Internal Organ Involvement Research Articles

Pediatric drug reaction with eosinophilia and systemic symptoms: A 12-year retrospective study in a tertiary center.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare and severe adverse drug reaction involving multiple organs. Data on DRESS syndrome among children are currently limited. The purpose of this study was to determine the clinical features, causative drugs, systemic organ involvement, laboratory findings, disease severity score, and treatment outcomes in pediatric DRESS patients. The medical records of all pediatric DRESS patients, based on the RegiSCAR diagnostic criteria and admitted to King Chulalongkorn Memorial Hospital, Bangkok, Thailand from January 2010 to December 2021, were reviewed. Twenty-two cases were identified (males 54.5%) with a median age of 9.5 years. Anticonvulsants (54.5%) and antibiotics (27.3%) were the leading culprit drugs. Skin rash was reported in all cases, followed closely by liver involvement (95.5%). Eosinophilia and atypical lymphocytosis were identified in 54.5% and 31.8% of cases, respectively. The median latency period was 17.5 days. Liver enzyme elevation was detected at an average onset of 20.0 days and hepatocellular type was the most common pattern of liver injury. Nineteen patients (86.4%) were treated with systemic corticosteroids with prednisolone being the most prescribed medication. One case developed Graves' disease after DRESS and multiple relapses of DRESS. One case (4.5%) died due to refractory status epilepticus that was unrelated to DRESS. Anticonvulsants were the major cause of DRESS in pediatric patients. High suspicion for DRESS is crucial in patients receiving these drugs and presenting with fever, rash, and internal organ involvement.

Bone Mineral Density and Fracture Risk Assessment in Patients of Systemic Sclerosis

Background: Systemic sclerosis (SSc) is a chronic disease presenting in the age group of 20-50yrs and females are more affected than males. Osteoporosis is common in many rheumatological diseases and an association has been well established in Rheumatoid Arthritis and Systemic Lupus Erythematosus. However, there is no such robust data in patients affected with SSc. Our study aimed to measure Bone Mineral Density and assess the risk of fracture in patients of systemic sclerosis using the Fracture Risk Assessment using (FRAX) algorithm. Methodology: The case control study conducted in a tertiary care hospital included 24 patients of age 18-45years with systemic sclerosis and 24 age and sex matched healthy controls. 62.5% cases were antiScl-70 antibody positive, 62.5% had interstitial lung disease and 37.5% had pulmonary arterial hypertension. The BMD of Scl-70 antibody positive patients was 0.64± 0.08 gm/sq.cm at neck of femur, 0.70± 0.08 gm/sq cm at lumbar spine and 0.37± 0.06 gm/sq. cm at forearm. A significant lower T score at neck of femur was noted in cases as compared to controls (-1.91± 10 vs -0.32± 1.06, p=0.001). The mean FRAX score value for major osteoporotic fracture in cases, was 1.46± 0.61% with maximum value of 3% and minimum value of 0.9%. Whereas, for the hip fracture mean FRAX was 0.83± 0.07% with range of 0.7% to 1.9%. A significant difference was noted in risk of major osteoporotic fractures between cases and controls (p=0.017). On multivariate regression models computed to analyse the covariates of BMD for neck of femur, lumbar spine and forearm, it was observed that serum vitamin D levels, presence of pulmonary hypertension and/or ILD were significant covariates of BMD neck of femur. Conclusion: Systemic sclerosis is associated with significantly low bone mineral density compared to controls. The bone mass was negatively correlated with involvement of internal organs and serum iPTH levels. SSc may be considered as an independent risk factor for osteoporosis and patients of SSc should be evaluated for BMD with DEXA scan to prevent osteoporotic fractures.

Nailfold Capillaroscopy Performed by Rheumatology Nurse-reliable and Delivering Significant Impact on Clinical Diagnosis Revision and Subsequent Major Medication Changes

Introduction: Nailfold Capillaroscopy (NFC) is a microscopic examination equivalent of an in vivo assessment of microcirculation. It is an invaluable examination in patients presenting with Raynaud’s phenomenon (RP) (triphasic color changes of fingers commonly on cold exposure) which might underlie a spectrum of rheumatic diseases e.g. scleroderma and mixed connective tissue diseases (CTD). Early diagnosis of these disease can allow investigation of other internal organ involvement and starting appropriate drug therapy e.g. immunosuppressants for immune-related part, and vasodilatory therapy as a symptomatic treatment. Methodology NFC was performed by Optilia[Formula: see text]Digital Video microscopy with 200x lens with LED illumination. Rheumatologists had performed NFC from 6/2020-11/2022 for existing scleroderma and mixed CTD patients. Rheumatology nurses started to perform NFC on patients with RP since 11/2022 (study period till 4/2024) after undergoing certified workshop training and having direct supervision from in-house rheumatologists. 3 rheumatology nurses had been involved in acquiring video images from NFC and 1 of them, an APN, drafted the reports which would be finally authorized by rheumatologists. Clinical data, revised diagnosis and drug changed of performed NFC patients were analyzed. Result: 56 female and 15 male with average 51 years old were performed NFC. All had abnormal NFC findings. Crucially there were accompanying diagnosis revision and subsequent major medication changes (rheumatologists’ initiation or stepping-up of immunosuppressants and vasodilatory agents): Rheumatologists verified all images and agreed on the final conclusions, except 1 (1.5%) reported as non-specifics findings by nurse and edited as late-stage scleroderma by rheumatologist. Conclusion: Rheumatology nurses are competent and reliable in performing NFC and had expanded patients’ group from known scleroderma and CTD to patients with RP in a wider scope. Waiting time was reasonably around 1 month. The significant clinical impact is apparent with the findings helping the rheumatologists to revise diagnosis timely and to adjust drug treatment accordingly.

Insights into Mefenamic Acid-Induced Allergic Reactions: A Comprehensive Clinical and Research Review

This study investigates the incidence of Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) syndrome associated with the over-the-counter non-steroidal anti-inflammatory drug (NSAID) mefenamic acid. Approximately 10% of individuals exposed to mefenamic acid experience DRESS syndrome, a severe and potentially fatal allergic reaction characterized by fever, skin rash, lymphadenopathy, hematological abnormalities, and internal organ involvement. Leukocytosis with eosinophilia (90%) and mononucleosis (40%) has been linked to certain cases, emphasizing the diversity of clinical presentations. The causative medication must be promptly discontinued upon identification of DRESS syndrome, with documented evidence indicating improved prognosis with early drug removal. Despite the severity of these reactions, the study sheds light on the alarming accessibility of mefenamic acid and other banned medications in developing nations, particularly exemplified by the Indian Pharmacopoeia Commission’s alert on mefenamic acid issued on December 7, 2023. The lack of effective law enforcement and medical awareness poses significant challenges, allowing banned drugs, including Nimesulide and Rofecoxib, to persist in the market despite being banned by the USFDA. This research underscores the critical need for global collaboration to address regulatory gaps, enhance medical awareness, and enforce stringent measures to restrict the availability of harmful medications in developing nations. Urgent attention to these issues is imperative to safeguard public health and ensure the effective implementation of drug regulations on a global scale.

Electrocardiographic Findings in Systemic Sclerosis

Abstract Systemic sclerosis (SSc) is an autoimmune connective tissue disease which affects various tissues and organs, including skin, lungs, kidneys, gastrointestinal tract and cardiovascular system. Cardiac involvement is the most commonly recognized problem and a significant cause of morbidity. Abnormal ECG is present in 25-75% of patients with SSc and is considered to be an independent redictor of mortality. It is known that the supraventricular arrhythmias are considered as more common in SSc patients, occurring in about two-thirds of the cases, and more often than ventricular tachyarrhythmias. It has been established that right bundle branch block is associated with an increased risk of mortality and that it is an independent predictor of mortality, and should be considered as a marker of the severity of the disease in SSc. The prolonged QTc interval is an independent risk factor for a sudden cardiac death reflecting the instability of repolarization and predisposing the onset of cardiac arrhythmias.The prognosis of the disease depends on the SSc subtype and the involvement of internal organs. SSc is a lifelong disease and cannot be cured, but knowing that cardiac dysfunction significantly worsens the prognosis, early detection of cardiac complications and appropriate therapy can influence its progress and improve the patients’ quality of life.

Systemic lymphangitic carcinomatosis from unknown primary cancer mimicking lung disease in a new coronavirus infection (COVID-19): a case report

lymphangitic carcinomatosis is characterized by the spread of cancer cells through pulmonary lymphatic vessels, which results in secondary interstitial inflammation. The diagnosis of lymphangitic carcinomatosis is challenging due to nonspecific clinical symptoms and radiological findings that similar to those of interstitial lung disease.The purpose of the study was to demonstrate difficulties in the diagnosis of lymphangitic carcinomatosis with a systemic dissemination from unknown primary site. Case presentation. We report on a case of lymphangitic carcinomatosis from cancer of unknown primary origin with systemic involvement of internal organs imitating pulmonary disease caused by coVid-19. patient K., 58 years old, was treated for 12 bed-days in therapy departments with the diagnosis of bilateral viral coVid-19 pneumonia. clinical, laboratory and instrumental data were nonspecific. despite treatment, heart failure and kidney disease progressed to terminal stages, leading to the death of the patient. Autopsy revealed systemic lymphangitic carcinomatosis from cancer of unknown primary origin with involvement of the lungs, heart, liver, pancreas, spleen, kidneys and adrenal glands. lymphangitic carcinomatosis mimicked interstitial lung disease in a novel coronavirus infection coVid-19. metastasis to the heart mimicked coronary disease, and metastases to the kidneys led to acute kidney failure, which along with acute respiratory and heart failure caused the death of the patient.Conclusion. lymphangitic carcinomatosis, which does not have specific clinical manifestations, is able to mimic interstitial lung diseases, including a new coronavirus infection. cancer of unknown primary and multiple visceral metastases in this case confirm the concept of the independence of different types of tumor progression.

Juvenile Scleroderma.

Localised scleroderma and systemic sclerosis are rare chronic fibrosing disorders seen in children, and are collectively referred to as Juvenile Scleroderma. Histopathology of the two forms is non-distinct but they differ in terms of vasculopathy, internal organ involvement, morbidity and mortality. Raynaud's phenomenon with digital tip ulcers is considered hallmark of systemic sclerosis. Quality of life gets greatly affected by these diseases. Early identification in the inflammatory phase of the disease, effective treatment and strict surveillance remain crucial for better outcomes. Emerging vascular and immunosuppressive strategies, coupled with efforts from scientific community to develop better biomarkers and monitoring tools, help constantly to improve survival rates.

Abstract 285: Intraventricular And Subarachnoid Hemorrhage Secondary To Arteriovenous Malformation, A Diagnostic Neurologic Manifestation Of Osler‐Weber‐Rendu Disease

Introduction Osler‐Weber‐Rendu disease, also known as hereditary hemorrhagic telangiectasia (HTT), is a developmental vascular disease characterized by multiple arteriovenous malformations (AVMs) due to genetic defects in endothelial angiogenesis pathways. HHT is a clinical diagnosis based on the fulfillment of the Curacao Diagnostic Criteria, including involvement of internal organs with AVMs, significant recurrent nosebleeds, telangiectasias, and family history of HHT. HHT has a prevalence of 1.5 to 2 people/10,000, although this is considered a falsely low representation due to missed diagnosis and variable penetrance. Autosomal dominantly inherited heterozygous mutations disrupt the TGF‐beta‐mediated pathways causing aberrant blood vessel development with fragility and AVM formation. There are two forms of HHT: (HHT1) has a higher incidence in women and typically involves pulmonary and cerebral arteriovenous malformations (AVM). Mutations in the endoglin gene are thought to be responsible for HTT1 and comprise approximately 61% of the cases. HTT2 is associated with a higher rate of liver AVMs, involves a mutation in Activin A Receptor‐Like Type 1 (ACVRL1), and is responsible for approximately 37% of cases. Other genetic mutations include SMAD4 and GDF2, which are transmitter and binding proteins and occur about 2% of the time. Methods Case report and review of literature Results Case:A 52‐year‐old woman with a large frontoparietal arteriovenous malformation originally found at the age of 17, with a history of frequent epistaxis, mucocutaneous telangiectasias and two children with epistaxis and telangiectasias. Her diagnosis of HHT was made at her third hjospitalization due to a non‐traumatic subarachnoid hemorrhage. A Chest CT angiography and a CT angiography of the intra and extracranial vasculature demonstrated an extensive right holohemispheric cerebral AVM, an unruptured 8‐mm basilar artery aneurysm, and multiple enlarged pulmonary arteries with narrow inlet and outlets concerning for pulmonary AVMs. A subsequent digital subtraction angiogram (DSA) of the brain confirmed the extensive holohemispheric AVM, unruptured basilar artery aneurism with subarachnoid hemorrhage originating from the AVM. On examination she had multiple lingual, and hard/soft palate telangiectasias and an extensive anterior pedal telangiectasia. Given the heavy cerebral AVM burden with multiple cerebral artery feeders, the AVM angiosclerosis and basilar artery aneurysm embolization were deferred. The patient proceeded under medical management alone with a plan for personal and familial genetic testing. Conclusion HHT is a rare cause of cerebral AVMs at high risk of spontaneous bleeding. Managing these AVMs can be a challenge, given the extent and location of these. However, diagnosis of HHT can inform prognosis and guide medical management due to improved prognosis for HHT‐related AVMs and aneurysms compared to non‐HHT AVMs.

The Impact of Progressive Pulmonary Fibrosis in Systemic Sclerosis-Associated Interstitial Lung Disease.

Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by immune dysregulation and progressive fibrosis, typically affecting the skin, with variable internal organ involvement. Interstitial lung disease (ILD), with a prevalence between 35 and 75%, is the leading cause of death in patients with SSc, indicating that all newly diagnosed patients should be screened for this complication. Some patients with SSc-ILD experience a progressive phenotype, which is characterized by worsening fibrosis on high-resolution computed tomography (HRCT), a decline in lung function, and premature mortality. To assess progression and guide therapeutic decisions, regular monitoring is essential and should include pulmonary function testing (PFT), symptom assessment, and repeat HRCT imaging when indicated. Multidisciplinary discussion allows a comprehensive evaluation of the available information and its consequences for management. There has been a shift in the approach to managing SSc-ILD, which includes the addition of targeted biologic and antifibrotic therapies to standard immunosuppressive therapy (particularly mycophenolate mofetil or cyclophosphamide), with autologous hematopoietic stem-cell transplantation and lung transplantation reserved for refractory cases.

Dermatomyositis: A Review of Diagnosis and Classification Criteria

Dermatomyositis is an autoimmune disorder affecting skin and muscle and one of the subgroups of idiopathic inflammatory myopathy (IIM). Dermatomyositis is more common among women than men and it may occur in adults or children. This disease is also related to malignancy and internal organ involvement. However, around 20% of cases are present without muscle damage. The presence of myositis-specific autoantibodies may help in diagnosis and predict the prognosis of the disease. Early diagnosis of the disease is foremost required for appropriate management. Furthermore, validated diagnostic and classification criteria are important in the clinical setting and studies. Keywords: autoimmune, classification criteria, dermatomyositis, diagnosis

The Laboratory Test for the Diagnosis of Idiopathic Inflammatory Myopathies

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of autoimmune muscle diseases with systemic involvement. Patients with IIM present with varying degrees of muscle disease, cutaneous manifestations, and internal organ involvement. The diagnosis of IIM is frequently delayed because of non-specific symptoms including weakness, easy fatigability, or myalgia. Laboratory tests can give clues for the diagnosis of myositis and other muscle diseases. Creatine kinase can be the screening tool for muscle diseases. About 40-60% of IIM had positive antinuclear antibody (ANA). Thus, positive ANA results increase the possibility of IIM diagnosis. In addition, the identification of myositis-related autoantibodies can help the diagnosis and classification. Myositis-specific autoantibodies (MSA) are found exclusively in IIMs and facilitate the identification of subsets of patients with relatively homogeneous clinical features. Myositis-associated antibodies are frequently found in association with other MSA; however, they may also be detected in various connective diseases.

P14 A rare adverse event of eosinophilic fasciitis secondary to immune checkpoint inhibitor therapy

P14 A rare adverse event of eosinophilic fasciitis secondary to immune checkpoint inhibitor therapy

Disseminated Kaposi’s sarcoma in patient with human immunodeficiency virus infection

Kaposis sarcoma is the most common acquired immunodeficiency syndrome-defining malignancy. With the introduction of highly active antiretroviral therapy into practice, the incidence of Kaposis sarcoma has declined significantly. However, cases associated with late diagnosis of HIV infection are still recorded. Herein, we present the clinical case of a patient with HIV infection and generalized Kaposis sarcoma with multiple lesions in the skin, mucous membranes, and organs. If the lesions in the skin and mucous membranes did not cause difficulties, distinguishing pulmonary lesions from disseminated tuberculosis and cytomegalovirus infection is difficult. Extremely severe Kaposis sarcoma with an unfavorable outcome is caused by severe immunosuppression and, apparently, an inflammatory immune reconstitution syndrome. In autopsy, in addition to the skin and oral mucosal lesions, foci in the epiglottis, larynx, trachea, lungs, colon, duodenum, peribronchial, mesenteric lymph nodes, liver, spleen, and lumbar muscles were noted. Diagnosing internal organ involvement in Kaposis sarcoma is not easy and requires the involvement of specialists in various fields.

Dystrophic calciphylaxis in panniculitis: features of the clinical picture and diagnosis

The clinical presentation of lobular panniculitis (PN) associated with calciphylaxis (CP, calcification) can vary widely and may be associated with joint and internal organs involvement, making the diagnosis of the disease difficult.Objective: to evaluate the frequency and significance of CP in patients with PN using long-term prospective follow-up.Material and methods. From 2018 to 2023, at the V.A. Nasonova Research Institute of Rheumatology 217 patients with referral diagnosis "erythema nodosum" or "panniculitis" were examined. In 19.3% of cases (9 men and 33 women aged 37 to 72 years) CP was confirmed with an average disease duration of 56.3±11.2 months. Clinical examination of patients was performed according to the standards recommended by the Russian Association of Rheumatologists. International criteria were used to confirm the diagnosis of systemic lupus erythematosus (SLE), idiopathic inflammatory myopathies (IIM), systemic sclerosis (SS), and lipodermatosclerosis (LDS). In 12 patients with indurations, pathological examination of biopsy specimens of skin and subcutaneous fatty tissue from the area of induration was performed, which allowed confirming the diagnosis of idiopathic lobular PN (ILPN) in 3 cases. Four grades of calcification were distinguished according to the size and depth of the calcifications. In addition, considering the type of radiological changes and clinical manifestations, four subtypes of CP were identified: mousse-like, stone-like, mesh-like and lamellar-like.Results and discussion. In the study group, the ratio of women to men was 3.6:1, and the mean age was 43.8±7.6 years. On clinical examination we determined, in 60% of cases CP predominantly stone-like subtype (71.4%) of first grade (47.6%), which was significantly more frequently located on the upper and/or lower extremities and/or trunk (57.1%; p=0.05). Using clinical, laboratory and instrumental data, we confirmed the development of CP in ILPN (n=3), SLE (n=3), LDS (n=21), IIM (n=5), SS (n=1), and idiopathic CP (n=9) with a mean disease duration of 8.7±2.4 years.An increase in ESR and CRP levels occurred in different diseases, while urinary syndrome was associated with SLE (66.6%) and an increase in creatinine phosphokinase with IIM. Decreased calcium and 25-hydroxyvitamin D levels and increased phosphorus and parathyroid hormone levels were found in many patients studied.Conclusion. In the absence of clear diagnostic criteria for CP in patients with PN, early diagnosis is critical for the development of an effective multidisciplinary treatment plan.

Recurrence of composite hemangioendothelioma of the kidney after surgical resection

Composite hemangioendothelioma is an extremely rare form of kidney tumor. The tumor mainly occurs in the extremities, head and neck; internal organs involvement is rarely reported. Patient, 61-year-old male, was admitted to the urology department for a left kidney tumor, which was found accidentally during an ultrasound examination. Magnetic resonance imaging showed an irregularly shaped tumor measuring 5.0 × 6.0 × 4.0 cm and located in the lower pole of the left kidney. The tumor was surgically removed with resection of the capsule of the kidney lower pole. Immunohistochemical study revealed diffuse bright expression of CD31 (clone JC70A), CD34 (clone QBEnd 10), ERG (clone ER111), FLI-1 (clone MRQ-1) in tumor cells. The index of proliferative activity Ki-67 (clone SP6) was 40 %. The morphological picture and immunophenotype of the tumor correspond to composite hemangioendothelioma of the retroperitoneal space. Magnetic resonance imaging of the retroperitoneal space on follow-up visit in 9 months visualized a tumor of the left kidney measuring 8.3 × 8.4 × 7.8 cm. Radical nephrectomy was performed. Pathology examination showed that tumor tissue was mainly represented by solid fields of the spindle cell component. In samples of the border between the tumor and fatty pararenal tissue, tumor invasion was observed up to the adjacent striated muscles, tumor growth into the tissue of the kidney gate was also found. Taking into account the morphological picture and the earlier immunohistochemical study, the removed tumor corresponds to composite hemangioendothelioma.Composite hemangioendothelioma is a tumor of low malignant potential. It is extremely rare for this tumor to affect the kidney. At the same time, in the described case, the tumor was initially located in the retroperitoneal space, with involvement of the kidney capsule, and was assessed as a benign lesion. After 9 months, there was a recurrence of the tumor localized in the kidney with damage to the elements of the renal sinus, retroperitoneal tissue, and lumbar muscles. In this case, the tumor has significant malignant potential.

Scrotal Kaposi's Sarcoma in HIV-negative Patient: A Case Report and Review of the Literature.

Background: Kaposi's sarcoma (KS) is an indolent angio-proliferative tumor proliferation with spindle cells originating from endothelial and immune cells infected with human herpes virus type 8. (HHV-8: also known as Kaposi sarcoma herpes virus [KSHV]). HHV-8 was identified as the causative agent of KS. This virus is present in 95-98% of cases with KS. Kaposi's sarcoma was first described by a Hungarian dermatologist 1872 named Moritz Kaposi.[1]
 The lesions are characterized by the proliferation of spindle cells of endothelial origin, which present different degrees of abnormal vascularization, inflammatory infiltrates, and fibrosis. Kaposi's Sarcoma (KS) is a malignancy that generally affects the skin, and can be systemic with internal organ involvement. It originates from the vascular endothelium. KS's relationship with human immunodeficiency virus (HIV) infection is well known.
 In this article, we will present a 73-year-old male patient with 3 purple scrotal lesions up to 0.5 cm in size.
 Conclusion; Kaposi's sarcoma of the scrotum in a negative patient is a rare pathology. However, in cases of scrotal lesions that last over time, a differential diagnosis should be made and Kaposi's sarcoma should be taken into consideration. Also, a screening for other accompanying lesions, especially a detailed examination of the gastrointestinal tract is important in cases of Kaposi’s sarcoma of the scrotum.

A Challenging Case of Scleroderma: Diagnosis, Treatment, and Management

Scleroderma is a relatively rare autoimmune disorder that causes progressive hardening, tightening, and scarring of the skin, as well as internal organ involvement. This case study presents a 56-year-old African American female with diffuse cutaneous scleroderma who has had recurrent complications since 2017, including earaches and fingertip infections. The patient’s medical history, clinical course, diagnosis, and treatment options, including NSAIDs, low-dose corticosteroids, and methotrexate, are discussed. Osteopathic manipulative medicine is explored as a potential symptomatic treatment for scleroderma. Early diagnosis and chronic management for scleroderma patients are emphasized to avoid severe complications such as pulmonary hypertension, which can lead to cor pulmonale and right-sided heart failure. Further research is needed on prophylactic treatments for scleroderma complications and the potential link between multiple autoimmune disorders and increased susceptibility to scleroderma. This case study provides medical professionals with a better understanding of scleroderma and the available treatment options to improve patient quality of life.

Management of Uttana vatarakta (limited cutaneous systemic sclerosis): An experience through Ayurveda management strategies

Abstract Scleroderma (systemic sclerosis) is a group of rare diseases that involve the hardening and tightening of the skin and connective tissues. Diffuse cutaneous scleroderma and Limited Cutaneous Systemic Sclerosis (LcSSC) are the two major classifications of it. LcSSc usually affects the skin and further spreads to muscles and joints, but in about 10% cases, involvement of internal organs like gastrointestinal tract and manifestations like pulmonary hypertension is seen even after 10–15 years. The prevalence of the disease is 17.6/100,000 population. As far as the management of LcSSC in conventional medicine is concerned, other than symptomatic relief; immunosuppressive drugs are given, but long-term use of such medicines suppresses the immunity of the patient and hence the patient may get affected with opportunistic infections and causes severe complications. Hence, some alternatives in this direction are being explored from other systems of medicines for the betterment of patients. The direct description of the disease scleroderma is not available in Ayurvedic literature, but based on its presentation, it can be correlated with different stages of Vatarakta (~rheumatism due to blood tissue). A 20-year-old young male patient with presentation of limited scleroderma managed with Ayurveda therapy by Virechana (~therapeutic purgation) followed by Shamana (~palliative) therapy for one and half months and obtained encouraging results, inferring that Ayurveda therapeutic protocols can be successfully adopted in cases like LcSSC.

Advances in understanding of the pathogenesis and therapeutic implications of drug reaction with eosinophilia and systemic symptoms: an updated review.

Drug reaction with eosinophilia and systemic symptoms or drug-induced hypersensitivity syndrome (DRESS/DIHS) is one type of severe cutaneous adverse reaction (SCAR). It is featured by fever, widespread skin lesions, protracted clinical course, internal organ involvement, and possibly long-term autoimmune sequelae. The presence of high-risk human leukocyte antigen (HLA) alleles, hypersensitivity reaction after culprit drug ingestion, and human herpesvirus reactivation may all contribute to its complex clinical manifestations. Some recent studies focusing on the roles of involved cytokines/chemokines and T cells co-signaling pathways in DRESS/DIHS were conducted. In addition, some predictors of disease severity and prognosis were also reported. In this review, we provided an update on the current understanding of the pathogenesis, potential biomarkers, and the relevant therapeutic rationales of DRESS/DIHS.

Character of somatic and psycho-emotional disorders in children with various types of juvenile arthritis

Rheumatic diseases in children are a source of serious medical, social, and personal problems due to the prevalence, and severity of pain syndrome, progressive course with involvement of internal organs and disorders in the psycho-emotional sphere in the pathological process. The nature of somatic and psycho-emotional disorders was studied in 70 children aged 7 years - 16 years old with various types of Juvenile Rheumatoid Arthritis (JRA). The severe course of the disease, and as a result, disability, leads to emotional instability and social maladjustment of children. The predominance of articular forms with a primary lesion of the joints of the lower extremities and their deformation was remarkable. In children with a systemic variant of JRA, kidney damage was detected in the form of urinary syndrome and tubulointerstitial nephritis. Headache was the leading clinical manifestation of autonomic disorders caused by vascular disorders.