Lymphocytic Interstitial Pneumonia Research Articles

A Historic Cohort Analysis of Radiographic and Serologic Findings in Patients With Scleroderma and Interstitial Lung Disease.

Few studies have investigated associations between rheumatologic serology patterns and different interstitial lung disease (ILD) patterns. We present novel findings of a historic cohort study (n = 454) with data collected from 2011 to 2021 within our hospital system. In this institutional review board-approved study, data regarding rheumatologic serologies and ILD patterns were noted based on chart review in patients with scleroderma. The Kruskal-Wallis rank sum and χ2 tests were used for statistical analysis. Results showed a statistically significant association between anti-U1 snRNP with lymphoid interstitial pneumonia, which has not been previously described. We demonstrated novel serologic associations with ILD patterns, which have important clinical implications. More robust and high-powered studies are needed to elucidate the role of serologic testing and their association with ILD phenotypes.

Clinicopathological and ultrastructural study of African swine fever outbreak in North-East India

The present investigation focuses on examining the clinical, histopathological, and ultrastructural changes that occurred in pig, during an outbreak of African swine fever (ASF) in 2022 in Assam, India. The disease initially manifested as a per-acute case with high mortality but without any evident clinical signs. Subsequently, some animals exhibited an acute form of the disease characterized by high fever (104–106 °F), anorexia, vomiting, respiratory distress, and bleeding from the anal and nasal orifices. During acute African swine fever virus (ASFV) infections, elevated levels of pro-inflammatory IL-1α, IL-1β, IL-6, TNF, CCL2, CCL5, and CXCL10 were detected in the palatine tonsil, lymph nodes, spleen, and kidney using qPCR assay. These molecular changes were associated with haemorrhages, edemas, and lymphoid depletion. Postmortem examinations revealed prominent features such as splenomegaly with haemorrhages, haemorrhagic lymphadenitis, severe petechial haemorrhage in the kidney, pneumonia in the lungs, and necrotic palatine tonsil. Histopathological analysis demonstrated lymphocyte depletion in lymphoid organs, multi-organ haemorrhages, and interstitial pneumonia in the lungs. Scanning electron microscopy (SEM) further confirmed lymphocyte depletion in lymphoid organs through lymphocyte apoptosis and kidney damage with distorted tubules due to red blood cell destruction. Transmission electron microscopy reaffirmed lymphocyte apoptosis by observing chromatin condensation and nucleus margination in lymphocytes of lymphoid organs. These findings provide comprehensive insights into the clinical, histopathological, and ultrastructural aspects of ASF outbreak in pigs. Understanding the pathological changes associated with ASF can contribute to improved diagnosis, prevention, and control measures for this highly contagious and economically devastating viral disease.

Connective tissue disease related interstitial lung disease: a single center experience

Objectives: We aimed to determine the clinical features of the patients followed by our hospital with the diagnosis of connective tissue disease related interstitial lung disease (CTD-ILD). Methods: The study included 113 patients who were followed up with the diagnosis of CTD-ILD, admitted to Kahramanmaraş Sütçü İmam University, Faculty of Medicine, Rheumatology Department between January 2019 and December 2020. Demographic characteristics, laboratory data and high-resolution computed tomography (HRCT) patterns of the patients were recorded retrospectively. Results: Of 113 patients diagnosed with CTD-ILD; 90 were female, 23 were male. When the distribution of connective tissue disease (CTD) evaluated; 50 were rheumatoid arthritis, 41 were systemic sclerosis, 8 were sjogren's syndrome, 4 were systemic lupus erythematosus, 7 were overlap syndrome and 3 were undifferentiated connective tissue disease. There was no statistically significant difference between laboratory parameters. In the HRCT evaluation, 86 patients had nonspecific interstitial pneumonia (NSIP), 25 patients had usual interstitial pneumonia (UIP), and 2 patients had lymphocytic interstitial pneumonia (LIP) pattern. The most common pattern, including rheumatoid arthritis, was NSIP. Conclusions: In our study, as inconsistent with the literature the most common pattern in RA patients was found to be NSIP.

Lymphoid Interstitial Pneumonia Associated With Hashimoto Thyroid Disease

Lymphoid Interstitial Pneumonia Associated With Hashimoto Thyroid Disease

Imaging Features of Idiopathic Interstitial Lung Diseases.

Idiopathic interstitial pneumonias (IIPs) are a group of diffuse parenchymal lung diseases of unclear etiology and are distinguished from diffuse parenchymal lung diseases of known cause, such as connective tissue disease-related interstitial lung diseases or hypersensitivity pneumonitis by history, physical exam, imaging, serologic testing, and, when necessary, histopathology. The 2013 American Thoracic Society (ATS)/European Respiratory Society (ERS) guidelines are the most widely accepted classification of IIPs and include the following diagnoses: idiopathic pulmonary fibrosis, idiopathic nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, idiopathic lymphocytic interstitial pneumonia, idiopathic pleuro-parenchymal fibroelastosis, respiratory bronchiolitis-interstitial lung disease, and desquamative interstitial pneumonia. The gold standard for diagnosis of IIP involves multidisciplinary discussion among pulmonologists, radiologists, and pathologists. The focus of this review will be to discuss the imaging features of the most common IIPs and the role of multidisciplinary discussion as the gold standard for diagnosis.

NKX2.1 mutation revealed by a lymphoid interstitial pneumonia in an adult with rheumatoid arthritis.

This is the first case of a 37-year-old female patient carrier of a heterozygous NKX2.1 mutation associated with RA-ILD with a histological pattern of LIP. This case illustrates the wide panel of ILD subtypes associated with NKX2.1 mutations. https://bit.ly/3F49OTS.

77 - Lymphoid interstitial pneumonia in a patient with untreated rheumatoid arthritis

77 - Lymphoid interstitial pneumonia in a patient with untreated rheumatoid arthritis

Sjogren’s Syndrome with Interstitial Lung Disease

Sjogren’s syndrome (SS) is an autoimmune disease affecting exocrine glands with known properties to cause chronic systemic multi-organ disease involvement. It produced the typical features of sicca syndrome, but due to insidious onset of the disease, patient may present with complications of the condition at the initial presentation. We present a case of primary Sjogren’s syndrome (SS) who first presented with pulmonary manifestations and was subsequently diagnosed as Lymphocytic Interstitial Pneumonia (LIP). The patient was extensively investigated and received appropriate treatment modalities.

Multiple pulmonary masses with diffuse cystic lesions

A 50-year-old female patient, presenting with a past history of Reynaud's syndrome, xerostomia and xerophthalmia, was admitted to Fujian Provincial Hospital because of coughing for 10 days and left anterior chest pain for 1 day. Chest imaging showed multiple nodules and masses, and diffuse cystic lesions in both lungs. Based on the differential diagnosis of multiple pulmonary masses and diffuse cystic lesions respectively, autoantibodies, radionuclide dynamic imaging of the parotid, positron emission tomography-CT, CT-guided percutaneous transthoracic needle biopsy, and other examinations were performed. The diagnosis of diffuse large B-cell lymphoma stage ⅣA (lung, parotid gland) and Sjögren's syndrome was confirmed. By analyzing the imaging features and pathogenesis in detail, the diffuse cystic lesions of both lungs were considered to be related to lymphocytic interstitial pneumonia caused by Sjögren's syndrome. The pulmonary space-occupying lesions in the lungs were significantly absorbed after RCHOP regimen for lymphoma.

Lymphoid Interstitial Pneumonia (LIP) Revisited: A Critical Reappraisal of the Histologic Spectrum of "Radiologic" and "Pathologic" LIP in the Context of Diffuse Benign Lymphoid Proliferations of the Lung.

The use of lymphoid interstitial pneumonia (LIP) as a diagnostic term has changed considerably since its introduction. Utilizing a multi-institutional collection of 201 cases from the last 20 years that demonstrate features associated with the LIP rubric, we compared cases meeting strict histologic criteria of LIP per American Thoracic Society (ATS)/European Respiratory Society (ERS) consensus ("pathologic LIP"; n=62) with cystic cases fulfilling radiologic ATS/ERS criteria ("radiologic LIP"; n=33) and with other diffuse benign lymphoid proliferations. "Pathologic LIP" was associated with immune dysregulation including autoimmune disorders and immune deficiency, whereas "radiologic LIP" was only seen with autoimmune disorders. No case of idiopathic LIP was found. On histology, "pathologic LIP" represented a subgroup of 70% (62/88) of cases with the distinctive pattern of diffuse expansile lymphoid infiltrates. In contrast, "radiologic LIP" demonstrated a broad spectrum of inflammatory patterns, airway-centered inflammation being most common (52%; 17/33). Only 5 cases with radiologic cysts also met consensus ATS/ERS criteria for "pathologic LIP." Overall, broad overlap was observed with the remaining study cases that failed to meet consensus criteria for "radiologic LIP" and/or "pathologic LIP." These data raise concerns about the practical use of the term LIP as currently defined. What radiologists and pathologist encounter as LIP differs remarkably, but neither "radiologic LIP" nor "pathologic LIP" present with sufficiently distinct findings to delineate such cases from other patterns of diffuse benign lymphoid proliferations. As a result of this study, we believe LIP should be abandoned as a pathologic and radiologic diagnosis.

A rare cause of spontaneous pneumothorax: radiological findings and surgical approach

Birt-Hogg-Dubé Syndrome (BHDS) is a rare genetic condition that affects the connective tissue of kidneys, lungs, and skin, causing an aberration in the proteic folliculin (FLCN) pathway. In the lungs, the altered FLCN is found in the outer part of the alveoli, particularly in the lower lobes, causing parenchymal weakness and subsequent cystic degeneration. Because of its rarity, a comprehensive management protocol is not available yet. We present a case of a man with left recurrent pneumothorax due to bilateral multiple bullae, as revealed by a preoperative chest Computed Tomography (CT) scan. Given the characteristic radiological features of BHDS, the CT scan should always be considered for the differential diagnosis to exclude other more common cystic diseases, such as lymphoid interstitial pneumonia, Langerhans cell histiocytosis, or lymphangioleiomyomatosis. Considering the different options suitable for bullous diseases, we decided to treat the patient with a pleural abrasion through a biportal Video-Assisted Thoracic Surgery approach and sterile talc pleurodesis. No more spontaneous episodes were reported one year later. Bullectomy was not carried out because the diffuse cystic intraoperative pattern would never be solved by marginal parenchymal resections. Relapsing spontaneous pneumothorax could be a spy of a hidden genetic disease (such as BHSD), and a CT scan should be considered case-by-case because it may provide crucial information regarding the overall lung pattern. In such cases, pleural abrasion with talc pleurodesis is a considerably effective treatment.

Spectrum of high-resolution computed tomography pattern in lungs in patients with connective tissue disorders.

Connective tissue disease associated with interstitial lung disease, or CT-ILD, is a lung condition that affects a large number of patients with a connective tissue disease. Our aim in this study is to correlation between images of high-resolution computed tomography (HRCT) of different connective tissue diseases associated interstitial lung diseases (CTD-ILDs). We shall be aiming to investigate the feasibility of HRCT imaging and thereby avoid lung biopsy in such patients. Rheumatoid arthritis predominantly presented with usual interstitial pneumonia (UIP) (47.8%), followed by nonspecific interstitial pneumonia (NSIP) (30.4%). Mixed connective tissue disorder predominantly presented with NSIP and UIP (42.8%), followed by organizing pneumonia (OP) (14.2%). Systemic lupus erythematosus predominantly presented with UIP (38.8%), followed by NSIP (27.7%). Sjogren's syndrome predominantly presented with lymphocytic interstitial pneumonia (40%), followed by UIP (26.6%). Scleroderma predominantly presented with UIP (45.4%), followed by NSIP (36.4%). Sarcoidosis predominantly presented with UIP (75%), followed by NSIP (25%). Dermatomyositis predominantly presented with NSIP (50%), followed by UIP and OP each (25%). Both clinicians and radiologists should be aware of the expected evolution of HRCT changes in a variety of CT-ILDs.

Lymphocytic Interstitial Pneumonia in a Man with Human Immunodeficiency Virus Infection.

Lymphocytic Interstitial Pneumonia in a Man with Human Immunodeficiency Virus Infection.

Evolving Concepts and Controversies in Interstitial Lung diseases: Update in 2023

Recent studies elicited evolving concepts and controversies in interstitial lung diseases and proposed substantial changes in diagnostic and therapeutic approaches to interstitial lung diseases. Dr. Averill A. Liebow first coined the term usual interstitial pneumonia (UIP) in 1960’s as distinct pathologic pattern of fibrosis in idiopathic pulmonary fibrosis (IPF), the prototype of progressive fibrosing interstitial lung disease with poor prognosis. Advances in omics led to a better understanding of molecular pathogenesis of UIP and shed light on various types of familial pulmonary fibrosis as well as familial IPF. The concept of progressive pulmonary fibrosis was introduced to acknowledge additional types of progressive fibrosing interstitial lung diseases with the clinical and pathologic phenotypes very similar to those of UIP/IPF. As such, some authors have proposed a paradigm shift by considering UIP as a stand-alone diagnostic entity to encompass other fibrosing interstitial lung diseases that undergo the same relentless progression as IPF. Cicatricial organizing pneumonia is a variant of organizing pneumonia that can be reminiscent of UIP on histopathology but usually follows a stable clinical course unlike UIP. There has been significant disconnection in fundamental understanding as well as diagnostic criteria of lymphocytic interstitial pneumonia among pathologists, pulmonologists and radiologists, which needs to be resolved. The concept and histopathologic criteria of granulomatous and lymphocytic interstitial lung disease are also elusive and require clarification as well. In this review, these topics will be covered based on current literature.

The role of computed tomography in a diagnostic approach to cystic lung diseases and their differential diagnosis

In everyday routine, lung cysts are commonly seen in Computed Tomography, as far as manydifferentconditionsanddiseasesareassociatedwithaircysts.Thus,correctdiag-many different conditions and diseases are associated with air cysts. Thus, correct diag-nosisofcysticlungdiseases,whichshowawidespectrum,isachallengeforradiologists.nosis of cystic lung diseases, which show a wide spectrum, is a challenge for radiologists. Fordiagnosisanddifferentialdiagnosis,firstofall,cystsshouldbedistinguishedfromFor diagnosis and differential diagnosis, fi rst of all, cysts should be distinguished from suchotherairfilledlesions,likecavities,bullae,pneumatocele,emphysema,honeycomb-such other air fi lled lesions, like cavities, bullae, pneumatocele, emphysema, honeycomb-ingandcysticbronchiectasis.Second,cystscanbecategorizedassingle/localizedversusing and cystic bronchiectasis. Second, cysts can be categorized as single/localized versus multiple/diffuse.Solitary/localizedcystsincludeincidentalcysts,congenitalcysticdis-multiple/diffuse. Solitary/localized cysts include incidental cysts, congenital cystic dis-easesandcysticcancers.Multiple/diffusecystscanbefurthercategorizedaccordingtoeases and cystic cancers. Multiple/diffuse cysts can be further categorized according to thepresenceorabsenceofassociatedradiologicfindings.Multiple/diffusecystswithoutthe presence or absence of associated radiologic fi ndings. Multiple/diffuse cysts without associatedfindingsincludelymphangioleiomyomatosisandBirt-Hogg-Dubésyndrome.associated fi ndings include lymphangioleiomyomatosis and Birt-Hogg-Dubé syndrome. Multiple/diffusecystsmaybeassociatedwithground-glassopacityorsmallnodules.Multiple/diffuse cysts may be associated with ground-glass opacity or small nodules. Multiple/diffusecystswithnodulesincludeLangerhanscellhistiocytosis,cysticmetas-Multiple/diffuse cysts with nodules include Langerhans cell histiocytosis, cystic metas-tasisandamyloidosis.Multiple/diffusecystswithground-glassopacityincludepneumo-tasis and amyloidosis. Multiple/diffuse cysts with ground-glass opacity include pneumo-cystispneumonia,desquamativeinterstitialpneumoniaandlymphocyticinterstitialpneu-cystis pneumonia, desquamative interstitial pneumonia and lymphocytic interstitial pneu-monia.Thestepwiseradiologicdiagnosticapproachcanbehelpfulinreachingacorrectmonia. The stepwise radiologic diagnostic approach can be helpful in reaching a correct diagnosisforvariouscysticlungdiseases.diagnosis for various cystic lung diseases.

Cystic lung disease

The term cystic lung disease encompasses a heterogeneous group of entities characterised by round lung lesions that correspond to cysts with fine walls, which usually contain air. The differential diagnosis of these lesions can be challenging, requiring both clinical and radiological perspectives. Entities such as pulmonary emphysema and cystic bronchiectasis can simulate cystic disease.High-resolution computed tomography (HRCT) is the imaging technique of choice for the evaluation and diagnosis of cystic lung disease, because it confirms the presence of lung disease and establishes the correct diagnosis of the associated complications. In many cases, the diagnosis can be established based on the HRCT findings, thus making histologic confirmation unnecessary. For these reasons, radiologists need to be familiar with the different presentations of these entities.A wide variety of diseases are characterised by the presence of diffuse pulmonary cysts. Among these, the most common are lymphangioleiomyomatosis, which may or may not be associated with tuberous sclerosis, Langerhans cell histiocytosis, and lymphocytic interstitial pneumonia. Other, less common entities include Birt-Hogg-Dubé syndrome, amyloidosis, and light-chain deposit disease.This article describes the characteristics and presentations of some of these entities, emphasizing the details that can help differentiate among them.

DIAGNOSIS AND MANAGEMENT OF CTLA-4 VARIANT IMMUNE DYSREGULATION

<h3>Introduction</h3> Cytotoxic T-lymphocyte antigen 4 (CTLA-4) is a negative regulator of the immune system and when deficient, can result in immune dysregulation. Here we present a patient with progressive immune dysregulation and confirmed CTLA-4 haploinsufficiency. <h3>Case Description</h3> A 25-year-old male diagnosed with Evans syndrome and CNS vasculitis in childhood was stabilized on mycophenolate mofetil (MMF). At age 14, he developed pneumonia and had a lung biopsy consistent with lymphocytic interstitial pneumonia. Immune evaluation confirmed common variable immunodeficiency with dysregulated T and B cell subsets, and he was started on immunoglobulin replacement therapy. Genetic evaluation revealed 2 CTLA-4 variants of uncertain significance and functional assays confirmed CTLA-4 haploinsufficiency. Subsequently, he developed splenomegaly, chronic lung disease, and EBV viremia. A repeat lung biopsy was consistent with polymorphic EBV-positive lymphoproliferative disorder, prompting rituximab therapy. CTLA-4 Ig could not be utilized because of the potential of worsening chronic EBV. He was prescribed sirolimus. Acyclovir and trimethoprim-sulfamethoxazole were prescribed prophylactically for T cell lymphopenia. <h3>Discussion</h3> This patient with EBV and CTLA-4-driven lung disease is susceptible to worsening lung and lymphoproliferative diseases and recurrent infections. Management options include rituximab for EBV, sirolimus for immune dysregulation, and when possible, CTLA-4 Ig once EBV is controlled. The manifestations of CTLA-4 haploinsufficiency are unique and patients require close clinical follow up. Additional biomarkers of dysregulation may include monitoring of dysregulated T and B cell subsets. Management aims include targeted therapy to control immune dysregulation and potentially definitive hematopoietic stem cell transplantation.

Enfermedades quísticas pulmonares

The term cystic lung disease encompasses a heterogeneous group of entities characterized by round lung lesions that correspond to cysts with fine walls, which usually contain air. The differential diagnosis of these lesions can be challenging, requiring both clinical and radiological perspectives. Entities such as pulmonary emphysema and cystic bronchiectasis can simulate cystic disease.High-resolution computed tomography (HRCT) is the imaging technique of choice for the evaluation and diagnosis of cystic lung disease, because it confirms the presence of lung disease and establishes the correct diagnosis of the associated complications. In many cases, the diagnosis can be established based on the HRCT findings, thus making histologic confirmation unnecessary. For these reasons, radiologists need to be familiar with the different presentations of these entities.A wide variety of diseases are characterized by the presence of diffuse pulmonary cysts. Among these, the most common are lymphangioleiomyomatosis, which may or may not be associated with tuberous sclerosis, Langerhans cell histiocytosis, and lymphocytic interstitial pneumonia. Other, less common entities include Birt-Hogg-Dubé syndrome, amyloidosis, and light-chain deposit disease.This article describes the characteristics and presentations of some of these entities, emphasizing the details that can help differentiate among them.

Severe extra-glandular involvement and pleural effusions complicating primary Sjogren’s syndrome: a case report

BackgroundSjogren’s syndrome, an autoimmune disease of the exocrine glands, results in keratoconjunctivitis sicca, xerostomia, and dental caries. It is often overlooked, considered by clinicians to be a benign disease. However, it can cause life-threatening extra-glandular complications that affect multiple organ systems.Case presentationHere we present a 78-year-old Caucasian woman with a history of primary Sjogren’s syndrome (pSS) whose symptoms of keratoconjunctivitis sicca were managed managed conservatively. She was evaluated for sub-acute shortness of breath. Imaging showed severe bronchiectasis with features of lymphocytic interstitial pneumonia. She also had exudative bilateral pleural effusions and skin ulcers, likely vasculitic in origin. The workup was significant for anti-Ro antibody, pancytopenia, hypocomplementia, cryoglobulinemia and monoclonal gammopathy, all of which reflect disease severity. Although there was no evidence of malignancy, she developed B-cell non-Hodgkin lymphoma during follow-up.ConclusionsPrimary Sjogren’s syndrome can result in severe multi-organ disease. Pleural effusions are a rare complication of pSS, with only ten cases reported in the literature over the last 30 years, and tend to respond well to steroids. Prognostic biomarkers for disease severity include hypocomplementia, cryoglobulinemia, monoclonal gammopathy, and hypergammaglobulinemia. In this report we review the literature and the management of the disease.

High-Resolution Computed Tomography of Cystic Lung Disease.

The cystic lung diseases (CLD) are characterized by the presence of multiple, thin-walled, air-filled spaces in the pulmonary parenchyma. Cyst formation may occur with congenital, autoimmune, inflammatory, infectious, or neoplastic processes. Recognition of cyst mimics such as emphysema and bronchiectasis is important to prevent diagnostic confusion and unnecessary evaluation. Chest CT can be diagnostic or may guide the workup based on cyst number, distribution, morphology, and associated lung, and extrapulmonary findings. Diffuse CLD (DCLDs) are often considered those presenting with 10 or more cysts. The more commonly encountered DCLDs include lymphangioleiomyomatosis, pulmonary Langerhans' cell histiocytosis, lymphoid interstitial pneumonia, Birt-Hogg-Dubé syndrome, and amyloidosis/light chain deposition disease.