Non-necrotizing Epithelioid Cell Research Articles

Interstitial Lung Disease in Primary Biliary Cholangitis: A Cohort Prospective Study.

Interstitial lung disease (ILD) has been recognized as an extrahepatic manifestation ofprimary biliary cholangitis (PBC), althoughlimited data are available on its prevalence and clinical significance. Therefore, we evaluated the occurrence and clinical features of ILD in a cohort of PBC patients. Ninety-three individuals without concomitant rheumatic diseases were enrolled in our prospective cohort study. All patients underwent chest high-resolution computed tomography (HRCT). Liver-related and lung-related survival wereassessed. A lung-related outcome was defined as death from ILD complications; a liver-related outcome was defined as liver transplantation or death from liver cirrhosis complications. HRCT findings suggestive ofILD were detected in 38 patients (40.9%). A sarcoid-like pattern of PBC-associated ILD was the most frequent, followed by subclinical ILD and organizing pneumonia. Patients with ILD were less likely to have liver cirrhosis and liver-related symptoms and presented with higher serum immunoglobulin M(IgM) and M2 subtype antimitochondrial antibodies (AMA-M2) positivity rates. In a multivariate analysis, the absence of liver disease symptoms at the disease presentation (OR 11.509; 95% CI 1.210-109.421; p = 0.033), the presence of hepatic non-necrotizing epithelioid cell granulomas (OR 17.754; 95% CI 1.805-174.631; p = 0.014), higher serum IgM (OR 1.535; 95% CI 1.067-2.208; p = 0.020) and higher blood leukocyte count (OR 2.356; 95% CI 1.170-4.747; p = 0.016) were independent risk factors associated with ILD in PBC. More than a third of patients with ILD showed no respiratory symptoms, and only one ILD-related death occurred during a follow-up of 29.0 months (IQR 11.5; 38.0). Patients with ILD had better liver transplant-free survival.ILD in PBC had a benign course and was associated with a lower liver disease severity. PBC-associated ILD should be included in a list of differential diagnoses of ILD.

Sarcoidosis with marked necrosis in enlarged lymph nodes mimics mycobacterial infection: a case report

BackgroundSarcoidosis is pathologically characterized by the formation of non-necrotizing epithelioid cell granulomas. However, pathological findings of patients with sarcoidosis have rarely revealed necrosis. We report here on a patient with sarcoidosis which needed to be distinguished from infectious disease because of marked necrosis in the lymph nodes.Case presentationA 46-year-old Japanese woman was referred to our hospital due to a dry cough and appetite loss. A chest X-ray and computed tomography revealed markedly enlarged mediastinal and hilar lymph nodes and hepatosplenomegaly. Surgical biopsy of these lymph nodes was performed in order to make a diagnosis. Pathological findings revealed epithelioid cell granuloma with marked necrosis that suggested infectious etiology such as mycobacterial and fungal infections. In addition to the pathological findings, immunoglobulin A (IgA) antibody for Mycobacterium avium complex (MAC), enlargement of lymph nodes and hepatosplenomegaly indicated disseminated MAC, while sarcoidosis was considered as another important differential diagnosis according to elevated angiotensin-converting enzyme, soluble interleukin-2 receptor and uveitis. While waiting for the results of the cultures of acid-fast bacilli, the symptoms of cough and consumption had worsened, and initiation of therapy was required before the confirmed diagnosis. The therapy for MAC was initiated because it was feared that immunosuppressive therapy containing corticosteroid for sarcoidosis could worsen the patient’s condition if MAC infection was the main etiology. However, the treatment for MAC was not effective, and it was clarified that no acid-fast bacilli were cultured in the liquid culture medium, so the diagnosis was corrected to sarcoidosis after reconsideration of clinical and pathological findings. Prednisolone (30 mg/day) was administered orally, and the patient’s symptoms and radiological findings improved.ConclusionSarcoidosis must be considered even if pathological findings reveal marked necrosis, because rare cases of sarcoidosis exhibit extensive necrosis in lymph nodes. It is extremely important to carefully examine the clinical and pathological findings through discussion with the examining pathologist to reach the correct diagnosis.

Pulmonary sarcoidosis: from clinical features to pathology- narrative review.

Sarcoidosis is a multisystem disease with unknown causes. The prevalence of sarcoidosis that occurs worldwide is highly variable. It is pathologically characterized by the formation of non-necrotizing epithelioid cell granuloma, mainly affecting the respiratory tract and involving extrapulmonary organs including the skin, heart, extrathoracic lymph nodes, central nerves and eyes. A correct diagnosis of sarcoidosis depends on the clinical symptoms, imaging observations, and characteristic histopathological findings. This study aims to review the recent contributions of pulmonary sarcoidosis. It is based on a search of a published article and review on pulmonary sarcoidosis. PubMed, Embase and CNKI were searched for latest developments from 1977. The most common clinical symptoms of sarcoidosis include dry cough, dyspnea, and chest discomfort. The imaging manifestations of sarcoidosis can be divided into typical and atypical findings. Lymphadenopathy is a typical imaging manifestation of sarcoidosis. Sarcoidosis is also a highly variable multisystem disease with an unpredictable clinical course. Clinically encountered cases cover a wide range from asymptomatic patients with incidental findings in radiographic images to chronic progressive diseases. Corticosteroids are the most widely recommended as the first-line treatment for symptomatic or organ-threatening disease in sarcoidosis, but they are associated with substantial toxic effects. Meanwhile, for sarcoidosis, the follow-up is also an important part of the diagnosis and treatment. Pulmonary sarcoidosis needs further recognition. In this paper, the clinical features, imaging observations, pathology, and treatment modalities for pulmonary sarcoidosis are presented and discussed vis-à-vis the current dilemma in diagnosis and therapy.

Sarcoidosis and Monoclonal Gammopathy of Undetermined Significance (MGUS): A True Association or Just a Coincidence?

Sarcoidosis is a systemic inflammatory disease characterized by the presence of noncaseating granulomas in different organs. Sarcoidosis associated with monoclonal gammopathy of undetermined significance (MGUS) is a rare finding with only 10 cases reported to date. We describe a 79-year-old male patient who presented with dry mouth for 4 months. Lip biopsy done prior to admission showed nonnecrotizing epithelioid cell granulomas. On admission, laboratory analysis was significant for elevated calcium, decreased parathyroid hormone, increased erythrocyte sedimentation rate, undetectable parathyroid hormone-related peptide (PTHrp), mildly decreased 25-hydroxyvitamin D, elevated 1,25-dihydroxyvitamin D, elevated angiotensin converting enzyme, and positive Bence Jones protein in the urine. Serum protein electrophoresis showed an elevated gamma globulin level at 38% and an IgG monoclonal gammopathy with an M-spike of 1.47. Bone marrow biopsy was consistent with MGUS. The patient showed significant improvement with steroids and was discharged with close follow-up from nephrology and oncology. Salivary gland involvement in patients with sarcoidosis is a rare finding. Our case is a valuable addition to the small number of cases described in the literature supporting an association between plasma cell disorders and sarcoidosis. Larger prospective studies are needed to determine if a true association between the two diseases exists.

Pyloric Gland Metaplasia of the Ileocecal Valve: Clinicopathologic Correlates of Inflammatory Bowel Disease

Pyloric gland metaplasia of the ileocecal valve, in the setting of Crohn’s disease, is an unusual clinical entity. Though its etiology and pathogenesis remains unclear, metaplastic changes have been associated with chronic inflammation and inflammatory bowel disease. Herein, we report a case of a 23-year-old male who presented for surveillance colonoscopy after being diagnosed with Crohn's disease four years ago. Diagnostic colonoscopy revealed stenosis of the ileocecal valve as well as a 5 mm polypoid circumferential non-obstructing lesion. Excisional biopsy followed by histopathology revealed pyloric metaplasia and non-necrotizing epithelioid cell granuloma. We discuss the clinical significance of pyloric gland metaplasia of the ileocecal valve in the context of inflammatory bowel disease.

Features of Granulematous Inflammation in Crohn’s Disease

The Crohn’s disease is a multisystem chronic idiopathic disease from inflammatory bowel disease group, which is characterized by the development of immune granulomatous inflammation with the formation of non-necrotizing epithelioid cell sarcoid granulomas as in the wall of the intestine, and extra-intestinal. Granulomas are found, on average, in half of patients with Crohn’s disease, and is twice more often at children, and also in operational material, in comparison with biopsy. Communication of development of granulomas with the autophagy-related genes is revealed and possibility of existence of two phenotypes or genotypes of a disease, depending on existence of a granulematous inflammation though it is impossible to exclude that in some cases granulomas remained not found. Further researches will allow to specify the frequency of development and pathogenesis of granulomas, and also their clinical and predictive value.

Sarcoidosis del adulto de inicio en la infancia: a propósito de un caso

Sarcoidosis, a multisystem disorder of unknown etiology that involves multiple organs, is rare in children. The true incidence and prevalence of childhood sarcoidosis is unknown. As in adults, many children with sarcoidosis may be asymptomatic; the disease may remain undiagnosed. A complete and systematic evaluation of the patient is essential for the sarcoidosis diagnosis in children. Here, we describe a case of 12-year-old female who presented with 2 years history of uveitis and hepatosplenomegaly. A chest computerized tomography revealed scattered peripheral pulmonary nodules and bilateral hiliar lymphadenopathy. Bone marrow aspiration and liver biopsy were not diagnostic. A lung biopsy showed non-necrotizing epithelioid cell granulomas. She was diagnosed with sarcoidosis according to demonstration of granulomatous inflammation and the exclusion of confusable entities

A Case of Sarcoidosis Presented Initially with Abnormal Liver Chemistry and Subsequently Developed Pulmonary Symptoms

Introduction: Sarcoidosis is a multisystem disease characterized by the presence of non-caseating granulomas in affected tissue. The common age of presentation is 20-40 years. Black people are three to four times more likely to have sarcoidosis, and may have more severe disease than Caucasians. Gastrointestinal tract involvement in sarcoidosis is rare, while liver involvement is common, following lung and lymph nodes. Up to 35% of patients with sarcoidosis have abnormal liver tests, and about 60% of patients with hepatic involvement have symptoms of fever, night sweats, anorexia, and weight loss. Case: We present a case of a 53-year-old male with comorbidities of hypertension, diabetes mellitus, and dyslipidemia, who initially presented with elevated blood glucose level. Liver tests were noted to be abnormal (elevated alkaline phosphatase (1021 U/L), and transaminases (AST 71 U/L and ALT 131 U/L). Further work-up, including hepatitis panel, ANA, ASMA, and AMA were negative. Chest X-ray, echocardiogram, abdominal ultrasound, and MRCP were negative for any acute pathology. Needle biopsy of liver revealed liver parenchyma with rare aggregates of histiocytic cells, consistent with non-nectrotizing granuloma. He presented six months later with persistent cough for more than a month. In view of recent abnormal liver biopsy, CT scan of the chest was done, which revealed multiple parenchymal and subpleural nodules in a perilymphatic distribution, consistent with sarcoidosis. Whole body gallium scan showed abnormal uptake visualized in both hila and lungs, consistent with active sarcoidosis. Angiotensin converting enzyme (ACE) level, which was initially normal (39 U/L), was found to be elevated (117 U/L) when patient presented with pulmonary symptoms. He was started on prednisone, and the pulmonary symptoms, as well as liver enzymes, improved significantly (alkaline phosphatase 170 U/L, AST 16 U/L and ALT 29 U/L) on follow-up visits.Figure: Liver biopsy showing hepatic parenchyma with non-necrotizing epithelioid cell granuloma.Conclusion: This is an interesting case of an adult male who initially was diagnosed to have hepatic sarcoidosis without symptoms and signs of portal hypertension. He eventually had pulmonary involvement with symptoms.

Recurrence of primary biliary cirrhosis and development of autoimmune hepatitis after liver transplant: A blind histologic study

This long-term study aimed to evaluate recurrence and evolution of primary biliary cirrhosis (PBC) after orthotopic liver transplantation (OLT). We reviewed "blindly" allograft biopsy specimens of women who underwent transplantation for PBC (n = 84), and women who received a transplant for chronic hepatitis C virus infection (CHCV ) (n = 108). All needle liver biopsy specimens obtained more than 6 months post-OLT were examined, including 83 specimens from 44 PBC patients and 152 specimens from 58 CHCV patients. Granulomatous destructive cholangitis was found in five biopsies from four PBC patients (P = 0.0048). Non-necrotizing epithelioid cell granulomas were present in four biopsies from four PBC patients, and in two biopsies from one CHCV patient. Piecemeal necrosis (P = 0.0002), lobular necroinflammatory activity (P < 0.0001), steatosis (P < 0.0001) and fibrosis (P < 0.0001) were more prevalent in CHCV patients than PBC patients. Four PBC patients developed histologic evidence of autoimmune hepatitis (AIH), at a mean time of 3.66 years post-OLT. One of these patients had histologic features of AIH/PBC overlap syndrome. All four patients developed bridging fibrosis (n = 2) or cirrhosis (n = 2). No other PBC patient had evidence of cirrhosis after OLT. Histologic findings indicative of recurrent PBC were present in 15.9% of the PBC patients undergoing biopsy in this series. However, this group of patients did not suffer significant bile duct loss or fibrosis, as compared to the control group, suggesting that recurrent PBC is a mild or slowly progressive disease. Histologic evidence of AIH was observed in allograft biopsies of some PBC patients.

Diagnostic Value of Epithelioid Cell Granulomas in Bronchoscopic Biopsies

The granulomatous inflammatory response is a manifestation of many lung diseases. To evaluate the diagnostic value of epithelioid cell granulomas in bronchoscopic biopsies in daily clinical practice. The data of 105 patients with epithelioid cell granulomas in biopsy tissue who had undergone the bronchoscopic lung biopsy or bronchial biopsy at the Centre of Pulmonology and Allergology of Vilnius University Hospital Santariski klinikos (Vilnius, Lithuania) were examined. All cases were divided into non-necrotizing epithelioid cell granulomas and epithelioid cell granulomas with necrosis. Of all the cases 66% had non-necrotizing epithelioid cell granulomas and 34% had epithelioid cell granulomas with necrosis. Without respect to the presence of necrosis in granulomas, the majority of the patients (79%) had sarcoidosis or tuberculosis; 94% of the patients with sarcoidosis had non-necrotizing epithelioid cell granulomas and the remaining 6% had granulomas with necrosis. The sensitivity of non-necrotizing epithelioid cell granuloma for the diagnosis of sarcoidosis was 94% and specificity 60%. The positive and negative predictive values were 68% and 92%, respectively. Of the patients with tuberculosis 76% had epithelioid cell granulomas with necrosis and 24% had non-necrotizing epithelioid cell granulomas. The sensitivity of epithelioid cell granuloma with necrosis for the diagnosis of tuberculosis was 76% and specificity 85%. The positive and negative predictive values were 69%, and 88%, respectively. A significant overlap in types of granulomatous inflammation between tuberculosis and sarcoidosis was found. The type of epithelioid cell granuloma alone was not sufficient for the final clinical diagnosis.