Lymphoproliferative Lung Disorders: A Radiologic-Pathologic Overview. Part I: Reactive disorders

Carrillo, Jorge; Restrepo, Carlos S.; Christenson, Melissa Rosado de; León, Paulina Ojeda; Rivera, Adelaida María Gaviria; Koss, M. N.

doi:10.1053/j.sult.2013.05.002

Cited by 37 publications

(27 citation statements)

References 50 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Lymphoproliferative disorders, which can arise in the setting of certain CTDs such as SjS, can mimic cellular NSIP and LIP histologically, radiologically, and clinically, and need to be ruled out when the inflammatory infiltrate is unusually florid. 22,55,[69][70][71][72] Of course, most of the histologic patterns, particularly UIP, have idiopathic forms that occur in the absence of CTD. On the other hand, it is also the case that any of these patterns can be the initial manifestation of a patient's yet to be clinically diagnosed CTD.…”

Section: Histologic Differential Diagnosis Of Lung Disease In Connectmentioning

confidence: 99%

Histopathology of Lung Disease in the Connective Tissue Diseases

Vivero¹,

Padera²

2015

Rheumatic Disease Clinics of North America

View full text Add to dashboard Cite

Section: Histologic Differential Diagnosis Of Lung Disease In Connectmentioning

confidence: 99%

Histopathology of Lung Disease in the Connective Tissue Diseases

Vivero¹,

Padera²

2015

Rheumatic Disease Clinics of North America

View full text Add to dashboard Cite

“…6, 12, 13, 17, 18 Along these lines, biopsies of CVID ILD demonstrate benign lymphoproliferative pathology (such as follicular bronchiolitis, lymphocytic interstitial pneumonitis, and nodular lymphoid hyperplasia) in nearly all cases. 12, 19, 20 Intrinsic immune dysregulation, perhaps in the absence of infection, can result in ILD that is pathologically similar to that seen CVID. This is demonstrated by the characteristic lymphoproliferative ILD seen in patients with monogenic immune dysregulation disorders such as cytotoxic T lymphocyte-associated protein-4 (CTLA-4) deficiency and signal transducer and activator of transcription 3 (STAT3) gain-of-function mutations as well as autoimmune diseases like Sjogren’s Syndrome.…”

Section: Introductionmentioning

confidence: 99%

Progression of Common Variable Immunodeficiency Interstitial Lung Disease Accompanies Distinct Pulmonary and Laboratory Findings

Maglione

Overbey

Cunningham‐Rundles

2015

The Journal of Allergy and Clinical Immunology: In Practice

View full text Add to dashboard Cite

Background Common variable immunodeficiency may be complicated by interstitial lung disease, leading to worsened morbidity and mortality in some. While immunomodulatory treatment has efficacy, choice of patient, duration of treatment, and long-term follow-up are not available. Interstitial lung disease appears stable in certain instances, so it is not known whether all patients will develop progressive disease or require immunomodulatory therapy. Objective This study aims to determine if all common variable immunodeficiency patients with interstitial lung disease have physiological worsening, and if clinical and/or laboratory parameters may correlate with disease progression. Methods Retrospective review of medical records at Mount Sinai Medical Center in New York was conducted for referred patients with common variable immunodeficiency, CT scan-confirmed interstitial lung disease, and periodic pulmonary function testing covering 20 or more months prior to immunomodulatory therapy. Fifteen patients were identified from the retrospective review and included in this study. Results Nine of the 15 common variable immunodeficiency patients had physiological worsening of interstitial lung disease adapted from consensus guidelines, associated with significant reductions in FEV1, FVC, and DLCO. Those with progressive lung disease also had significantly lower mean IgG levels, greater increases and highest levels of serum IgM, and more significant thrombocytopenia. Conclusion Interstitial lung disease resulted in physiological worsening in many, but not all subjects, and was associated with suboptimal IgG replacement. Those with worsening pulmonary function tests, elevated IgM, and severe thrombocytopenic episodes appear to be at highest risk for progressive disease. Such patients may benefit from immunomodulatory treatment.

show abstract

“…77, 89, 117 Granulomatous inflammation is also frequently seen in CVID patients in lungs as well as other organs. 118 Organizing pneumonia is also found in PAD patients and is non-specific reactive inflammation that results from diverse causes of lung injury and is characterized by plugs of granulation tissue and whorls of fibroblasts, known as Masson bodies, in the alveolar spaces.…”

Section: Interstitial Lung Diseasementioning

confidence: 99%

“…Nodular lymphoid hyperplasia is also seen in PAD, particularly in patients with CVID or PI3Kδ syndrome, consisting of interstitial inflammation with more well-demarcated follicles than LIP and may be a precursor of mucosal-associated lymphoid tissue lymphomas but it's exact relationship with malignancy is unclear (Figure 3D). 117, 122 The ectopic B cell follicles that characterize these benign lymphoproliferative ILD develop and express markers of germinal centers and proliferation despite the B cell maturation defects inherent to PAD (Figure 4). 107 This observation is notable because CVID is characterized by poor T cell-dependent isotype-switched antibody responses to vaccination and impaired development of memory B cells, both of which classically utilize germinal center reactions.…”

Section: Interstitial Lung Diseasementioning

confidence: 99%

Lung Disease in Primary Antibody Deficiencies

Schussler

Beasley

Maglione

2016

The Journal of Allergy and Clinical Immunology: In Practice

View full text Add to dashboard Cite

Primary antibody deficiencies (PADs) are the most common form of primary immunodeficiency and predispose to severe and recurrent pulmonary infections which can result in chronic lung disease including bronchiectasis. Chronic lung disease is among the most common complications of PAD and a significant source of morbidity and mortality for these patients. However, the development of lung disease in PAD may not be solely the result of recurrent bacterial infection or a consequence of bronchiectasis. Recent characterization of monogenic immune dysregulation disorders and more extensive study of common variable immunodeficiency has demonstrated that interstitial lung disease (ILD) in PAD can result from generalized immune dysregulation and frequently occurs in the absence of pneumonia history or bronchiectasis. This distinction between bronchiectasis and ILD has important consequences in the evaluation and management of lung disease in PAD. For example, treatment of ILD in PAD typically utilizes immunomodulatory approaches in addition to immunoglobulin replacement and antibiotic prophylaxis which are the stalwarts of bronchiectasis management in these patients. Additionally, while all antibody deficient patients are at risk of developing bronchiectasis, ILD occurs in some forms of PAD much more commonly than others, suggesting that distinct but poorly understood immunological factors underlie development of this complication. Importantly, ILD can have earlier onset and may worsen survival more than bronchiectasis. Further efforts to understand the pathogenesis of lung disease in PAD will provide vital information for the most effective methods of diagnosis, surveillance, and treatment of these patients.

show abstract

Lymphoproliferative Lung Disorders: A Radiologic-Pathologic Overview. Part I: Reactive disorders

Cited by 37 publications

References 50 publications

Histopathology of Lung Disease in the Connective Tissue Diseases

Histopathology of Lung Disease in the Connective Tissue Diseases

Progression of Common Variable Immunodeficiency Interstitial Lung Disease Accompanies Distinct Pulmonary and Laboratory Findings

Lung Disease in Primary Antibody Deficiencies

Contact Info

Product

Resources

About