Causes Of Granulomatous Inflammation Research Articles

An Unaccustomed Presentation of Besnier-Boeck-Schaumann Disease

Eliminating other potential causes of granulomatous inflammation helps to diagnose the mysterious sarcoidosis. All clinicians face a diagnostic dilemma because of the clinical polymorphism and the multisystemic sarcoidosis symptoms. After the lungs, the skin is the organ most frequently impacted by sarcoidosis. Before, during, or after systemic involvement, dermatological symptoms can occur, and the kind of skin lesion can have a bearing on the prognosis. Additionally, compared to a biopsy of visceral organs, a skin lesion biopsy is easier to perform and less intrusive. Therefore, there are some instances in which cutaneous symptoms can help with the diagnosis and outlook of a systemic illness. A high index of clinical suspicion is needed for early diagnosis and timely management, which can improve the quality of life for those patients and reduce morbidy and mortality due to this disease. This article has emphasised the prevalence, clinical characteristics, and treatment of the common skin lesions of sarcoidosis.

Sarcoidosis: Epidemiology and clinical insights.

Sarcoidosis is characterized by noncaseating granulomas which form in almost any part of the body, primarily in the lungs and/or thoracic lymph nodes. Environmental exposures in genetically susceptible individuals are believed to cause sarcoidosis. There is variation in incidence and prevalence by region and race. Males and females are almost equally affected, although disease peaks at a later age in females than in males. The heterogeneity of presentation and disease course can make diagnosis and treatment challenging. Diagnosis is suggestive in a patient if one or more of the following is present: radiologic signs of sarcoidosis, evidence of systemic involvement, histologically confirmed noncaseating granulomas, sarcoidosis signs in bronchoalveolar lavage fluid (BALF), and low probability or exclusion of other causes of granulomatous inflammation. No sensitive or specific biomarkers for diagnosis and prognosis exist, but there are several that can be used to support clinical decisions, such as serum angiotensin-converting enzyme levels, human leukocyte antigen types, and CD4 Vα2.3+ T cells in BALF. Corticosteroids remain the mainstay of treatment for symptomatic patients with severely affected or declining organ function. Sarcoidosis is associated with a range of adverse long-term outcomes and complications, and with great variation in prognosis between populations. New data and technologies have moved sarcoidosis research forward, increasing our understanding of the disease. However, there is still much left to be discovered. The pervading challenge is how to account for patient variability. Future studies should focus on how to optimize current tools and develop new approaches so that treatment and follow-up can be targeted to individuals with more precision.

Granulomatous AppendicitisA Rare Case Report

In 1953, Meyerding and Bertram were the first person to report non specific granulomatous inflammation in appendix. Granulomatous appendicitis is a rare condition which usually account to less than 2% of the cases, however idiopathic granulomatous appendicitis is very rare, and diagnosis is usually made by excluding all other conditions. It is challenging to diagnose tuberculous appendicitis based on clinical findings, hence histopathological examination is required. Hereby, authors report a case of 26-year-old male who presented with pain abdomen in the Department of Surgery. On imaging, appendix was thickened, and acute appendicitis was considered as a probable diagnosis. On histopathological examination, it was diagnosed as granulomatous inflammation suggestive of tuberculosis. The present case is presented for its rarity as appendicitis is considered as an abdominal emergency, so prompt management and diagnosis is required by excluding all other causes of granulomatous inflammation in appendix.

A Rare Case of Sarcoidosis With Urologic Symptoms As the Presenting Feature

Sarcoidosis is a multisystem idiopathic disease that can affect virtually any organ of the human body. However, genitourinary tract involvement is rare. We describe the case of a 33-year-old man with post-coital right scrotal pain. Scrotal ultrasound showed two vascularized nodular lesions in the right testicle and one in the left. A thoracic and abdominopelvic computed tomography scan showed micronodular infiltrate in the liver, spleen, lungs, and millimetric retroperitoneal and iliac lymph nodes. Levels of alpha-fetoprotein and human chorionic gonadotropin beta were normal. On positron emission tomography, the previously documented micronodular infiltrate exhibited features suggestive of an inflammatory etiology. The aspiration cytology of an iliac ganglion was described as normal, with no malignant cells. A liver biopsy revealed non-caseating epithelioid granulomas characteristic of granulomatous hepatitis. After exclusion of other causes of granulomatous inflammation, conjugation of clinical and histological features led us to the diagnosis of sarcoidosis with pulmonary, hepatic, splenic, and genitourinary involvement. This clinical report describes one of the rare occasions when the reproductive tract is affected by sarcoidosis and is the first organ to present signs of involvement by the disease, which reinforces the importance of considering sarcoidosis in the differential diagnosis of urologic conditions. The diagnosis of testicular sarcoidosis is challenging and the issue of its impact on fertility is particularly important.

Pulmonary Sarcoidosis: Diagnosis and Differential Diagnosis.

Sarcoidosis is a multisystem disorder of unknown origin and poorly understood pathogenesis that predominantly affects lungs and intrathoracic lymph nodes and is characterized by the presence of noncaseating granulomatous inflammation in involved organs. The disease is highly heterogeneous and can mimic a plethora of other disorders, making diagnosis a challenge even for experienced physicians. The evolution and severity of sarcoidosis are highly variable: many patients are asymptomatic and their disease course is generally benign with spontaneous resolution. However, up to one-third of patients develop chronic or progressive disease mainly due to pulmonary or cardiovascular complications that require long-term therapy. The diagnosis of sarcoidosis requires histopathological evidence of noncaseating granulomatous inflammation in one or more organs coupled with compatible clinical and radiological features and the exclusion of other causes of granulomatous inflammation; however, in the presence of typical disease manifestations such as Löfgren’s syndrome, Heerfordt’s syndrome, lupus pernio and asymptomatic bilateral and symmetrical hilar lymphadenopathy, the diagnosis can be established with high level of certainty on clinical grounds alone. This review critically examines the diagnostic approach to sarcoidosis and emphasizes the importance of a careful exclusion of alternative diagnoses.

A RARE CASE OF SARCOIDOSIS CAUSING OSTEOLYTIC LESIONS IN BOTH JAWS AND SKULL

<h3>Background</h3> A 65-year-old woman presented to the oral maxillofacial department with a nonhealing oral antral fistula. Radiographic examination revealed opacification of the left maxillary antrum and an ill-defined radiolucency in the maxilla. The patient continued to develop further osteolytic lesions in the skull and mandible, leading to anesthesia of the third division of the trigeminal nerve. <h3>Objective</h3> To discuss the clinicopathological correlation, differential diagnosis, and pathway to achieving a definitive diagnosis in this challenging case. <h3>Results</h3> Clinical symptoms included a history of fatigue, painful joints, and chest pain. Multiple biopsies taken from the osteolytic lesions of the mandible, maxilla, and a supraclavicular lymph node revealed naked noncaseating granulomas. Computed tomography (CT) of the head and neck revealed an irregular, diffuse, mottled appearance of the bone in the left maxillary alveolus, right mandible, and left temporal bone. Angiotensin-converting enzyme and serum calcium levels were normal. CT scans showed hilar lymphadenopathy, which was not visible on plain film radiography. Exclusion of other causes of granulomatous inflammation eventually led to a diagnosis of sarcoidosis. The patient has been discussed at a multiple disciplinary team meeting and referred to a specialist team. <h3>Conclusions</h3> Sarcoidosis is a chronic multisystem granulomatous disease characterized histologically by naked noncaseating granulomas. It has an unknown etiology and no pathognomonic diagnostic test, making definitive diagnosis difficult. This patient presented with rare and aggressive features appearing initially to be from the maxillary antrum, eventually affecting the jaws and skull with only subtle symptoms of the more cardinal features of the disease.

Update in treatment of pulmonary sarcoidosis

Sarcoidosis is a chronic granulomatous multisystem disease with an unknown etiology. The diagnosis of sarcoidosis relies on the presence of noncaseating granuloma on histopathological examination with compatible clinical presentation and exclusion of other causes of granulomatous inflammation. Lungs and intrathoracic lymph nodes are the most commonly affected organs. Most of the patients are asymptomatic. Symptomatic patients generally have spontaneous resolution. 25% of patients have progressive lung disease and 10% of patients have organ failure. Corticosteroids, immunosuppressive, cytotoxic, and antimalarial drugs are used for sarcoidosis treatment. The decision of appropriate treatment is very important. The aim of this review is to summarize the actual treatment of pulmonary sarcoidosis.

Comparison of Fine Needle Aspiration Cytology, Ziehl-Neelsen Staining and GeneXpert Methods in Suspected Cases of Tubercular Lymphadenopathy

Introduction: The Extrapulmonary Tuberculosis (EPTB) is challenging due to the pauci-bacillary nature of the disease and limited tests available for early diagnosis. It is the most common extrapulmonary manifestation of tuberculosis. The samples from extrapulmonary infection will have low bacterial count as compared with sputum specimens. It is also necessary to rule out other causes of granulomatous inflammation on Fine Needle Aspiration Cytology (FNAC) and confirmation by Ziehl Neelsen (ZN) stain and Cartridge Based Nucleic Acid Amplification Test (CBNAAT). In December 2010, World Health Organisation (WHO) recommended GeneXpert/CBNAAT to be used as the initial diagnostic test in suspected EPTB cases. Aim: To compare FNAC and ZN stain with CBNAAT in the diagnosis of suspected tubercular lymphadenopathy in a tertiary care centre and to know the importance of CBNAAT in the modern era. Materials and Methods: This was a hospital based prospective study carried out over a period of 12 months (July 2019 to June 2020) in the Department of Pathology and Microbiology, ESIC Medical College. All presumptive cases of tubercular lymphadenopathy and purulent aspirates from the lymph nodes of various sites were included in the study. Smears were made after FNA and stained with Haematoxylin and Eosin (H&amp;E) stain and ZN stain and sample was also processed for CBNAAT in all cases of lymphadenopathy. Statistics were done using SPSS software version 20.00. Results: The total number of cases with presumptive tubercular lymphadenitis was 119. Majority of the aspirates are from jugular lymph nodal and cervical swellings 77 (64.7%) out of 119. FNAC has detected tuberculosis in 20 (16.8%) cases and ZN stain detected Acid Fast Bacilli (AFB) in 6 (5%) cases. CBNAAT has detected 28 (23.5%) cases, among them 15 (12.6%) cases which were not detected by FNAC. Diagnostic Performance of CBNAAT versus FNAC 28 out of 119 showed sensitivity, specificity, negative predictive value and positive predictive value 80%, 85.8%, 65% and 45.5%. Conclusion: The CBNAAT can be added with FNAC to get more specific results. CBNAAT is less sensitive for blood stained samples than purulent samples and hence FNAC still remains as the cheapest and first line test to diagnose in cases suspected of tubercular lymphadenoapathy.

Idiopathic Granulomatous Mastitis of the Breast: Radiologic-Pathologic Correlation.

Granulomatous inflammation is an uncommon inflammatory condition of the breast that includes both infectious (bacterial, fungal, parasitic) and noninfectious (autoimmune, sarcoidosis, idiopathic granulomatous mastitis [IGM], reaction to foreign materials) etiologies. IGM is the most common subset of granulomatous inflammation where no underlying etiology is established. Infectious causes of granulomatous inflammation should be excluded, as these have established treatments that can significantly improve patient outcomes. IGM should be considered in the differential when mastitis is refractory to antibiotics. Patients usually present with an erythematous, tender, palpable unilateral breast mass. The most common mammographic presentation is a focal or global asymmetry. The imaging appearance mimics breast cancer, therefore diagnosis usually requires tissue sampling with histopathologic analysis and cultures to exclude infection. When patients are diagnosed with IGM, this poses a clinical dilemma as there are a variety of treatment options available, including oral and intralesional steroids. The time course of the disease is often prolonged by multiple recurrences, and specific treatment remains an area of ongoing research. The purpose of this article is to review the range of clinical features, imaging manifestations, associated histopathology, and management of IGM.

Diatoms: A novel cause of granulomatous inflammation of the head and neck

We report the first 4 cases of intraoral nonnecrotizing granulomatous foreign body reactions to diatoms, plausibly as a result of exogenous material introduced following iatrogenic or traumatic injury. Clinical and histopathologic findings of 4 intraoral cases of nonnecrotizing granulomatous foreign body reaction to diatoms, single-celled algae belonging to the taxonomic phylum Bacillariophyta, are reported. The lesions presented either in the jaws or in the soft tissue overlying the alveolar bone, in some instances mimicking an inflammatory lesion of odontogenic etiology. Microscopically, the lesions presented as nonnecrotizing granulomatous inflammation associated with either spherical and radially symmetric or rectangular and bilaterally symmetric diatomaceous foreign material. The diagnosis of a diatom-associated foreign body reaction necessitates familiarization with the histopathologic features of these organisms to accurately characterize the nature of such lesions.

A Sarcoidosis Case With Galaxy Sign Admitted With A Rare Symptom : Hemoptysis

30 year-old male patient applied to hospital with complaint of hemoptysis. 30 year-old male patient applied to hospital with complaint of hemoptysis. Bilateral extensive nodular infiltrations and mediastinal lymphadenopathies were detected. Wedge resection and parietal pleura biopsy were performed for the patient. The pathology report indicated that the case should be further examined from the aspect of granulomatous diseases, especially the sarcoidosis. The diagnosis was made as galaxy sign of sarcoidosis. Hemoptysis is a rare symptom seen in sarcoidosis. The hemoptysis is observed at advanced stage of sarcoidosis and in presence of cavity in sarcoidosis. There is no study in literature regarding the initial complaint of hemoptysis in sarcoidosis case, for whom the galaxy appearance was observed radiologically. Here, we present the etiological causes of granulomatous inflammation in sarcoidosis with non-typical clinic and radiology.

The Elliptical Aggregates – Idiopathic Granulomatous Mastitis

Idiopathic granulomatous mastitis was initially scripted in 1972 by Kessler and Wolloch and is cogitated as an infrequent, benign, chronic inflammatory disorder. It commonly implicates females of reproductive age group, especially women who have breast fed in the preceding five to six years. Idiopathic granulomatous mastitis is commonly discerned in Asia and Mediterranean region although true incidence is undetermined. Diverse ethnicities delineate a diverse disease prevalence. Elimination of adjunctive causes of granulomatous inflammation makes idiopathic granulomatous mastitis a diagnosis of exclusion. As per the natural history, idiopathic granulomatous mastitis is designated as a self limiting disorder 1.

Role of Bronchoscopic Techniques in the Diagnosis of Thoracic Sarcoidosis.

The diagnosis of sarcoidosis relies on clinical and radiological presentation, evidence of non-caseating granulomas in histopathology and exclusion of alternative causes of granulomatous inflammation. Currently, a proper diagnosis, with a high level of confidence, is considered as key to the appropriate diagnosis and management of the disease. In this sense, this review aims to provide a brief overview on the role of bronchoscopy in the diagnosis of thoracic sarcoidosis, incorporating newer techniques to establish, including endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA), endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA), transesophageal ultrasound-guided needle aspiration with the use of an echo bronchoscope (EUS-B-FNA) and transbronchial lung cryobiopsy (TBLC). Most of the literature reports the diagnostic superiority of endosonographic techniques, such as EBUS-TBNA alone or in combination with EUS-FNA, over conventional bronchoscopic modalities in diagnosing Scadding stages I and II of the disease. Moreover, TBLC may be considered a useful and safe diagnostic tool for thoracic sarcoidosis, overcoming some limitations of transbronchial lung biopsy (TBLB), avoiding more invasive modalities and being complementary to endosonographic procedures such as EBUS-TBNA.

Pattern Analysis of granulomatous inflammatory lesions

Pattern Analysis of granulomatous inflammatory lesions - IJPO- Print ISSN No: - 2394-6784 Online ISSN No:- 2394-6792 Article DOI No:- 10.18231/j.ijpo.2019.069, Indian Journal of Pathology and Oncology-Indian J Pathol Oncol

A rare case of primary tuberculosis in a solitary thyroid nodule

Tuberculosis (TB) of the thyroid gland is extremely rare with fewer than 200 cases reported worldwide. We report a case of primary TB of the thyroid in a 73-year-old gentleman with no risk factors for TB, presenting with a painless right neck mass of one week's duration. A pre-operative ultrasound scan reported a solid-cystic, right TIRADS 5 nodule, and initial fine needle cytology showed granulomatous inflammation and was negative for AFB on Ziehl-Neelsen stain. A right hemithyroidectomy was done subsequently, and histology demonstrated necrotizing granulomatous inflammation, but TB-PCR detected Mycobacterium DNA.Primary TB of the thyroid is rare and can manifest in patients with no apparent risk factors for TB. Initial cytology may only yield granulomatous inflammation and surgery is needed to ascertain the exact cause of granulomatous inflammation and additional TB-PCR can help clinch the diagnosis.

Sarcoidosis: a Critical Review of History and Milestones.

Sarcoidosis is a chronic systemic disease of unknown origin and uncertain prognosis that most commonly affects young adults, and frequently presents with bilateral hilar lymphadenopathy, pulmonary infiltrates and ocular and skin lesions. The diagnosis is established when characteristic clinical-radiological features are supported by compatible histopathology of epithelioid cell granulomas, following exclusion of known causes of granulomatous inflammation. Indeed, sarcoidosis belongs to a large family of disorders that share granuloma formation as common denominator. Since its first description by Jonathan Hutchinson in 1869, sarcoidosis has generated enormous interest and considerable controversy. In Hutchinson's day, it was considered a dermatological condition, which gradually evolved into a multisystem disorder associated in the majority of cases with respiratory abnormalities. With time, it has also become clear that sarcoidosis occurs throughout the world, affecting individuals of both genders and all races, although its prevalence varies widely across ethnic and racial groups. In recent years, advances in different disciplines, particularly biochemistry, genetics, immunology and molecular biology, have improved dramatically our understanding of the disease. Yet, the critical questions regarding who gets sarcoidosis and whether it has an infectious origin remain unanswered. Sarcoidosis has a distinguished medical history that covers the last 150 years. Right from the time of seminal contributions by Hutchinson, Besnier and Boeck medical discussion on sarcoidosis has always been animated and to a certain extent emotional. Such discussions will inevitable continue until the true cause of the disease has been found, hopefully in the near future.

Case Report: Orofacial Granulomatosis

Orofacial granulomatosis (OFG) is an uncommon condition that presents as non-tender, non-pruritic, chronic oral labial swelling. It is a diagnosis of exclusion, and requires a full systemic workup to exclude other causes of granulomatous inflammation, such as Crohn’s disease, sarcoidosis, and tuberculosis. The cause of OFG is unknown, and there is significant debate as to whether it is in fact a separate entity, or merely an initial and/or localized form of Crohn’s disease (CD). We describe a case of OFG in a 37 year-old male from Newfoundland, Canada, and discuss in detail the systemic workup, and the reasoning behind the diagnosis. A photo of the lesion is included, as well as histopathology showing non-caseating granulomatous inflammation. Short discussions of etiology and treatment options are also included.

Diagnosis of neurosarcoidosis: Necessity of biopsy

Sarcoidosis can affect any part of the central nervous system presenting with an extremely diverse clinical picture. Clinical presentations actually depend on the localization ofgranulomas in the central nervous system. Making diagnosis according to the localization and the clinical variations is often a clinical challenge. DIAGNOSIS OF NEUROSARCOIDOSIS: Diagnosis is based on the clinical picture, clinical and radiological findings (magnetic resonance imaging with contrast endocranium), laboratory findings (angio-tenzin-converting enzyme and chitotriosidase in cerebrospinal fluid); however, it is necessary first to exclude all other possible causes of granulomatous inflammation. Recent studies in patients with neurosarcoidosis show a high value of at least one marker of the disease. The safest way and the gold standard in diagnosing this disease would be histopathological confirmation, which is rarely performed due to its invasiveness. New diagnostic methods will contribute to better methods of bypassing invasive procedures, and they will significantly facilitate the diagnosis of neurosarcoidosis, which is a real challenge even for experienced clinicians who deal with this disease.

Actinic Granuloma of the Conjunctiva in Young Women

To report the occurrence of actinic granuloma of the conjunctiva in young women. Retrospective case series. Three eyes of 3 young women with a unilateral conjunctival mass of recent onset. Three young women (21, 23, and 23 years of age) sought treatment for painless red masses of the conjunctiva that were thought clinically to be pingueculitis, actinic keratosis, or ocular surface squamous neoplasia (OSSN), but were diagnosed histopathologically as actinic granuloma. Special stains were performed to exclude infectious and autoimmune causes of granulomatous inflammation, followed by dermatologic work-up and systemic investigations. Diagnosis of actinic granuloma of the conjunctiva was confirmed. Patients' characteristics (age, sex, early onset), clinical presentation, same geographical location, typical histopathologic features, and lack of systemic association. All 3 cases were in young women with a rapid-onset conjunctival growth with conjunctival feeder vessels and intrinsic vessels; however, there was no surface keratin, and rose bengal stain results were negative. Histopathologic analysis of all 3 cases revealed elastolysis and granulomatous inflammation characterized by multinucleated giant cells, some of which displayed elastophagocytosis that was highlighted by Verhoeff-van Giesen elastic stain. There was absence of cytologic atypia of surface epithelium. Mucin stains and special stains for an infectious cause demonstrated negative results. Serologic investigations and systemic work-up results were negative, which excluded any systemic associations with this condition. This led to the final diagnoses of actinic granuloma of the conjunctiva. Conjunctival actinic granuloma is an underrecognized entity. We present 3 cases of this condition to highlight its occurrence in young women and the role of histopathologic examination to avoid misinterpretation of this condition. Both clinician and pathologist should consider this entity as a differential diagnosis of benign conjunctival lesions and OSSN. The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Concomitant sarcoidosis and a connective tissue disease: Review of the clinical findings and postulations concerning their association

Known causes of granulomatous inflammation must be excluded before the diagnosis of sarcoidosis can be secured. We explored the possibility that connective tissue diseases (CTDs) could be a cause of granulomatous inflammation through an analysis of patients cared for in 2 medical centers and a review of the medical literature. Patients with both a diagnosis of sarcoidosis and a CTD were identified at two medical centers. In addition, a literature search identified reported cases of patients with both diagnoses. We identified 15 patients at 2 medical centers plus 53 previous reported patients in the medical literature (total=68) with diagnoses of both sarcoidosis and a CTD. The patients were predominantly female. Scleroderma was the most common CTD. Only 2/59 (3%) with chest radiographs recorded had fibrocystic (stage 4) disease. In 48/67 (72%) patients where the time of diagnosis was recorded, sarcoidosis was diagnosed simultaneously or after the CTD. Sarcoidosis in only one organ ("isolated sarcoidosis") was associated with sarcoidosis being diagnosed simultaneously or after the CTD (p=0.0001). These data suggest that a significant portion of patients with CTDs and sarcoidosis may actually not have the latter disease. Rather, the CTD may "cause" granulomatous inflammation. Alternate explanations for these findings include that sarcoidosis and/or the connective tissue disease was misdiagnosed in these patients. The diagnosis of a concomitant connective tissue disease and sarcoidosis must be made with extreme caution.