Multisystem Inflammatory Disease Research Articles

The co-occurrence of sarcoidosis and anti-PLA2R-associated membranous nephropathy in a patient with underlying genetic susceptibility

BackgroundSarcoidosis is a multisystemic inflammatory disease, characterized by the presence of non-caseating, epithelioid granulomas. Glomerular disease in patients with sarcoidosis is rare and membranous nephropathy (MN) is cited as the most common. The association between the two diseases remained unclear. This article reported a case of co-occurrence of sarcoidosis and anti-PLA2R-associated MN, to provide a possible relationship between these two entities.Case presentationA 61-year-old Chinese Han woman with a history of sarcoidosis was admitted to our hospital for nephrotic syndrome. Her sarcoidosis was diagnosed according to the adenopathy observed on the computed tomography scan and the biopsy of lymph nodes. The MN presented with nephrotic syndrome with a PLA2R antibody titer of 357RU/ml, and the final diagnosis was based on a renal biopsy. The patient’s sarcoidosis was remitted after treatment with prednisone. One year later MN was diagnosed, and she was treated with prednisone combined with calcineurin inhibitors, based on a full dose of renin-angiotensin system (RAS) inhibitor. The patient’s sarcoidosis had been in remission while the MN was recurrent, and her renal function deteriorated to end-stage renal disease 6 years later due to discontinuation of immunosuppression. A genetic test led to the identification of the HLA-DRB1*0301 and HLA-DRB1*150 genes associated with both sarcoidosis and MN, which provides a new possible explanation of the co-occurrence of these two diseases.ConclusionThis case suggested for the first time a potential genetic connection between idiopathic MN and sarcoidosis which needs further studies in the future.

Review study of Psoriasis in Iraq

Abstracts are written in Indonesian and English. Describe the introduction, the purpose of writting, research methods, result and conclusion. Minimum 150 and maximum 250 words.. Psoriasis is a multisystem inflammatory disease with predominantly skin and joint involvement. Beyond the physical dimensions of disease, psoriasis has an extensive emotional and psychosocial effect on patients, affecting social functioning and interpersonal relationships. As a disease of systemic inflammation, psoriasis is associated with multiple comorbidities, including cardiovascular disease and malignancy. The diagnosis is primarily clinical and a skin biopsy is seldom required. Depending on the severity of disease, appropriate treatment can be initiated. For mild to moderate disease, first-line treatment involves topical therapies including corticosteroids, vitamin D3 analogues, and combination products. These topical treatments are efficacious and can be safely initiated and prescribed by primary care physicians. Patients with more severe and refractory symptoms might require further evaluation by a dermatologist for systemic therapy .Psoriasis is a chronic, noncommunicable, painful, disfiguring and disabling disease for which there is no cure and with great negative impact on patients’ quality of life . It can occur at any age. The etiology of psoriasis remains unclear. Psoriasis can also be provoked by external and internal triggers, including mild trauma, sunburn, infections, systemic drugs and stress . Psoriasis involves the skin and nails, and is associated with a number of comorbidities. Skin lesions are localized or generalized, mostly symmetrical, sharply demarcated, red papules and plaques, and usually covered with white or silver scales. Lesions cause itching, stinging and pain. Individuals with psoriasis are reported to be at increased risk of developing other serious clinical conditions such as cardiovascular and other noncommunicable diseases . Psoriasis causes great physical, emotional and social burden . Disfiguration, disability and marked loss of productivity are common challenges for people with psoriasis. Topical and systemic therapies as well as phototherapy are available.

Vogt-Koyanagi-Harada Disease Presented with Bilateral Optic Disc Edema

Vogt-Koyanagi-Harada (VKH) disease is a multisystem inflammatory disease that occurs when body tissues including melanocytes are damaged by autoimmunity. The typical symptoms of VKH are bilateral multifocal uveitis and serous retinal detachment. Our patient was misdiagnosed as idiopathic intracranial hypertension because there were no other characteristic neurological symptoms other than bilateral optic disc edema. However, intracranial pressure was normal and pleocytosis was found, so VKH disease could be diagnosed. Bilateral optic disc edema may be a initial manifestation of VKH disease.

E050 Angiotensin converting enzyme for the diagnosis of childhood sarcoidosis

Abstract Background/Aims Childhood sarcoidosis is a rare multisystem inflammatory granulomatous disease. The prevalence of sarcoidosis in children is unknown due to its rarity. A review of sarcoidosis in Denmark found an incidence in children < 15 years of 0.22-0.27 per 100 000/year. Diagnosis can be challenging given the varied presentations due to multisystem involvement. There is no one laboratory test that is diagnostic for sarcoidosis. Angiotensin converting enzyme (ACE) has been shown to be elevated in 50% of late-onset childhood sarcoidosis. Reference levels are laboratory and age dependent, and healthy children can have levels that are 40-50% higher than in adults. Our aim was to gain a better understanding of the use of ACE in clinical practice and to review its role in diagnosis and management of sarcoidosis. Methods We collated the list of all patients under 18 years who had ACE levels done at our hospital between 1/12/2021 and 30/11/2022 via clinical pathology. Electronic patient records were reviewed for all patients to gather information on demographics, and clinical symptoms. Results We had a total of 98 ACE blood tests done for 58 patients. Clinical notes and results were available for 34 of these patients with a total of 48 ACE results. Age range of patients was 3-17 years with a mean age of 12 years. 19 (56%) patients were female, 15 (44%) were male. 50% were of white ethnicity, 12% of black ethnicity, 3% Indian ethnicity, 3% Pakistani ethnicity, 3% Chinese ethnicity and 29% ethnicity not recorded. 45 were requested by in hospital specialties, 2 from other hospitals and 1 from a GP. Of those requested in hospital 73% had been requested by ophthalmology, rheumatology or dermatology, with the remainder requested by a range of other paediatric specialties. Results ranged from 11-195 U/L, with a median of 76 U/L. Hospital reference range is 13-64 U/L and 18 patients had results >64 U/L. Reference ranges for paediatric patients in literature is 29-112 U/L. 6 patients had a result>112U/L. One patient had a diagnosis of sarcoidosis. Following the initial results a further 4 years of results were analysed from 02/01/2018-30/11/2022. 10 further patients had ACE results >112U/L. Of these, none had sarcoidosis. Conclusion These results support that the diagnosis of sarcoidosis is rare. ACE results need to be interpreted being mindful that normal childhood ACE levels may be higher than the reference ranges given by hospitals, which may be based on adult ranges. There is need for more research in this area to ascertain diagnostic ACE levels and role of ACE in disease monitoring. I plan to lead a trainee multicentre retrospective project looking at the diagnosis of paediatric sarcoidosis, and within this review the role of ACE levels. Disclosure M.C. Mackay: None. S. Deepak: None. K. Warrier: None. S. Rangaraj: None.

Myocarditis in children 2024, new themes and continued questions.

While pediatric myocarditis incidence has increased since the coronavirus disease 2019 (COVID-19) pandemic, there remain questions regarding diagnosis, risk stratification, and optimal therapy. This review highlights recent publications and continued unanswered questions related to myocarditis in children. Emergence from the COVID-19 era has allowed more accurate description of the incidence and prognosis of myocarditis adjacent to COVID-19 infection and vaccine administration as well that of multi-system inflammatory disease in children (MIS-C). As cardiac magnetic resonance technology has shown increased availability and evidence in pediatric myocarditis, it is important to understand conclusions from adult imaging studies and define the use of this imaging biomarker in children. Precision medicine has begun to allow real-time molecular evaluations to help diagnose and risk-stratify cardiovascular diseases, with emerging evidence of these modalities in myocarditis. Recent information regarding COVID-19 associated myocarditis, cardiac magnetic resonance, and molecular biomarkers may help clinicians caring for children with myocarditis and identify needs for future investigations.

CINCA/NOMID is a rare autoinflammatory syndrome in rheumatological practice. Experience of diagnosis, management and therapy with interleukin-1 inhibitors

Objective: to present the experience of diagnosis, management and therapy with interleukin-1 inhibitors (iIL1) in patients with Chronic Infantile Onset Neurologic Cutaneous Articular/Neonatal Onset Multisystem Inflammatory Disease (CINCA/NOMID) according to the Russian Federal Rheumatological Center data.Material and methods. From 2007 to 2023, eight patients were included in the study (7 men) aged 10 months to 33 years, including 3 with a disease duration of more than 10 years (13, 17 and 33 years). Genetic testing was performed in all patients and mutations in the NLRP3 gene were identified in 6 cases.Results and discussion. The age of onset of the disease ranged from 0 to 6 months. The delay in diagnosis and prescription of therapy ranged from 10 months to 33 years. All patients had the classic manifestations of CINCA/NOMID, including fever, rash, central nervous system (CNS) involvement, elevated ESR and CRP levels, 6 patients had articular manifestations, 7 had ocular manifestations and 6 had sensorineural hearing loss. Amyloidosis was detected in 1 case. All patients were prescribed iIL1. Anakinra was used in 6 patients (in 5 as the first line, in 1 as the second line therapy) with a positive response; subsequently 2 of these patients were switched to canakinumab once every 4 weeks (1 patient deteriorated and was readministered anakinra). Five patients received canakinumab (3 as first-line therapy, 2 as second-line therapy), 1 patient was switched to anakinra due to insufficient CNS response. The response to iIL1 therapy was positive in all patients, but incomplete in some of them due to the severity of the manifestations and the presence of irreversible organ damage.Conclusion. Patients with CINCA/NOMID have a severe disease and a poor prognosis. In this context, early administration of iIL1 is necessary. In the case of CNS involvement, the use of anakinra is preferable, as it is characterized by better penetration of the blood-brain barrier and is therefore more effective. Later it is possible to switch the patient to canakinumab, however, to achieve a complete response, it is sometimes necessary to increase the dose of the drug and reduce the interval between doses.

Sudden Visual Loss due to Arteritic Anterior Ischaemic Optic Neuropathy: A Rare Manifestation of Eosinophilic Granulomatosis with Polyangiitis.

eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystem inflammatory disease characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of various organs. While the eye is uncommonly affected in patients with EGPA, multiple ophthalmic manifestations have been reported, which can result in serious visual impairment without timely treatment. we report the case of a 79-year-old woman with a history of asthma and nasal polyps who presented with low-grade fever, mild alteration of mental status, and fatigue. Chest X-ray revealed bilateral interstitial infiltrates. Lab tests showed elevated C-reactive protein level and eosinophilia (eosinophil count, 4.6 x109 cells/l); blood cultures and parasitological examination of stools tested negative. Four days after presentation, the patient reported sudden and severe blurring of vision in her left eye. Ophthalmological examination revealed bilateral swollen optic disc and visual field loss, more severe in the left eye. A diagnosis of EGPA complicated by arteritic anterior ischaemic optic neuropathy (A-AION) was proposed, while an alternative or concurrent diagnosis of giant cell arteritis was ruled out based on clinical picture. Immunosuppressive treatment with high-dose intravenous glucocorticoids was promptly started. The patient's visual defect did not improve; however, two months later, no worsening was registered on ophthalmic reassessment. A-AION is an infrequent but severe manifestation of EGPA, requiring prompt diagnosis and emergency-level glucocorticoid therapy to prevent any further vision loss. Disease awareness and a multidisciplinary approach are crucial to expedite diagnostic work-up and effective management of EGPA-related ocular complications. Arteritic ischaemic optical neuropathy is a potential cause of sudden and severe visual loss in eosinophilic granulomatosis with polyangiitis (EGPA) patients.Visual loss due to arteritic ischaemic optical neuropathy is rarely reversible; however, a timely glucocorticoid treatment may prevent further progression of visual impairment.Multidisciplinary approach is crucial to expedite diagnostic work-up and effective management of EGPA patients with ocular complications.

Clinical Characteristics of Cryopyrin-Associated Periodic Syndrome and Long-Term Real-World Efficacy and Tolerability of Canakinumab in Japan: Results of a Nationwide Survey.

We assess the clinical characteristics of patients with cryopyrin-associated periodic syndrome (CAPS) in Japan and evaluate the real-world efficacy and safety of interleukin-1 (IL-1) inhibitors, primarily canakinumab. Clinical information was collected retrospectively, and serum concentrations of canakinumab and cytokines were analyzed. A total of 101 patients were included, with 86 and 15 carrying heterozygous germline and somatic mosaic mutations, respectively. We identified 39 mutation types, and the common CAPS-associated symptoms corresponded with those in previous reports. Six patients (5.9% of all patients) died, with four of the deaths caused by CAPS-associated symptoms. Notably, 73.7% of patients (100%, 79.6%, and 44.4% of familial cold autoinflammatory syndrome, Muckle-Wells syndrome, and chronic infantile neurological cutaneous articular syndrome/neonatal onset multisystem inflammatory disease, respectively) achieved complete remission with canakinumab, and early therapeutic intervention was associated with better auditory outcomes. In some patients, canakinumab treatment stabilized the progression of epiphysial overgrowth and improved height gain, visual acuity, and renal function. However, 23.7% of patients did not achieve inflammatory remission with crucial deterioration of organ damage, with two dying while receiving high-dose canakinumab treatment. Serological analysis of canakinumab and cytokine concentrations revealed that the poor response was not related to canakinumab shortage. Four inflammatory nonremitters developed inflammatory bowel disease (IBD)-unclassified during canakinumab treatment. Dual biologic therapy with canakinumab and anti-tumor necrosis factor-α agents was effective for IBD- and CAPS-associated symptoms not resolved by canakinumab monotherapy. This study provides one of the largest epidemiologic data sets for CAPS. Although early initiation of anti-IL-1 treatment with canakinumab is beneficial for improving disease prognosis, some patients do not achieve remission despite a high serum concentration of canakinumab. Moreover, IBD may develop in CAPS after canakinumab treatment.

Experience with interleukin-23 inhibitor guselkumab using in patients with severe psoriasis and the impact of the disease on the psycho-emotional background

Psoriasis is a chronic immune-mediated disease that leads not only to damage to the skin and its appendages, but is also associated with concomitant systemic diseases, including damage to the musculoskeletal system, cardiovascular pathologies, kidney disease, metabolic syndrome and changes in the nervous system. Not long ago, a term was introduced to unite psoriasis and concomitant comorbid diseases – psoriatic disease. Recently, special attention has been paid to assessing the psychoemotional state and quality of life of patients with psoriasis who bear the burden of a chronic disease. It is no secret that any skin disease significantly worsens the quality and standard of life of the patient and can lead to social and professional stigma and discrimination, and a number of restrictions. Psoriatic disease is recognized as a multisystem inflammatory disease and a holistic approach to treatment is recommended, focusing on comorbidities, including mental health, psychosocial well-being and quality of life. The Dermatological Life Quality Index (DLQI) is one of the most convenient indices that allows you to assess the severity of the burden of chronic skin diseases, including psoriasis, on a person’s daily activities; a correlation was found between the severity of psoriasis, the presence of concomitant diseases, especially psoriatic arthritis and the DLQI level. DLQI is higher in patients with moderate to severe psoriasis, in patients with mild psoriasis (low PASI index), but with damage to socially significant areas of the skin (face, hands and feet, scalp, genitals, nail plates), in patients with active manifestations of psoriatic disease in the form of concomitant diseases, which complicates the choice of drug for treatment. The availability, effectiveness, and treatment regimens of modern drugs play an important role in the psycho-emotional state of patients. Today the biological drugs have been actively used in the treatment of psoriasis, which have a number of advantages compared to drugs of basic anti-inflammatory therapy and phototherapy, and, accordingly, are more effective and are of value for patients who experience emotional discomfort from a chronic skin disease in everyday life. The purpose of this article was to study the prevalence and nature of mental and psychiatric pathologies in patients with psoriasis, the possible improvement of DLQI in patients with psoriasis using a biological drug from the group of interleukin-23 inhibitors guselkumab.

Cardiac Involvement in Neurosarcoidosis: A Single-Center Investigation

Sarcoidosis is a multisystem inflammatory granulomatous disease. Among systemic sarcoidosis manifestations, cardiac or nervous system involvement can result in significant morbidity and mortality. We describe the overlapping incidence of cardiac sarcoidosis (CS) within a neurosarcoidosis (NS) cohort and determine the frequency of other nonsarcoid cardiac diseases in these patients. We performed a retrospective chart review of patients evaluated at the University of Utah from 2010 to 2022. Patients were included if they had (1) at least one instance of a diagnostic code for sarcoidosis in their medical record-International Classification of Diseases (ICD) 9 code 135 or ICD 10 code D86; (2) at least one outpatient visit in the Neurology Department within the University of Utah electronic health record with a diagnosis of definite, probable, or possible NS based on 2018 consensus criteria; (3) at least one outpatient visit in the Cardiology Department within the University of Utah electronic health record; and (4) ECG available in their medical record for review. Of 64 definite, probable, or possible patients with NS in the University of Utah cohort, 52 met our inclusion criteria and were included in this study. Of 52 patients with NS who met our inclusion criteria, 65.38% were female, with an average age of 60.9 years (range 38-84). More than half (58%) were obese (BMI ≥ 30). CS was diagnosed in 6 patients with NS (12%). Symptoms suggestive of possible cardiac dysfunction included lower extremity edema (50%), palpitations (46%), chest pain (44%), and shortness of breath (27%). ECG abnormalities included nonspecific T-wave change (40%) and right bundle branch block (17%). Three patients experienced ventricular tachycardia: sustained in one patient and nonsustained in 2 patients. Cardiac MRI was performed in 17 patients (32.7%) and in 3 patients (17.6%), which revealed diffuse myocardial enhancement suggesting CS. In this cohort, 12% of patients with NS also had confirmed CS. In addition, these patients had a high burden of cardiovascular disease not directly attributed to sarcoidosis. Our data suggest that patients with NS require comprehensive cardiac evaluation. Future studies are needed to clarify the extent of the direct contribution of granulomatous inflammation on the cardiovascular system from the indirect contribution of treatments such as glucocorticoids that lead to increased risk of cardiovascular disease in sarcoidosis.

Sarcoidosis and skin manifestations

Sarcoidosis is a multisystemic inflammatory disease of unknown etiology, affecting all organs and systems. Skin involvement is seen in one-third of cases. Cutaneous findings alone do not determine the prognosis of sarcoidosis, but they are useful in early diagnosis. There are specific and nonspecific cutaneous findings. The most common cutaneous findings are papules and plaques. This review summarizes the cutaneous manifestations of sarcoidosis.

Sarcoidosis. A review of diagnosis, clinical features and treatment

Introduction: Sarcoidosis is a progressive chronic multisystemic inflammatory disease of unclear pathogenesis, which presents with noncaseating granulomas and is complicated to diagnose due to the variable clinical presentation of patients. Sarcoidosis may attack any organ, but especially the pulmonary, cutaneous, lymphatic, ocular, hepatic and splenic systems. The most frequent presentations are prolonged cough, rash, visual problems, peripheral lymphadenopathy, tiredness, and occasional abnormality of the chest X-ray. The diagnostic evaluation of sarcoidosis is difficult and often involves a combined effort of clinical data and appropriate imaging modalities. Purpose: The purpose of this article is to review the available literature on the epidemiology, clinical manifestations and management of patients with sarcoidosis Materials and method: The available literature in PubMed was reviewed to write the article, using the keywords ,,sarcoidosis”, ,,cardiac sarcoidosis”, ,,pulmonary sarcoidosis” and ,,cutaneous sarcoidosis”. Conclusion: Sarcoidosis is associated with a significant increase in patient mortality, as well as decreased quality of life and physical disability. Efforts to accurately predict disease progression allow for more comprehensive and personalized patient care. As a life threatening disease, sarcoidosis needs to be diagnosed early and treated appropriately.

Alpha-1-antitrypsin-deficiency is associated with lower cardiovascular risk: an approach based on federated learning

BackgroundChronic obstructive pulmonary disease (COPD) is an inflammatory multisystemic disease caused by environmental exposures and/or genetic factors. Inherited alpha-1-antitrypsin deficiency (AATD) is one of the best recognized genetic factors increasing the risk for an early onset COPD with emphysema. The aim of this study was to gain a better understanding of the associations between comorbidities and specific biomarkers in COPD patients with and without AATD to enable future investigations aimed, for example, at identifying risk factors or improving care.MethodsWe focused on cardiovascular comorbidities, blood high sensitivity troponin (hs-troponin) and lipid profiles in COPD patients with and without AATD. We used clinical data from six German University Medical Centres of the MIRACUM (Medical Informatics Initiative in Research and Medicine) consortium. The codes for the international classification of diseases (ICD) were used for COPD as a main diagnosis and for comorbidities and blood laboratory data were obtained. Data analyses were based on the DataSHIELD framework.ResultsOut of 112,852 visits complete information was available for 43,057 COPD patients. According to our findings, 746 patients with AATD (1.73%) showed significantly lower total blood cholesterol levels and less cardiovascular comorbidities than non-AATD COPD patients. Moreover, after adjusting for the confounder factors, such as age, gender, and nicotine abuse, we confirmed that hs-troponin is a suitable predictor of overall mortality in COPD patients. The comorbidities associated with AATD in the current study differ from other studies, which may reflect geographic and population-based differences as well as the heterogeneous characteristics of AATD.ConclusionThe concept of MIRACUM is suitable for the analysis of a large healthcare database. This study provided evidence that COPD patients with AATD have a lower cardiovascular risk and revealed that hs-troponin is a predictor for hospital mortality in individuals with COPD.

"Extracorporeal membrane oxygenation outcomes in multisystem inflammatory syndrome of childhood - An extracorporeal life support organization registry study".

Multisystem inflammatory disease in childhood (MIS-C) is a novel pediatric syndrome after a COVID-19 infection that causes systemic injury, with potential life-threatening hemodynamic compromise requiring Extracorporeal Membrane Oxygenation (ECMO) support. We performed an observational retrospective cohort study in children aged 0-18 years with MIS-C and non-MIS-C myocarditis on ECMO between January 2020 and December 2021, using the ELSO Registry database. We aimed to compare the outcomes of both populations and to identify factors for decreased survival in MIS-C patients on ECMO. The Extracorporeal Life Support Organization (ELSO) Registry reported 310 pediatric ECMO patients with MIS-C (56.1%) and non-MIS-C myocarditis (43.9%). No difference was found in survival to hospital discharge between groups (67.2% for MIS-C vs 69.1% for non-MIS-C myocarditis, p 0.725). Multivariable analysis demonstrated that ECPR and co-infection were significantly associated with decreased survival to hospital discharge in MIS-C patients (OR 0.138, p 0.01 and OR 0.44, p 0.02, respectively). Outcomes of children with MIS-C on ECMO support are similar to those of non-MIS-C myocarditis despite higher infectious, multiorgan dysfunction and respiratory complications accompanying COVID-19 infections. The use of ECMO for MIS-C patients seems to be feasible and safe. Prospective studies on the use of ECMO support in MIS-C patients may improve outcomes in this pediatric population.

Ocular manifestations of juvenile lupus erythematosus: An 2023 update

Aims/Purpose: Juvenile Systemic Lupus Erythematosus (jSLE) is a chronic multisystem inflammatory disease that manifests before the age of 16 years, following a remitting ‐ relapsing course. The range of clinical presentations of juvenile Systemic Lupus Erythematosus (jSLE) in children is multifaceted. Ocular manifestations are uncommon for children with jSLE but can have a significant effect on their vision if not diagnosed early. Any part of the visual pathway can be affected by jSLE. The aim of this report is to systematically review the ocular manifestations of jSLE.Methods: A systematic review according to Preferred Reporting Items for Systematic Reviews and Meta‐Analyses (PRISMA) 2020 Guidelines was conducted searching MEDLINE and Scopus, including reference lists with appropriate terms for jSLE ocular manifestations. Epidemiological studies, preferably cross‐sectional studies, that describe the prevalence of ocular manifestations in children with a diagnosis or upon diagnosis of jSLE were assessed.Results: Our search yielded 131 results, screened by two independent reviewers. 33 studies were finally included in the review after preliminary assessment. Keratoconjunctivitis sicca, dry eye syndrome, retinopathy and optic neuritis are among the conditions that have been reported. Ocular manifestations in jSLE may be the result of three different mechanisms: the disease itself, infections related to immunosuppression and side effects of drug therapy. Retinal involvement is one of the most important manifestations, implying high possibility of visual loss. It is usually caused by immune complexes and autoantibodies deposition to the retinal vessels or hypertension resulting from kidney disease.Conclusions: Ocular manifestations can occur early in the course of jSLE, sometimes even before other systemic symptoms and can serve as markers of disease activity. Diagnosing ocular manifestations in children with jSLE is important for early intervention, monitoring disease activity, preventing vision loss and providing comprehensive care. By addressing ocular manifestations alongside systemic lupus management, healthcare providers can optimize outcomes and improve the overall well‐being of children with jSLE.

Post-COVID-19 Menstrual Abnormalities among Medical Students at a Tertiary Care Medical College in Southern India: A Cross-sectional Study

Introduction: The emergence of Coronavirus Disease-2019 (COVID-19) caught the world by surprise, leading to a widespread global pandemic that has had profound and distressing effects on various levels. Its impact has been far-reaching, causing severe consequences that continue to unfold. COVID-19 is a multisystem inflammatory disease involving all organs, including the female reproductive system. Aim: To determine the prevalence of COVID-19 among students and compare post-COVID-19 menstrual abnormalities in the home and hospital groups. Materials and Methods: A cross-sectional study was conducted to assess post-COVID-19 menstrual abnormalities among medical students at Department of Obstetrics and Gynaecology, NRI Institute of Medical Sciences, Visakhapatnam, Andhra Pradesh, in southern India from April to December 2022. A total of 600 medical students were approached, and 549 responded, among whom 258 students were reported as COVID-19 positive and 291 reported as COVID-19 negative. After excluding risk factors, 204 COVID-19 positive students were selected as the study population. Data were collected using a prevalidated questionnaire. The COVID-19 study population of 204 (100%) was further stratified into two groups based on the treatment taken at home 162 (79.4%) or in the hospital 42 (20.5%). Chi-square test and Fisher’s-exact tests were used to compare post-COVID-19 menstrual abnormalities, including cycle length abnormalities, Heavy Menstrual Bleeding (HMB), dysmenorrhoea, Premenstrual Syndrome (PMS), and hypomenorrhoea. Results: The prevalence of COVID-19 in the study population was 204, accounting for 37.15% of the total population 204/549. Out of the 204 students, 162 (79.4%) received home treatment, and 42 (20.5%) received hospital treatment. PostCOVID-19 menstrual cycle abnormalities were more prevalent in the hospital group 26/42 (61.9%) than in the home treatment group 76/162 (46.9%). The most common abnormality in both groups was PMS, with a prevalence of 21 (50%) in the hospital group and 38 (23.4%) in the home group. In the home group, 37 (22.8%) experienced longer cycles, 33 (20.3%) had dysmenorrhoea, and 19 (11.7%) had shorter cycles. In the hospital group, 14 (33.3%) had longer cycles, 13 (30.9%) experienced dysmenorrhoea, and 9 (21.4%) had shorter cycles. HMB was reported by 6 (14.2%) in the hospital group and 13 (8%) in the home group, while hypomenorrhoea occurred in 11 (6.7%) in the home group and 5 (11.9%) in the hospital group. A significant association (p=0.001) was found between the COVID-19 treatment groups and PMS. However, for other post-COVID-19 menstrual abnormalities, such as cycle length, dysmenorrhoea, HMB, and hypomenorrhoea, there was no significant association between these abnormalities and COVID-19 treatment groups. Conclusion: Post-COVID-19 menstrual abnormalities were more frequently observed in the hospital group compared to the home group. All post-COVID-19 menstrual irregularities resolved within six months, except for PMS.

Neutrophil lymphocyte ratio and platelet lymphocyte ratio as a marker for disease severity in children with COVID-19

Background: It had been observed that there is relationship of neutrophil lymphocyte ratio (NLR) and platelet lymphocyte ratio (PLR) with disease severity in children with COVID-19 and post-COVID, multisystem inflammatory disease in children (MIS-C). Study conducted to determine NLR and PLR correlation with this disease severity. Methods: Prospective descriptive analytical study conducted at the children’s hospital and university of child health sciences, Lahore. Consecutive confirmed cases of COVID-19 infection and post-COVID, MIS-C diagnosed on the basis of RT-PCR from nasopharyngeal swab and antibody test admitted from March 2020 to October 2021 were included through consecutive sampling. Complete blood (CBC) was done and NLR and PLR was determined Results: Majority 198 (74.2%) of the 267 patients had a diagnosis of COVID-19 and 69 (25.8%) were post-COVID MIS-C. There was a male preponderance 177 (66.3%) and the mean age was 6.1±4.7 years (95% CI: 5.62-6.74). Majority children had mild disease 80 (30%), 34 (12.7%) were critical and there were 37 (13.8%) deaths. Underlying comorbidity was present in 55 (20.6%). As the severity of symptoms changed from asymptomatic to severe disease, there was a significant rise of mean NLR from 1.88±1.40 to 5.47±4.77 respectively (p<0.001). PLR however, failed to show any kind of association with severity of the symptoms (p=0.922). Conclusions: NLR served as a marker of disease severity among pediatric patients suffering from COVID-19 and MIS-C. However, PLR failed to show any relation with disease severity.

An unusual case of sarcoidosis presenting as calf pain.

Sarcoidosis is a multisystem chronic inflammatory disease predominantly affecting the lungs. Myositis as a presenting manifestation of sarcoidosis is extremely uncommon and seldom reported. Here we report a 20-year-old male who presented with bilateral calf pain for six months. On evaluation magnetic resonance imaging showed features of myositis, and muscle biopsy was suggestive of sarcoidosis with granulomatous vasculitis. Positron emission tomography-computed tomography scan revealed involvement of spleen in addition to the muscles. Patient was managed with corticosteroids and azathioprine, and showed good treatment response.

A case of cryopyrin-associated periodic syndrome due to somatic mosaic mutation complicated with recurrent circinate erythematous psoriasis.

Cryopyrin-associated periotic syndrome (CAPS) is a rare autoinflammatory disease (AID) caused by genetic variants in innate immunity genes. AIDs, including CAPS, mediate proinflammatory cytokines such as interleukin (IL)-1 and IL-18 and result in severe systemic inflammation. A gain-of-function mutation in the NLRP3 gene, which encodes the protein cryopyrin, was identified to be responsible for CAPS in 2001, and since then several additional pathogenic mutations have been found. Moreover, other phenotypes have been identified based on severity and symptomatology, including familial cold autoinflammatory syndrome, Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease (NOMID)/chronic neurologic cutaneous articular syndrome (CINCA). Prompt diagnosis of CAPS remains challenging, however, due to unspecific, extensive clinical signs, and delayed diagnosis and treatment targeting IL-1 lead to multiorgan damage. Another factor complicating diagnosis is the existence of somatic mosaic mutations in the NLRP3 gene in some cases, resulting in symptoms and clinical courses that are atypical. The frequency of somatic mosaic mutations in CAPS was estimated to be 19% in a systematic review. Psoriasis is a chronic inflammatory skin disease that affects about 3% of the global population. Although no reports have shown complication between CAPS and psoriasis, these diseases have several similarities and potential relationships, for instance activation of Th17 cells in the dermis and increased NLRP3 gene expression in psoriatic skin compared with normal skin. Here we report a case of CAPS due to a somatic mosaic mutation with recurrent circinate erythematous psoriasis.

Surgical treatment of Behcet's disease with severe aortic regurgitation.

Behcet's disease (BD) is a multisystem inflammatory disease that is characterized by oral aphthosis, genital aphthosis, ocular lesions, and cutaneous lesions. Although BD rarely affects the cardiovascular system, its symptoms can be shown as aortic regurgitation (AR), which requires surgical intervention. Due to the special pathogenesis of BD, a low preoperative diagnosis rate and a high incidence of serious complications, such as perivalvular leakage, valve detachment, and pseudoaneurysm after prosthetic valve replacement, surgical treatment of BD with severe AR has a poor prognosis. In recent years, new surgical strategies have been developed to improve treatment efficacy for this disease. This article reviews and summarizes the evolution of surgical techniques for BD with AR and aims to provide a reference for optimizing surgical strategies, improving perioperative management, and assisting prognosis in patients suffering from BD with severe AR.