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Angela Deyà-Martínez, Ana Esteve-Solé, Natalia Vélez-Tirado, Veronica Celis, Jordi Costa, Maria Cols, Cristina Jou, Alexandru Vlagea, Ana María Plaza-Martin, Manel Juan, Laia Alsina
BACKGROUND: One of the most frequent non-infectious complications of humoral immunodeficiencies with a CVID-like pattern is a particular form of inflammatory lung disease which is called granulomatous-lymphocytic interstitial lung disease (GLILD). Its development worsens patient prognosis, with a significant decrease in survival. Currently there are no unified guidelines regarding its management, and different combinations of immunosuppressants have been used with variable success. METHODS: Clinical and radiological data were collected from patient's medical charts...
March 12, 2018: Pediatric Allergy and Immunology
Amika K Sood, William Funkhouser, Brian Handly, Brent Weston, Eveline Y Wu
PURPOSE OF REVIEW: Granulomatous-lymphocytic interstitial lung disease (GLILD) has classically been associated with common variable immune deficiency (CVID), but is increasingly being reported in other immunodeficiencies. We describe the second reported case of GLILD in a patient with 22q11.2 deletion syndrome (22q11.2DS) and review the recent literature surrounding GLILD. RECENT FINDINGS: GLILD is characterized by granulomata and lymphoproliferation. Consensus statements and retrospective and case-control studies have better elucidated the clinicopathological and radiographic manifestations of GLILD, allowing for its differentiation from similar conditions like sarcoidosis...
February 22, 2018: Current Allergy and Asthma Reports
Giorgia Bucciol, Angelamaria Petrone, Maria Caterina Putti
The autoimmune manifestations of primary immunodeficiencies, such as autoimmune lymphoproliferative syndrome (ALPS) and common variable immunodeficiency (CVID), often constitute a great therapeutic challenge and have a significant impact on patients' morbidity and mortality. The most common autoimmune presentations are autoimmune cytopenias, but organ-related autoimmunity is also frequently observed. From a pulmonology perspective, granulomatous/lymphocytic interstitial lung disease (GLILD) is a severe immunological complication which significantly worsens the clinical outcome of these patients and for which there are currently few guidelines or protocols for treatment...
October 2017: Pediatric Pulmonology
John R Hurst, Nisha Verma, David Lowe, Helen E Baxendale, Stephen Jolles, Peter Kelleher, Hilary J Longhurst, Smita Y Patel, Elisabetta A Renzoni, Clare R Sander, Gerard R Avery, Judith L Babar, Matthew S Buckland, Siobhan Burns, William Egner, Mark M Gompels, Pavels Gordins, Jamanda A Haddock, Simon P Hart, Grant R Hayman, Richard Herriot, Rachel K Hoyles, Aarnoud P Huissoon, Joseph Jacob, Andrew G Nicholson, Doris M Rassl, Ravishankar B Sargur, Sinisa Savic, Suranjith L Seneviratne, Michael Sheaff, Prashantha M Vaitla, Gareth I Walters, Joanna L Whitehouse, Penny A Wright, Alison M Condliffe
A proportion of people living with common variable immunodeficiency disorders develop granulomatous-lymphocytic interstitial lung disease (GLILD). We aimed to develop a consensus statement on the definition, diagnosis, and management of GLILD. All UK specialist centers were contacted and relevant physicians were invited to take part in a 3-round online Delphi process. Responses were graded as Strongly Agree, Tend to Agree, Neither Agree nor Disagree, Tend to Disagree, and Strongly Disagree, scored +1, +0...
July 2017: Journal of Allergy and Clinical Immunology in Practice
Stella Hartono, Megan S Motosue, Shakila Khan, Vilmarie Rodriguez, Vivek N Iyer, Rohit Divekar, Avni Y Joshi
BACKGROUND: A subset of patients with common variable immunodeficiency (CVID) develop granulomatous lymphocytic interstitial lung disease (GLILD), which is associated with early mortality. OBJECTIVE: To determine a set of clinical and/or laboratory parameters that correlate with GLILD. METHODS: A retrospective, nested case-control (patients with CVID diagnosed with GLILD compared with patients with CVID without a diagnosis of GLILD) medical record review was undertaken at Mayo Clinic, Rochester, MN...
May 2017: Annals of Allergy, Asthma & Immunology
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...
January 2017: Clinical and Experimental Immunology
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
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...
June 22, 2016: BMJ Case Reports
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...
January 2018: Clinical Respiratory Journal
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
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
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
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
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
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
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
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
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
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
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