scholarly journals Bowel Histology of CVID Patients Reveals Distinct Patterns of Mucosal Inflammation

2021 ◽  
Author(s):  
Cornelia M. van Schewick ◽  
David M. Lowe ◽  
Siobhan O. Burns ◽  
Sarita Workman ◽  
Andrew Symes ◽  
...  
Keyword(s):  
Giardia Lamblia ◽  
Gastrointestinal Symptom ◽  
Plasma Cells ◽  
Lymphoid Hyperplasia ◽  
Mucosal Inflammation ◽  
Histological Features ◽  
Abdominal Symptoms ◽  
Immune Mediated ◽  
Lymphocyte Phenotyping ◽  
Common Variable

AbstractDiarrhea is the commonest gastrointestinal symptom in patients with common variable immunodeficiency (CVID). Different pathologies in patients’ bowel biopsies have been described and links with infections have been demonstrated. The aim of this study was to analyze the bowel histology of CVID patients in the Royal-Free-Hospital (RFH) London CVID cohort. Ninety-five bowel histology samples from 44 adult CVID patients were reviewed and grouped by histological patterns. Reasons for endoscopy and possible causative infections were recorded. Lymphocyte phenotyping results were compared between patients with different histological features. There was no distinctive feature that occurred in most diarrhea patients. Out of 44 patients (95 biopsies), 38 lacked plasma cells. In 14 of 21 patients with nodular lymphoid hyperplasia (NLH), this was the only visible pathology. In two patients, an infection with Giardia lamblia was associated with NLH. An IBD-like picture was seen in two patients. A coeliac-like picture was found in six patients, four of these had norovirus. NLH as well as inflammation often occurred as single features. There was no difference in blood lymphocyte phenotyping results comparing groups of histological features. We suggest that bowel histology in CVID patients with abdominal symptoms falls into three major histological patterns: (i) a coeliac-like histology, (ii) IBD-like changes, and (iii) NLH. Most patients, but remarkably not all, lacked plasma cells. CVID patients with diarrhea may have an altered bowel histology due to poorly understood and likely diverse immune-mediated mechanisms, occasionally driven by infections.

scholarly journals Gut Microbiota and Acute Diverticulitis: Role of Probiotics in Management of This Delicate Pathophysiological Balance

2021 ◽  
Vol 11 (4) ◽  
pp. 298
Author(s):  
Andrea Piccioni ◽  
Laura Franza ◽  
Mattia Brigida ◽  
Christian Zanza ◽  
Enrico Torelli ◽  
...  
Keyword(s):  
Gut Microbiota ◽  
Diverticular Disease ◽  
Acute Diverticulitis ◽  
Bacterial Species ◽  
Mucosal Inflammation ◽  
Abdominal Symptoms ◽  
Pubmed Database ◽  
Anti Inflammatory ◽  
Key Terms

How can the knowledge of probiotics and their mechanisms of action be translated into clinical practice when treating patients with diverticular disease and acute diverticulitis? Changes in microbiota composition have been observed in patients who were developing acute diverticulitis, with a reduction of taxa with anti-inflammatory activity, such as Clostridium cluster IV, Lactobacilli and Bacteroides. Recent observations supported that a dysbiosis characterised by decreased presence of anti-inflammatory bacterial species might be linked to mucosal inflammation, and a vicious cycle results from a mucosal inflammation driving dysbiosis at the same time. An alteration in gut microbiota can lead to an altered activation of nerve fibres, and subsequent neuronal and muscular dysfunction, thus favoring abdominal symptoms’ development. The possible role of dysbiosis and mucosal inflammation in leading to dysmotility is linked, in turn, to bacterial translocation from the lumen of the diverticulum to perivisceral area. There, a possible activation of Toll-like receptors has been described, with a subsequent inflammatory reaction at the level of the perivisceral tissues. Being aware that bacterial colonisation of diverticula is involved in the pathogenesis of acute diverticulitis, the rationale for the potential role of probiotics in the treatment of this disease becomes clearer. For this review, articles were identified using the electronic PubMed database through a comprehensive search conducted by combining key terms such as “gut microbiota”, “probiotics and gut disease”, “probiotics and acute diverticulitis”, “probiotics and diverticular disease”, “probiotics mechanism of action”. However, the amount of data present on this matter is not sufficient to draw robust conclusions on the efficacy of probiotics for symptoms’ management in diverticular disease.

scholarly journals The Histopathology of Maedi

1971 ◽  
Vol 8 (1) ◽  
pp. 63-80 ◽  
Author(s):  
G. Georgsson ◽  
P. A. Pálsson
Keyword(s):  
Lymphoid Hyperplasia ◽  
Viral Pneumonia ◽  
Pulmonary Diseases ◽  
Cellular Infiltration ◽  
Epithelial Proliferation ◽  
Histological Features ◽  
Interstitial Inflammation ◽  
Smooth Musculature ◽  
The World ◽  
Alveolar Septa

The histopathology of natural and experimental maedi, a slow-viral pneumonia of sheep, was studied. The main histological features are chronic interstitial inflammation with dense cellular infiltration, hyperplasia of smooth musculature in alveolar septa, and slight fibrosis; peribronchial and perivascular lymphoid hyperplasia, and epithelial proliferation in small bronchi and bronchioles accompanied in far advanced cases by epithelialization of the alveoli. The histopathology of maedi bears a close resemblance to pulmonary diseases of sheep reported under different terms in various parts of the world.

scholarly journals Histology of Interstitial Lung Disease in Common Variable Immune Deficiency

2020 ◽  
Vol 11 ◽  
Author(s):  
Fatima Dhalla ◽  
Dylan J. Mac Lochlainn ◽  
Helen Chapel ◽  
Smita Y. Patel
Keyword(s):  
Immune Deficiency ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Fibrosis ◽  
Granulomatous Inflammation ◽  
Lymphoid Hyperplasia ◽  
Adverse Outcomes ◽  
Common Variable Immune Deficiency ◽  
Immune Deficiencies ◽  
Common Variable

Interstitial lung disease (ILD) is an important non-infectious complication in several primary immune deficiencies. In common variable immune deficiency (CVID) it is associated with complex clinical phenotypes and adverse outcomes. The histology of ILD in CVID is heterogeneous and mixed patterns are frequently observed within a single biopsy, including non-necrotising granulomatous inflammation, lymphoid interstitial pneumonitis, lymphoid hyperplasia, follicular bronchiolitis, organizing pneumonia, and interstitial fibrosis; ILD has to be differentiated from lymphoma. The term granulomatous-lymphocytic interstitial lung disease (GLILD), coined to describe the histopathological findings within the lungs of patients with CVID with or without multisystem granulomata, is somewhat controversial as pulmonary granulomata are not always present on histology and the nature of infiltrating lymphocytes is variable. In this mini review we summarize the literature on the histology of CVID-related ILD and discuss some of the factors that may contribute to the inter- and intra- patient variability in the histological patterns reported. Finally, we highlight areas for future development. In particular, there is a need for standardization of histological assessments and reporting, together with a better understanding of the immunopathogenesis of CVID-related ILD to resolve the apparent heterogeneity of ILD in this setting and guide the selection of rational targeted therapies in different patients.

scholarly journals Rangeliosis: histopathological analysis, hematology and molecular detection of canine Rangelia vitalii in Rio Grande do Sul, Brazil

2017 ◽  
Vol 47 (10) ◽  
Author(s):  
Gabriela Fredo ◽  
Ronaldo Viana Leite-Filho ◽  
Camila De Ávila Pietzsch ◽  
Caroline Pinto de Andrade ◽  
Naila Cristina Blatt Duda ◽  
...  
Keyword(s):  
Mononuclear Cells ◽  
Plasma Cells ◽  
Infectious Agent ◽  
Rio Grande ◽  
Extramedullary Hematopoiesis ◽  
Lymphoid Hyperplasia ◽  
Histopathological Analysis ◽  
Rio Grande Do Sul ◽  
Genetic Sequencing

ABSTRACT: In the period from January 2004 to December 2015, 56 dogs were diagnosed with rangeliosis in the Setor de Patologia Veterinária at Universidade Federal do Rio Grande do Sul (SPV-UFRGS). The main hematological abnormalities were thrombocytopenia and anemia. The affected dogs showed signs of apathy, anorexia, fetid and bloody diarrhea, vomiting, and dehydration. At necropsy, the main changes were jaundice, splenomegaly, hepatomegaly, and lymphadenomegaly. Histological analyses revealed parasitophorous vacuoles of Rangelia vitalii in cytoplasmic endothelial cells, mainly in the heart, kidneys, lymph nodes, intestines, and pancreas. Inflammation characterized by mononuclear cells was predominant in the analysis, and most was due to the presence of plasma cells. Other lesion types observed were lymphoid hyperplasia, extramedullary hematopoiesis, erythrophagocytosis, and erythroid lineage hyperplasia in bone marrow. Of the total number of animals, 49 were diagnosed using necropsy and histological analysis, and seven were diagnosed using a molecular analysis (i.e., PCR and genetic sequencing of blood samples). This paper presented a different method of diagnosing rangeliosis in canines. This approach involved histological methods including the quantification and determination of the intensity and distribution of the infectious agent in different organs.

scholarly journals Role of Mucosal Inflammation in Eosinophilic Esophagitis: Review of the Literature

2011 ◽  
Vol 2011 ◽  
pp. 1-7 ◽  
Author(s):  
Ghulamullah Shahzad ◽  
Paul Mustacchia ◽  
Marianne Frieri
Keyword(s):  
Eosinophilic Esophagitis ◽  
Acid Suppression ◽  
Treatment Modalities ◽  
Mucosal Inflammation ◽  
Esophageal Mucosa ◽  
Immune Mediated ◽  
Acid Suppression Therapy ◽  
Clear Differentiation ◽  
Histological Confirmation

Eosinophilic esophagitis (EE) is increasingly recognized in adults. It is an inflammatory disease of the esophageal mucosa, with variable presentation, unresponsive to acid suppression therapy. The diagnosis requires histological confirmation of intense eosinophilic infiltration on esophageal biopsy specimen, however exact criteria required to make a diagnosis of EE is still being debated and a clear differentiation from gastroesophageal reflux disease (GERD) is important. Allergen elimination or anti-inflammatory therapy may be effective in such patients. The imperfect diagnostic criteria for EE mandate an understanding of the immunology and the pathophysiology of the disease. It may facilitate the introduction of novel treatment modalities in an individual unresponsive to acid suppression therapy. This paper describes basic elements of the immune-mediated injury to the esophageal mucosa and management aspects to provide a better understanding of the condition.

scholarly journals The critical role of histology in distinguishing sarcoidosis from common variable immunodeficiency disorder (CVID) in a patient with hypogammaglobulinemia

2019 ◽  
Vol 15 (1) ◽  
Author(s):  
Rohan Ameratunga ◽  
Yeri Ahn ◽  
Dominic Tse ◽  
See-Tarn Woon ◽  
Jennifer Pereira ◽  
...  
Keyword(s):  
Renal Impairment ◽  
Common Variable Immunodeficiency ◽  
Cauda Equina Syndrome ◽  
Critical Role ◽  
Cauda Equina ◽  
Histological Features ◽  
Diagnostic Difficulties ◽  
Life Threatening ◽  
Immune Defects ◽  
Common Variable

Abstract Background Common variable immunodeficiency disorders (CVID) are a rare group of primary immune defects, where the underlying cause is unknown. Approximately 10–20% of patients with typical CVID have a granulomatous variant, which has closely overlapping features with sarcoidosis. Case presentation Here we describe a young man who sequentially developed refractory Evans syndrome, cauda equina syndrome and most recently renal impairment. Following immunosuppression, he has made a recovery from all three life-threatening autoimmune disorders. As the patient was hypogammaglobulinemic for most of the time while on immunosuppression, vaccine challenges and other tests were not possible. Histological features were in keeping with sarcoidosis rather than the granulomatous variant of CVID. In the brief period when immunosuppression was lifted between the cauda equina syndrome and renal impairment, he normalised his immunoglobulins, confirming sarcoidosis rather than CVID was the underlying cause. Conclusion We discuss diagnostic difficulties distinguishing the two conditions, and the value of histological features in our diagnostic criteria for CVID in identifying sarcoidosis, while the patient was hypogammaglobulinemic. The key message from this case report is that the characteristic histological features of CVID can be very helpful in making (or excluding) the diagnosis, particularly when other tests are not possible.

scholarly journals Primary Humoral Immune Deficiencies: Overlooked Mimickers of Chronic Immune-Mediated Gastrointestinal Diseases in Adults

2020 ◽  
Vol 21 (15) ◽  
pp. 5223
Author(s):  
Ida Judyta Malesza ◽  
Michał Malesza ◽  
Iwona Krela-Kaźmierczak ◽  
Aleksandra Zielińska ◽  
Eliana B. Souto ◽  
...  
Keyword(s):  
Immunoglobulin A ◽  
Gastrointestinal Diseases ◽  
Primary Immunodeficiencies ◽  
Gastrointestinal Disorders ◽  
Humoral Immune ◽  
Current State ◽  
Immune Mediated ◽  
Immune Deficiencies ◽  
Inflammatory Bowel ◽  
Common Variable

In recent years, the incidence of immune-mediated gastrointestinal disorders, including celiac disease (CeD) and inflammatory bowel disease (IBD), is increasingly growing worldwide. This generates a need to elucidate the conditions that may compromise the diagnosis and treatment of such gastrointestinal disorders. It is well established that primary immunodeficiencies (PIDs) exhibit gastrointestinal manifestations and mimic other diseases, including CeD and IBD. PIDs are often considered pediatric ailments, whereas between 25 and 45% of PIDs are diagnosed in adults. The most common PIDs in adults are the selective immunoglobulin A deficiency (SIgAD) and the common variable immunodeficiency (CVID). A trend to autoimmunity occurs, while gastrointestinal disorders are common in both diseases. Besides, the occurrence of CeD and IBD in SIgAD/CVID patients is significantly higher than in the general population. However, some differences concerning diagnostics and management between enteropathy/colitis in PIDs, as compared to idiopathic forms of CeD/IBD, have been described. There is an ongoing discussion whether CeD and IBD in CVID patients should be considered a true CeD and IBD or just CeD-like and IBD-like diseases. This review addresses the current state of the art of the most common primary immunodeficiencies in adults and co-occurring CeD and IBD.

Bone Marrow Histology in Patients with Paroxysmal Nocturnal Hemoglobinuria.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4215-4215
Author(s):  
Sandra van Bijnen ◽  
Konnie Hebeda ◽  
Petra Muus
Keyword(s):  
Bone Marrow ◽  
Mast Cells ◽  
Aplastic Anemia ◽  
Paroxysmal Nocturnal Hemoglobinuria ◽  
Plasma Cells ◽  
Bone Marrow Failure ◽  
Clone Size ◽  
Immune Mediated ◽  
Lymphoid Nodules ◽  
Pnh Clone

Abstract Abstract 4215 Introduction Paroxysmal Nocturnal Hemoglobinuria (PNH) is a disease of the hematopoietic stem cell (HSC) resulting in a clone of hematopoietic cells deficient in glycosyl phosphatidyl inositol anchored proteins. The clinical spectrum of PNH is highly variable with classical hemolytic PNH at one end, and PNH in association with aplastic anemia (AA/PNH) or other bone marrow failure states at the other end. It is still largely unknown what is causing these highly variable clinical presentations. Immune-mediated marrow failure has been suggested to contribute to the development of a PNH clone by selective damage to normal HSC. However, in classic PNH patients with no or only mild cytopenias, a role for immune mediated marrow failure is less obvious. No series of trephine biopsies has been previously documented of patients with PNH and AA/PNH to investigate the similarities and differences in these patients. Methods We have reviewed a series of trephine biopsies of 41 PNH patients at the time the PNH clone was first detected. The histology was compared of 27 patients with aplastic anemia and a PNH clone was compared to that of 14 patients with classic PNH. Age related cellularity, the ratio between myeloid and erythroid cells (ME ratio), and the presence of inflammatory cells (mast cells, lymphoid nodules and plasma cells) were evaluated. The relation with clinical and other laboratory parameters of PNH was established. Results Classic PNH patients showed a normal or hypercellular marrow in 79% of patients, whereas all AA/PNH patients showed a hypocellular marrow. Interestingly, a decreased myelopoiesis was observed not only in AA/PNH patients but also in 93% of classic PNH patients, despite normal absolute neutrophil counts (ANC ≥ 1,5 × 109/l) in 79% of these patients. The number of megakaryocytes was decreased in 29% of classic PNH patients although thrombocytopenia (< 150 × 109/l) was only present in 14% of the patients. Median PNH granulocyte clone size was 70% (range 8-95%) in classic PNH patients, whereas in AA/PNH patients this was only 10% (range 0.5-90%). PNH clones below 5% were exclusively detected in the AA/PNH group. Clinical or laboratory evidence of hemolysis was present in all classical PNH patients and in 52% of AA/PNH patients and correlated with PNH granulocyte clone size. Bone marrow iron stores were decreased in 71% of classic PNH patients. In contrast, increased iron stores were present in 63% of AA/PNH patients, probably reflecting their transfusion history. AA/PNH patients showed increased plasma cells in 15% of patients and lymphoid nodules in 37%, versus 0% and 11% in classic PNH. Increased mast cells (>2/high power field) were three times more frequent in AA/PNH (67%) than in PNH (21%). Conclusion Classic PNH patients were characterized by a more cellular bone marrow, increased erythropoiesis, larger PNH clones and clinically by less pronounced or absent peripheral cytopenias and more overt hemolysis. Decreased myelopoiesis and/or megakaryopoiesis was observed in both AA/PNH and classic PNH patients, even in the presence of normal peripheral blood counts, suggesting a role for bone marrow failure in classic PNH as well. More prominent inflammatory infiltrates were observed in AA/PNH patients compared to classical PNH patients. Disclosures: No relevant conflicts of interest to declare.

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