immunodeficiency-similarities-and-differences
#7
REVIEW
James W Verbsky, John M Routes
Common variable immunodeficiency (CVID) is a primary immunodeficiency that is characterized by hypogammaglobulinemia and poor/absent specific antibody production. Granulomatous and lymphocytic interstitial lung disease (GLILD) is an increasingly recognized complication of CVID, occurring in 10 to 20% of patients. GLILD is characterized by non-necrotizing granuloma, lymphocytic interstitial pneumonitis and follicular bronchiolitis-histological patterns that are typically present in the same biopsy. GLILD is a multisystem disease and is frequently accompanied by diffuse adenopathy, splenomegaly, and extrapulmonary granulomatous disease most commonly in the lymph nodes, spleen, liver, and gastrointestinal tract...
June 2014: Seminars in Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/24638297/common-variable-immunodeficiency-and-bronchiectasis-an-easily-missed-common-association
#8
Khalid Sherani, Hineshkumar Upadhyay, Abhay Vakil, Kelly Cervellione, Craig Thurm
SESSION TITLE: Infectious Disease Case Report Posters ISESSION TYPE: Case Report PosterPRESENTED ON: Sunday, March 23, 2014 at 01:15 PM - 02:15 PMINTRODUCTION: The etiology of bronchiectasis is varied with abnormal host defenses being one of the causes. We report the case of a 49-year-old male presenting with recurrent pneumonias. Imaging studies revealed bronchiectasis. Work up for bronchiectasis showed low immunoglobulin levels consistent with common variable immunodeficiency (CVID)...
March 1, 2014: Chest
https://www.readbyqxmd.com/read/24630587/-combined-variable-immunodeficiency-with-unusal-features-a-case-report
#9
F De Charry, A Peterschmitt Tonetti, C De Charry, L Baseggio, F Pasquet, S Khenifer, R Rassat, M Pavic
INTRODUCTION: The association granulomatosis - combined variable immunodeficiency (CVID) - is well known from the clinicians. However, the association with a large granular lymphocyte (LGL) leukemia has not been yet reported. CASE REPORT: We report a 50-year-old woman, followed for CVID associated with a granulomatous disease. During the follow-up, the patient developed a granulomatous lymphocytic interstitiel lung disease (GLILD). Secondarily, she presented a LGL leukemia...
May 2015: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/24354253/-granulomatous-lymphocytic-interstitial-lung-disease-in-common-variable-immunodeficiency
#10
M Prella Bianchi, I Letovanec, F Spertini, L P Nicod, R Lazor
Common variable immunodeficiency (CVID) is the most frequent primary immune deficiency. Recurrent infections are classical consequences of CVID, but their impact has been largely reduced by immunoglobulin replacement. CVID is also associated with various inflammatory and autoimmune manifestations resulting from abnormal cellular immunity. The lungs are especially affected by a recently described entity called granulomatous lymphocytic interstitial lung disease (GLILD). GLILD currently constitutes an important cause of morbidity and mortality in these patients...
November 20, 2013: Revue Médicale Suisse
https://www.readbyqxmd.com/read/23102069/granulomatous-lymphocytic-interstitial-lung-disease
#11
REVIEW
Evans R Fernández Pérez
As the use of high-dose intravenous gamma globulin and available antimicrobials expands, the incidence of noninfectious common variable immunodeficiency-related disorders, such as granulomatous-lymphocytic interstitial lung disease (GLILD), is likely to increase. A thorough clinical evaluation using a multidisciplinary care model is recommended to achieve accurate diagnosis of GLILD. Enough progress has been made in the understanding of GLILD over the last decade that clinicians are now poised to conduct therapeutic trials...
November 2012: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/22930256/use-of-combination-chemotherapy-for-treatment-of-granulomatous-and-lymphocytic-interstitial-lung-disease-glild-in-patients-with-common-variable-immunodeficiency-cvid
#12
RANDOMIZED CONTROLLED TRIAL
Nicole M Chase, James W Verbsky, Mary K Hintermeyer, Jill K Waukau, Aoy Tomita-Mitchell, James T Casper, Sumit Singh, Kaushik S Shahir, William B Tisol, Melodee L Nugent, R Nagarjun Rao, A Craig Mackinnon, Lawrence R Goodman, Pippa M Simpson, John M Routes
PURPOSE: A subset of patients with common variable immunodeficiency (CVID) develops granulomatous and lymphocytic interstitial lung disease (GLILD), a restrictive lung disease associated with early mortality. The optimal therapy for GLILD is unknown. This study was undertaken to see if rituximab and azathioprine (combination chemotherapy) would improve pulmonary function and/or radiographic abnormalities in patients with CVID and GLILD. METHODS: A retrospective chart review of patients with CVID and GLILD who were treated with combination chemotherapy was performed...
January 2013: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/22372173/an-18-year-old-woman-with-kabuki-syndrome-immunoglobulin-deficiency-and-granulomatous-lymphocytic-interstitial-lung-disease
#13
Jose Angelo A De Dios, Adnan A Javaid, Enrique Ballesteros, Mark L Metersky
Granulomatous lymphocytic interstitial lung disease, or GLILD, is an uncommon condition associated with common variable immunodeficiency (CVID). We present an interesting case of an 18-year-old woman with Kabuki syndrome and CVID who was seen in our clinic for an abnormal chest CT scan. She was subsequently diagnosed with GLILD. There are no established guidelines for the treatment of GLILD in CVID. Immune globulin replacement therapy is the main treatment for CVID and higher doses of intravenous immunoglobulin (IVIG) may prevent the progression of chronic lung disease...
January 2012: Connecticut Medicine
https://www.readbyqxmd.com/read/19900842/granulomatous-lymphocytic-interstitial-lung-disease-glild-in-common-variable-immunodeficiency-cvid
#14
REVIEW
Joon H Park, Arnold I Levinson
Infectious complications of the lung occur quite frequently in patients with common variable immunodeficiency (CVID), a clinical syndrome that represents a primary immunodeficiency. However, there appears to be noninfectious pulmonary complications in association with CVID as well, and recently the term granulomatous-lymphocytic interstitial lung disease (GLILD) has been created to describe these noninfectious, diffuse lung disease complications that develop in CVID patients. They exhibit both granulomatous and lymphoproliferative histologic patterns, consisting of lymphocytic interstitial pneumonia (LIP), follicular bronchiolitis, and lymphoid hyperplasia...
February 2010: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/16103407/possible-role-of-human-herpesvirus-8-in-the-lymphoproliferative-disorders-in-common-variable-immunodeficiency
#15
William H Wheat, Carlyne D Cool, Yoshikazu Morimoto, Pradeep R Rai, Charles H Kirkpatrick, Barbara A Lindenbaum, Christopher A Bates, Misoo C Ellison, Amanda E Serls, Kevin K Brown, John M Routes
Patients who have common variable immunodeficiency (CVID) and granulomatous/lymphocytic interstitial lung disease (GLILD) are at high risk for early mortality and B cell lymphomas. Infection with human herpes virus type 8 (HHV8), a B cell lymphotrophic virus, is linked to lymphoproliferative disorders in people who have secondary immunodeficiencies. Therefore, we determined the prevalence of HHV8 infection in CVID patients with GLILD. Genomic DNA isolated from peripheral blood mononuclear cells was screened by nested- and real time-quantitative PCR (QRT-PCR) for the presence of HHV8 genome...
August 15, 2005: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/15316526/granulomatous-lymphocytic-lung-disease-shortens-survival-in-common-variable-immunodeficiency
#16
Christopher A Bates, Misoo C Ellison, David A Lynch, Carlyne D Cool, Kevin K Brown, John M Routes
BACKGROUND: Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by low levels of serum immunoglobulins and an inability to make specific antibodies. OBJECTIVE: We sought to determine the prevalence, clinical characteristics, and effect on survival of noninfectious pulmonary disease in patients with CVID. METHODS: A retrospective analysis of 69 patients with CVID was performed. Patients were divided into 3 groups on the basis of the type of pulmonary disease present: group 1 (n=29), no pulmonary disease; group 2 (n=23), chronic respiratory symptoms without diffuse radiographic abnormalities; and group 3 (n=18), chronic respiratory symptoms and diffuse radiographic abnormalities...
August 2004: Journal of Allergy and Clinical Immunology
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