Manifestation Of Systemic Disease Research Articles

Cutaneous Langerhans cell histiocytosis and other systemic inflammatory or autoimmune disease manifestations in the setting of clonal hematopoiesis

Cutaneous Langerhans cell histiocytosis and other systemic inflammatory or autoimmune disease manifestations in the setting of clonal hematopoiesis

Stepping Stones In Prevention of Kidney Stone Disease In Pakistan

The prevalence and incidence of kidney stone disease (KSD) has increased in Pakistan since last 5 decades, it is not only the cause of pain, hematuria and fever but is also an important cause of chronic kidney disease, Morbidity and mortality as well if left untreated. It is recurrent disease and can be manifestation of systemic diseases, (D.M, obesity, HTN) genetic and environmental factors play a role in etiology of disease. In spite of modern methods of surgical removal available in Pakistan there is constant increase in number of patients. Economic burden of disease is quite significant, so established metaphylaxsis program is essential part of management of this surgical and medical disease. Main therapies based on history, stone analysis, 24 hour urine metabolic study constitutes the basis of prevention. Increasing fluid intake dietary modification, Pharmacotherapy is the main stay of metaphylaxsis. Compliance of the patient and establishment of specialized stone clinics should be managed jointly by urologist, nephrologist, dietitians, metabolic laboratory expert and Medical Social officers. Prevention is possible in many cases if these patients are managed jointly by multidisciplinary team.

Exploring Headaches in Pediatric Behçet Disease: Prevalence, Clinical Impact, and Management.

Behçet's Disease (BD), also recognized as Behçet Syndrome, manifests uniquely in pediatric populations as Pediatric Behçet's Disease (PBD), characterized by multisystemic inflammatory symptoms including recurrent oral and genital aphthae, and diverse ocular, vascular, and neurological involvements. This review elucidates the prevalence, burden, and management strategies of headaches in children with PBD, focusing on both primary headaches, such as migraine and tension-type headaches, and secondary headaches linked to systemic disease manifestations. It explores the pathophysiological underpinnings specific to PBD-related headaches and discusses the intricate relationship between systemic inflammatory processes and neurological symptoms. By examining the literature from 2004 to 2024, this study highlights the high frequency of headache in PBD patients, underscoring its diagnostic and clinical significance. We aim to provide a detailed understanding of headache management in PBD, emphasizing tailored therapeutic strategies that address the unique challenges faced by this patient population. This review also underscores the importance of comprehensive clinical evaluations to optimize outcomes and mitigate long-term sequelae, proposing that awareness and understanding of headache in PBD can significantly enhance both diagnosis and management.

P.066 A 65 year-old male with subacute asymmetric, proximal more than distal upper extremity weakness with associated paresthesias and pain

Background: Neuroborreliosis affects approximately 10-15% of people with untreated Lyme disease and typically declares itself 2-18 weeks after infection. North American neuroborreliosis often manifests with cranial nerve palsy, meningitis, and/or radiculoneuritis. Methods: Here we describe a case of North American neuroborreliosis and also highlight some of the rare manifestations of systemic Lyme disease. A 65-year old male presented with a subacute history of progressive upper extremity weakness, neck pain, and headache. This occurred in the context of a recent tick exposure. Results: MRI of the brachial plexus, serology and CSF studies, and EMG/NCS were consistent with a diagnosis of polyradicular neuroborreliosis. However, whole body imaging identified some concerning features suggestive of lymphoma: specifically a large necrotic mediastinal lymph node and a number of vascular abnormalities. In light of these findings, the differential also included neurolymphomatosis and a PET scan was conducted. Reassuringly, there was no increase in FDG avidity in the distribution of his affected nerves. Moreover, his neurologic symptoms exhibited clinical improvement following treatment of his neuroborreliosis. Conclusions: This case provides an excellent example of the clinical features of neuroborreliosis, but more importantly also highlights some of the rarer potential manifestations, which warrant further investigation.

Periodontal manifestations of local and systemic diseases: Color atlas and text.

Periodontal manifestations of local and systemic diseases: Color atlas and text.

Subcutaneous Nodules as Manifestations of Systemic Disease

The spectrum of disorders/phenomena encompassed in the practice of rheumatology is quite broad. In addition, our expertise is typically sought whenever other physicians encounter phenomena outside their knowledge base. While skin alterations typically prompt referrals to dermatology practices, alterations underlying the skin (e.g., subcutaneous) may well represent localization in “no man’s land” or an orphaned localization, with rheumatology thus referred as to the specialty of last resort—one of the roles that rheumatology has fulfilled for more than half a century. The current review addresses the cacophony of disorders producing or associated with variouslysized subcutaneous nodules. Their classifications, while necessarily artificial, encompass the full spectrum of pathologic processes. They are delineated in the current style to facilitate the consideration required to distinguish among them and to facilitate recognize the underlying processes for which we as rheumatologists are renowned.

Ascites as primary manifestation of systemic diseases: two case reports.

Ascites as primary manifestation of systemic diseases: two case reports.

Interferon alpha promotes caspase-8 dependent ultraviolet light-mediated keratinocyte apoptosis via interferon regulatory factor 1.

Ultraviolet (UV) light is a known trigger of both cutaneous and systemic disease manifestations in lupus patients. Lupus skin has elevated expression of type I interferons (IFNs) that promote increased keratinocyte (KC) death after UV exposure. The mechanisms by which KC cell death is increased by type I IFNs are unknown. Here, we examine the specific cell death pathways that are activated in KCs by type I IFN priming and UVB exposure using a variety of pharmacological and genetic approaches. Mice that overexpress Ifnk in the epidermis were exposed to UVB light and cell death was measured. RNA-sequencing from IFN-treated KCs was analyzed to identify candidate genes for further analysis that could drive enhanced cell death responses after UVB exposure. We identify enhanced activation of caspase-8 dependent apoptosis, but not other cell death pathways, in type I IFN and UVB-exposed KCs. In vivo, overexpression of epidermal Ifnk resulted in increased apoptosis in murine skin after UVB treatment. This increase in KC apoptosis was not dependent on known death ligands but rather dependent on type I IFN-upregulation of interferon regulatory factor 1 (IRF1). These data suggest that enhanced sensitivity to UV light exhibited by lupus patients results from type I IFN priming of KCs that drives IRF1 expression resulting in caspase-8 activation and increased apoptosis after minimal exposures to UVB.

Assessment of oral manifestations of systemic health related knowledge of medical students pursuing internship programme at a tertiary health care centre

AimClinicians across the entire medical profession are responsible for detection, prevention and imparting importance of oral health to the patients. However, many studies conducted so far show lack of knowledge of medical students regarding dental conditions. The present observational study was undertaken to evaluate the knowledge of oral manifestations of systemic diseases amongst medical students undergoing Internship program at a tertiary centre. Materials & methodA self-structured Questionnaire comprising of 20 questions was used as a survey tool to assess the understanding of 100 medical students undergoing internship program. They were explained about the purpose of the study. The questionnaire consisted of an objective part Yes/No (7 questions) and subjective part designed on likert scale(13 questions). There were questions on their view on current curriculum for dentistry in medical undergraduate training and possible enhancement in teaching modalities. The subjective part had five options to choose from, Strongly agree, Agree, Neutral, Disagree And Strongly Disagree. Statistical analysisThe responses of objective questions was recorded and percentage calculated for both options. The total score was obtained for each participant based on their reponses to the subjective questions based on the Likert scale. Student t-test was applied to calculate means between male and female students. ResultsFor all objective questions, percentage was calculated for affirmative and negative responses. The responses were calculated on scale5-1 for the subjective questions. Reverse scoring was done for questions 10,11,12 i. e scale 1–5. The maximum achievable score in this category was 65 which was further divided into three category as Excellent score range 55–65, Good score range 45–54, Unsatisfactory score range 35–44. The number of students in excellent category were 11, Good 66, unsatisfactory 23. The average score obtained was 48.175. The mean scores for males was 48.0161 and for females it was 48.142. The scores were slightly better for females but the difference was statistically insignificant as (p-value = 0.455056). ConclusionThe present study showed good level of Knowledge on oral manifestations of systemic diseases among medical students as compared to other studies which forms an integral part of medical curriculum. The students responses on enhancing dental curriculum and teaching aids for improved education tools can help the faculty provide a better teaching learning experience.

Enfermedades inflamatorias de páncreas y vía biliar

Inflammatory involvement of the bile duct and pancreas can present in different ways depending on the etiology and place of involvement. These are rare diseases; the most common is autoimmune pancreatitis, which has two subtypes: type 1, which is considered the pancreatic manifestation of systemic IgG4-related disease (IgG4-RD) and type 2, which is considered a primarily pancreatic disease. The most common clinical presentation is in the form of jaundice and pancreatic masses with or without abdominal pain, which necessitates a differential diagnosis with pancreatic adenocarcinoma. Treatment is based on corticosteroids, with good response rates, but there is a non-negligible risk of recurrence which sometimes requires treatment with anti-CD20 (rituximab) or immunosuppressants. IgG4-RD can also affect the bile duct, in which case it is called IgG4-related sclerosing cholangitis, and treatment is similarly based on corticosteroids. Lastly, recurrent pyogenic cholangitis is a rare type of inflammatory disease of the bile duct characterized by the formation of recurrent intrahepatic and extrahepatic bile duct stones and recurrent episodes of cholangitis that often require endoscopic, radiological, or surgical treatment in addition to medical treatment.

Cutaneous manifestations of systemic diseases in Afro‐Caribbean skin

AbstractThere is a need for training dermatologists in skin of colour's common and less common dermatoses due to the increasing diversity of the world population. The aim of this review is to highlight on cutaneous manifestations of systemic diseases in Afro‐Caribbean people, especially sarcoidosis, dermatomyositis, systemic sclerosis, systemic lupus.

Adult-Onset Still's Disease (AOSD): Advances in Understanding Pathophysiology, Genetics and Emerging Treatment Options.

Adult-onset Still's disease (AOSD) is a multisystemic complex disorder clinically characterised by episodes of spiking fever, evanescent rash, polyarthritis or diffuse arthralgias; multiorgan involvement may develop according to the hyper-inflammatory extent. The pathogenesis of AOSD is not completely recognised. The central role of macrophage activation, which results in T helper 1 (Th1) cell cytokine activation, is well established. Pro-inflammatory cytokines such as interleukin (IL)-1, IL-6 and IL-18 play a fundamental role in disease onset and progression. The disease may develop in both children and adults with overlapping clinical features, and although several subsets depending on the clinical manifestations and the cytokines expressed have been identified, the dichotomy between systemic juvenile idiopathic arthritis (sJIA) and AOSD nowadays has been overcome, and the pathology is considered a disease continuum between ages. Various therapeutic approaches have been evaluated thus far, and different compounds are under assessment for AOSD treatment. Historically, glucocorticoids have been employed for treating systemic manifestations of Still's disease, while conventional synthetic disease modifying anti-rheumatic drugs (csDMARDs) demonstrated efficacy in controlling the articular manifestations. Currently, biological (b) DMARDs are widely employed; IL-1 inhibitors such as anakinra and canakinumab have proven to have high efficacy and an excellent safety profile and the anti-IL-6 tocilizumab is approved for sJIA, with several trials and longitudinal studies confirming its efficacy and safety. Moreover, in the light of the 'window of opportunity', new evidence showed that the earlier these treatments are initiated, the sooner clinical inactivity can be achieved. Other treatment options are being considered since several molecules involved in the disease pathophysiology can be targeted through various mechanisms. This review will provide a broad overview of AOSD pathophysiology, insights into specific organ manifestations and the currently available treatments with the identification of potential therapeutic targets involved in AOSD pathogenesis will be outlined.

Manifestations of systemic disease in the retina and fundus of cats and dogs.

The fundus is unique in that it is the only part of the body that allows for a noninvasive and uninterrupted view of vasculature and nervous tissue. Utilization of this can be a powerful tool in uncovering salient incidental findings which point to underlying systemic diseases, and for monitoring response to therapy. Retinal venules and arterioles allow the clinician to assess changes in vascular color, diameter, outline, and tortuosity. The retina and optic nerve may exhibit changes associated with increased or decreased thickness, inflammatory infiltrates, hemorrhages, and detachments. While some retinal manifestations of systemic disease may be nonspecific, others are pathognomonic, and may be the presenting sign for a systemic illness. The examination of the fundus is an essential part of the comprehensive physical examination. Systemic diseases which may present with retinal abnormalities include a variety of disease classifications, as represented by the DAMNIT-V acronym, for Degenerative/Developmental, Anomalous, Metabolic, Neoplastic, Nutritional, Inflammatory (Infectious/Immune-mediated/ischemic), Toxic, Traumatic and Vascular. This review details systemic illnesses or syndromes that have been reported to manifest in the fundus of companion animals and discusses key aspects in differentiating their underlying cause. Normal variations in retinal anatomy and morphology are also considered.

Oral manifestations of systemic disorders – part 1

Headlines Oral manifestations may be the first sign of a systemic condition or disease Clinical recognition of oral manifestations related to systemic diseases may be difficult and can delay diagnosis Oral conditions related to the treatment of systemic diseases are clinically important to recognize Oral health personnel should be educated to be able to identify and diagnose and manage oral manifestations of systemic diseases and conditions in a timely manner

Pathogenetic Bases of Systemic Morphological Manifestations of Coal Worker’s Pneumoconiosis: A Review

Introduction: Miners are at increased risk of developing pneumoconiosis that poses a serious threat to their health. Progressive pneumosclerosis and bronchial mucosa atrophy are typical pathological changes in this disease. At the same time, there is evidence of systemic manifestations of pneumoconiosis. In this regard, a deeper understanding of how long-term exposure to coal dust affects pathophysiological processes not only in the target organ, but also in the whole body is required. Objective: To generalize and systematize ideas about the pathogenetic foundations of systemic morphological manifestations of black lung disease. Materials and methods: To obtain information, we have carried out a search for relevant studies published in 1995–2022 in Scopus, PubMed, and RISC databases using the following keywords: pneumoconiosis, miners, morphological changes, pathogenesis, free radical oxidation, inflammation, immune response, cytokines, growth factors, and endothelial dysfunction. We selected 59 full-text publications presenting the results of completed studies on the mechanisms of development of systemic morphological changes in coal miner’s pneumoconiosis. The systematic review was made according to PRISMA guidelines and using certain PICO(S) criteria. Results: It has been shown that long-term exposure to coal dust causes activation of macrophage-related inflammation in the respiratory system, free radical oxidation, modulation of intracellular signaling pathways, and programmed cell death. Numerous studies have demonstrated the role of inflammation as the main pathogenetic factor of pneumoconiosis, which is closely related to oxidative stress and immune response. Inflammatory mediators ensure the immune response, regeneration and sclerosis of damaged tissues. In case of high cumulative doses of coal dust, these processes begin to have a destructive and tissue-damaging effect in both the lungs and other organs due to the systemic action of the above mediators. The key role of the endothelium in the development, course and outcome of inflammation is noted.

Spontaneous Coronary Artery Dissection (SCAD) from an Interventionalist Perspective.

Spontaneous coronary artery dissection (SCAD) is an increasingly recognized cause of acute coronary syndrome (ACS), particularly among women < 50years of age. Here, we aim to review the pathogenesis of SCAD, discuss SCAD as an initial manifestation of systemic arterial disease, and highlight invasive strategies as well as unique challenges in the care of women with SCAD. A paradigm shift has occurred in the care of SCAD patients in the past decade as recommendations for conservative management have become widespread. Invasive interventions are reserved for patients with hemodynamic compromise or active ischemia due to increased periprocedural complications and failure rates. Certain patient populations have been identified for larger territory infarcts and proximal disease including patients with known connective tissue disease, premenopausal women, and patients with pregnancy-associated SCAD (P-SCAD). Current recommended management of SCAD is conservative. Despite a growing awareness of SCAD and its known association with systemic arteriopathies in women, evidence-based data remains scarce. Future studies focused on identifying genetic factors, optimal medical therapy after SCAD, and techniques to minimize interventional complications are needed.

An Aptamer-Based Proteomic Analysis of Plasma from Cats (Felis catus) with Clinical Feline Infectious Peritonitis.

Feline infectious peritonitis (FIP) is a systemic disease manifestation of feline coronavirus (FCoV) and is the most important cause of infectious disease-related deaths in domestic cats. FIP has a variable clinical manifestation but is most often characterized by widespread vasculitis with visceral involvement and/or neurological disease that is typically fatal in the absence of antiviral therapy. Using an aptamer-based proteomics assay, we analyzed the plasma protein profiles of cats who were naturally infected with FIP (n = 19) in comparison to the plasma protein profiles of cats who were clinically healthy and negative for FCoV (n = 17) and cats who were positive for the enteric form of FCoV (n = 9). We identified 442 proteins that were significantly differentiable; in total, 219 increased and 223 decreased in FIP plasma versus clinically healthy cat plasma. Pathway enrichment and associated analyses showed that differentiable proteins were related to immune system processes, including the innate immune response, cytokine signaling, and antigen presentation, as well as apoptosis and vascular integrity. The relevance of these findings is discussed in the context of previous studies. While these results have the potential to inform diagnostic, therapeutic, and preventative investigations, they represent only a first step, and will require further validation.

Herpesviruses and SARS-CoV-2: Viral Association with Oral Inflammatory Diseases.

The oral cavity is a niche for diverse microbes, including viruses. Members of the Herpesviridae family, comprised of dsDNA viruses, as well as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), an ssRNA virus, are among the most prevalent viruses infecting the oral cavity, and they exhibit clinical manifestations unique to oral tissues. Viral infection of oral mucosal epithelia triggers an immune response that results in prolonged inflammation. The clinical and systemic disease manifestations of HHV have been researched extensively, and several recent studies have illuminated the relationship between HHV and oral inflammatory diseases. Burgeoning evidence suggests the oral manifestation of SARS-CoV-2 infection includes xerostomia, dysgeusia, periodontal disease, mucositis, and opportunistic viral and bacterial infections, collectively described as oral post-acute sequelae of COVID-19 (PASC). These diverse sequelae could be a result of intensified immune responses initially due to the copious production of proinflammatory cytokines: the so-called "cytokine storm syndrome", facilitating widespread oral and non-oral tissue damage. This review explores the interplay between HHV, SARS-CoV-2, and oral inflammatory diseases such as periodontitis, endodontic disease, and peri-implantitis. Additionally, the review discusses proper diagnostic techniques for identifying viral infection and how viral diagnostics can lead to improved overall patient health.

Persistent intracranial hyper-inflammation in ruptured cerebral aneurysm after COVID-19: case report and review of the literature

BackgroundThe systemic manifestations of coronavirus disease 2019 (COVID-19) include hyperinflammatory reactions in various organs. Recent studies showed evidence for the frequent involvement of central nervous system in affected patients; however, little is known about clinical features of cerebrovascular diseases in childhood-onset COVID-19.Case presentationA 10-year-old boy recovered from SARS-CoV-2 infection without complication. On 14 days after infection, he presented with loss of consciousness. A head computed tomography detected a ruptured cerebral aneurysm at the left posterior cerebral artery accompanying subarachnoid hemorrhage (SAH). Immediate surgical intervention did not rescue the patient, resulting in the demise 7 days after admission. Serological and genetic tests excluded the diagnosis of vasculitis and connective tissue disorders. Retrospective analysis showed markedly higher levels of interleukin (IL)-1β, IL-6 and IL-8 in the cerebrospinal fluid than the serum sample concurrently obtained. A review of literature indicated that adult patients with COVID-19 have a risk for the later development of SAH during the convalescent phase of COVID-19.ConclusionsSAH is a severe complication of COVID-19 in children and adults who have asymptomatic cerebrovascular aneurysms. The markedly high levels of cytokines detected in the cerebrospinal fluid suggested that intracranial hyperinflammatory condition might be one of the possible mechanisms involved in the rupture of a preexisting cerebrovascular aneurysms.

Predicting response to iron supplementation in patients with active inflammatory bowel disease (PRIme): a randomised trial protocol

IntroductionIron deficiency anaemia (IDA) is the most common systemic manifestation of inflammatory bowel disease (IBD) that has detrimental effects on quality of life (QoL) and disease outcomes. Iron deficiency (ID),...