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Vasculitis with liver involvement

Justyna Roszkiewicz, Elzbieta Smolewska
Within the last 30 years, the human immunodeficiency virus (HIV) infection has changed its status from inevitably fatal to chronic disorder with limited impact on life span. However, this breakthrough was mainly the effect of introduction of the aggressive antiviral treatment, which has led to the clinically significant increase in CD4+ cell count, resulting in fewer cases of the acquired immunodeficiency syndrome (AIDS) and improved management of opportunistic infections occurring in the course of the disease...
November 2016: Rheumatology International
A Torrelo, L Noguera-Morel, A Hernández-Martín, D Clemente, J M Barja, L Buzón, D Azorín, A A de Jesús, J C López-Robledillo, I Colmenero, H Kutzner, R Goldbach-Mansky, L Requena
BACKGROUND: Recurrent panniculitis in children with lipoatrophy has been loosely described and reported under different names, but has never been systematically evaluated by immunohistochemical stains. OBJECTIVE: To depict the profile of children with recurrent idiopathic panniculitis. METHODS: Study of clinical, histopathological and immunohistochemical features in five cases with recurrent idiopathic panniculitis. RESULTS: Five children with repeated attacks of painful subcutaneous nodules in association with fever, malaise and abdominal pain or arthralgia, with subsequent lipoatrophy were reviewed...
August 12, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
Ulf Müller-Ladner
BACKGROUND: Although the gastrointestinal (GI) tract including its related organs is not generally regarded as one of the primary organ systems of primary and secondary vasculitic disorders, there are numerous mechanisms of these diseases operative in or around the different structures and compartments of the GI tract. KEY MESSAGES: A majority of the respective clinical symptoms and problems are linked to an alteration of (peri)vascular homeostasis. Alteration of perivascular matrix metabolism can also affect the functional integrity and motility of the GI tract...
2016: Digestive Diseases
Juan S Calle Toro, Diana M Davalos, Jose D Charry, Ana M Arrunategi, Gabriel Tobon
Approximately 80% of patients with hepatitis C virus infection develop chronic liver disease as cirrhosis, and 40% develop autoimmune complications as mixed cryoglobulinemia (MC). Gastrointestinal involvement in MC is rare, and even more so is hepatic involvement. We report a case of an 87-year-old woman with a 10-year history of blood transfusion-acquired hepatitis C virus infection, without treatment. She consulted the emergency department for diffuse abdominal pain, associated with vomiting. After 2 weeks of hospitalization in the intensive care unit, a diagnosis of MC was made; cirrhosis and secondary mesenteric and hepatic vasculitis were confirmed by a diagnostic laparoscopy...
June 2016: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
M Y Pervakova, V L Emanuel, O N Titova, S V Lapin, V I Mazurov, I B Belyaeva, A L Chudinov, T V Blinova, E A Surkova
The deficiency of alpha-1 protease inhibitor, or alpha-1-antitrypsin (A1AT), predisposes to chronic lung diseases and extrapulmonary pathology. Besides classical manifestations, such as pulmonary emphysema and liver disease, alpha-1-antitrypsin deficiency (A1ATD) is also known to be associated with granulomatosis with polyangiitis (GPA or Wegener's granulomatosis). The aim of our study was to evaluate the frequency of allelic isoforms of A1AT and their clinical significance among GPA patients. Detailed clinical information, including Birmingham Vasculitis Activity Score (BVAS), incidence of lung involvement, anti-proteinase 3 (PR3) antibodies concentrations, and other laboratory data were collected in 38 GPA patients...
2016: International Journal of Rheumatology
Varun Aggarwal, Veronica Etinger, Andres F Orjuela
Kawasaki disease is a common nonspecific vasculitis seen in childhood. The most significant long-term sequela is coronary artery aneurysm. However, the spectrum of complications involves not only the heart, but also other organs such as the eyes, skin, kidneys, gallbladder, liver, and central nervous system. Sensorineural hearing loss (SNHL) is a relatively unrecognized complication of the disease. Although most of the complications (except coronary artery aneurysm) are self-limiting, SNHL can be persistent...
January 2016: Annals of Pediatric Cardiology
Shalini Thapar Laroia, Suman Lata
Chronic hepatitis caused by hepatitis B virus (HBV) is an endemic disease in India. It is associated with extrahepatic manifestations like polyarteritis nodosa (PAN) which is a vasculitis like disorder, presenting in subacute or chronic phase; involving visceral and systemic vessels. It should always be considered as a possible etiology of hypertension in an underlying setting of hepatitis B. We describe a 56-year-male patient with a history of chronic HBV who presented to the outpatient clinic with history of recent onset hypertension and suspected liver disease...
March 16, 2016: World Journal of Clinical Cases
Franco Dammacco, Vito Racanelli, Sabino Russi, Domenico Sansonno
Cryoglobulinemic vasculitis (CV) is a small-to-medium-vessel vasculitis that appears in 10-15 % of patients chronically infected with hepatitis C virus (HCV). The classic symptom triad of CV, purpura/asthenia/arthralgia, is accompanied by clinical features that include glomerulonephritis, neuropathy, interstitial pneumonitis, and cardiomyopathy, ranging in their severity from mild to life threatening. The risk of developing non-Hodgkin lymphoma is also higher. The cumulative 10-year survival rate of CV patients is significantly lower than in the age- and sex-matched general population, with death typically caused by nephropathy, malignancies, liver involvement, and severe infections...
August 2016: Clinical and Experimental Medicine
Fahd Khan, Srinivasan Srirangan, Yasser El-Miedany, Sanjeev Madaan
INTRODUCTION: This case highlights the complexities in the initial diagnosis and investigations of widespread lytic lesions initially perceived to be a widespread metastatic process and the consideration of alternative diagnosis. PRESENTATION OF CASE: A 57 year-old man with a background of psoriatic arthritis presented to the rheumatology department with lumbar back pain and sensory disturbance over L4/5. Magnetic resonance imaging (MRI) and bone scan identified lesions consistent with bony metastases at L5...
2016: International Journal of Surgery Case Reports
Margarida M Gonçalo, José C Cardoso, Miguel P Gouveia, Inês Coutinho, Ana R Gameiro, Maria M Brites, Óscar E Tellechea
OBJECTIVE: Exanthema in drug reaction with eosinophilia and systemic symptoms (DRESS) has no specific clinical diagnostic hallmark and there are few histopathologic studies. The aim of this study was to describe dermal-epidermal histopathologic features in DRESS and correlate them with the culprit drug, viral reactivation, or systemic organ involvement. METHODS: Skin biopsies were independently evaluated by 2 dermatopathologists who characterized the main histological patterns and scored dermal and epidermal changes, which were further correlated with clinical and laboratorial data...
June 2016: American Journal of Dermatopathology
Menno D Stellingwerff, Elisabeth Brouwer, Karel-Jan D F Lensen, Abraham Rutgers, Suzanne Arends, Kornelis S M van der Geest, Andor W J M Glaudemans, Riemer H J A Slart
Giant cell arteritis (GCA) is the most frequent form of vasculitis in persons older than 50 years. Cranial and systemic large vessels can be involved. [¹⁸F] fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) is increasingly used to diagnose inflammation of the large arteries in GCA. Unfortunately, no consensus exists on the preferred scoring method. In the present study, we aim to define the optimal FDG PET/CT scoring method for GCA diagnosis using temporal artery biopsy and clinical diagnosis as the reference method...
September 2015: Medicine (Baltimore)
Bhavtosh Dedania, George Y Wu
Hepatitis C virus (HCV) affects millions of people worldwide, and an estimated 3.2 million people in the United States. HCV is a hepatotropic and lymphotropic virus that causes not only liver disease, but also a significant number of extrahepatic manifestations (EHMs). Up to 74% of patients affected by HCV will have HCV-related EHMs of some severity in their lifetime. The EHMs vary from simple cutaneous palpable purpura to complex lymphoproliferative disorders, including lymphomas and immune-complex deposit diseases causing local and/or systemic complications...
June 28, 2015: Journal of Clinical and Translational Hepatology
Rossana Helbling, Sebastiano A G Lava, Giacomo D Simonetti, Pietro Camozzi, Mario G Bianchetti, Gregorio P Milani
OBJECTIVE: Involvement of the pancreato-biliary system has been occasionally noted in Henoch-Schönlein purpura. Furthermore, cases of this vasculitis syndrome sometimes develop in the context of a viral hepatitis or after hepatitis vaccination. METHODS: We completed a review of the literature. RESULTS: Fifty reports published between 1977 and 2015 were retained for the analysis. A pancreato-biliary involvement was recognized in 34 individually well-described patients (♂:♀ = 19:15) with severe abdominal pain: pancreatitis (N = 20), acalculous cholecystitis (N = 11), both pancreatitis and cholecystitis (N = 3)...
March 2016: Journal of Pediatric Gastroenterology and Nutrition
I Boukhris, S Azzabi, E Chérif, L Ben Hassine, Z Kaouech, C Kooli
AIM: Cryoglobulinemia is characterized by multiple organ involvement, mainly including the skin, liver, kidneys, and peripheral nerves. Our aim was to investigate the demographic, clinical, and serologic features, as well as survival in a group of 16 Tunisian patients with cryoglobulinemia. RESULTS: The study included 12 women and 4 men, and their mean age was 41 years. In all but two, the cryoglobulinemia was associated with another disease. These included lupus for 9, Sjögren syndrome for 2, and polyarteritis nodosa for one...
October 2015: Médecine et Santé Tropicales
Massimo Accorinti, Francesca Romana Pesci, Maria Pia Pirraglia, Roberta Priori, Paola Pivetti-Pezzi
OBJECTIVES: To report on the clinical course of ocular and extraocular involvement in a multidrug-resistant patient with Behçet's disease (BD). METHODS: A 22-year-old male with BD (bilateral panuveitis and retinal vasculitis, oral ulcers, erythema nodosum, arthralgia, epididymitis) was followed-up from 1999 to 2014. He was treated continuously with corticosteroids in combination with different immunosuppressive therapies (cyclosporine, azathioprine, methotrexate, interferon, infliximab, mycophenolate), which exerted numerous side-effects such as nephrotoxicity, nephrolithiasis, increase of liver enzymes, severe depression with suicidal ideation, severe leucopenia, pulmonary tuberculosis, pulmonary legionellosis, recurrent bronchopneumonia...
November 2015: Clinical and Experimental Rheumatology
Domenico Sansonno, Sabino Russi, Silvia Sansonno, Fabio Pavone, Franco Dammacco
INTRODUCTION: Hepatitis C virus (HCV) infection can be detected in virtually all patients with cryoglobulinemic vasculitis (CV). Among its many effects, the virus is able to stimulate the production of thymic stromal lymphopoietin (TSLP) by infected hepatocytes. In this study, we assessed the systemic levels and tissue distribution of TSLP in 60 chronically HCV-infected patients, 36 with and 24 without CV. METHODS: Serum TSLP levels were measured by an enzyme-linked immunosorbent assay (ELISA) method...
2015: Arthritis Research & Therapy
Clodoveo Ferri, Marco Sebastiani, Dilia Giuggioli, Michele Colaci, Poupak Fallahi, Alessia Piluso, Alessandro Antonelli, Anna Linda Zignego
The clinical course of chronic hepatitis C virus (HCV) infection is characterized by possible development of both liver and extrahepatic disorders. The tropism of HCV for the lymphoid tissue is responsible for several immune-mediated disorders; a poly-oligoclonal B-lymphocyte expansion, commonly observed in a high proportion of patients with HCV infection, are responsible for the production of different autoantibodies and immune-complexes, such as mixed cryoglobulins. These serological alterations may characterize a variety of autoimmune or neoplastic diseases...
March 27, 2015: World Journal of Hepatology
Patrice Cacoub, Cloe Comarmond, Fanny Domont, Léa Savey, David Saadoun
Cryoglobulinemic vasculitis (CryoVas) is a small-vessel vasculitis involving mainly the skin, the joints, the peripheral nervous system, and the kidneys. Type I CryoVas is single monoclonal immunoglobulins related to an underlying B-cell lymphoproliferative disorder. Type II and III cryoglobulins, often referred to as mixed cryoglobulinemia, consist of polyclonal immunoglobulin (Ig)G with or without monoclonal IgM with rheumatoid factor activity. Hepatitis C virus (HCV) infection represents the main cause of mixed CryoVas...
September 2015: American Journal of Medicine
Blenda Dias, Daniela Soares, Patrick Sampaio, Mittermayer Santiago
Granulomatosis with polyangiitis (GPA) is a small-vessel vasculitis consisting of necrotizing granulomatous lesions in airways and focal necrotizing glomerulonephritis. However, it may affect other sites such as the skin, central nervous system, eyes, heart, gastrointestinal tract, and liver. We describe a rare case of GPA in which the initial manifestation was the involvement of the parotid gland mimicking a pyogenic abscess.
2015: Case Reports in Medicine
E Rosenthal, P Cacoub
Patients with chronic hepatitis C virus (HCV) infection frequently present with extrahepatic manifestations covering a large spectrum, involving different organ systems leading to the concept of systemic HCV infection. These manifestations include autoimmune phenomena and frank autoimmune and/or rheumatic diseases and may dominate the course of chronic HCV infection. Chronic HCV infection causes liver inflammation affecting the development of hepatic diseases. HCV is also a lymphotropic virus that triggers B cells and promotes favorable conditions for B lymphocyte proliferation, including mixed cryoglobulinemia (MC) and MC vasculitis, which is the most prominent extrahepatic manifestation of chronic HCV infection...
April 2015: Lupus
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