540 results match your criteria: "Dermatologic Manifestations of Renal Disease"

Factors associated with early clinical remission in patients with idiopathic retroperitoneal fibrosis.

Rheumatology (Oxford)

December 2024

Department of Rheumatology and Clinical Immunology, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), The Ministry of Education Key Laboratory, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.

Objectives: Idiopathic retroperitoneal fibrosis (IRF) is a rare autoimmune-mediated condition characterized by fibro-inflammatory tissue development around the abdominal aorta and iliac arteries. Ureteral entrapment and hydronephrosis are the most common manifestations and acute renal failure or chronic renal insufficiency may occur as the consequence. Glucocorticoids and immunosuppressants may be effective but the therapeutic response and outcome are heterogeneous and hard to predict.

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Itch, also known as pruritus, is one of the most prevalent symptoms observed in dermatological practices. Itch frequently arises from primary pruritic dermatoses, although it may also manifest in the absence of a primary pruritic skin rash. The latter itchy condition is referred to as "cutaneous pruritus" in the Japanese guidelines published in 2020.

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Background: Cutaneous manifestations are frequently observed in end-stage kidney disease (ESKD) and affect the quality of life (QoL) of patients undergoing maintenance hemodialysis (HD). Even patients undergoing HD who frequently visit hospitals do not receive active dermatological care. The aims of this study were to assess the cutaneous manifestations of patients undergoing HD and investigate changes in their QoL before and after active treatment by dermatologists.

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Article Synopsis
  • - *Hypereosinophilic syndrome (HES) is a rare blood disorder that leads to high levels of eosinophils, which can cause damage to various tissues, often discovered incidentally due to its asymptomatic onset.* - *A clinical case involving a 69-year-old male with chronic conditions showed symptoms like skin lesions and blood abnormalities, leading to a diagnosis of idiopathic HES after further testing confirmed eosinophilic infiltration.* - *Eosinophilic disorders can have various clinical signs, especially skin-related issues, and are linked to chronic kidney disease; hence, early diagnosis and treatment are essential for better patient outcomes.*
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  • - Cholangiocarcinomas (CCAs) are complex malignant tumors from the biliary epithelium, with increasing global incidence and various risk factors such as chronic liver diseases, lifestyle choices, and genetic factors
  • - The review highlights the endocrine features related to CCAs, including associations with conditions like obesity and diabetes, alongside various paraneoplastic syndromes that manifest in patients
  • - Humoral hypercalcemia is noted as the most common paraneoplastic syndrome in CCAs, signaling poor prognosis and requiring treatment beyond the typical cancer therapy
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  • X-linked agammaglobulinemia (XLA) is a condition where individuals have recurrent infections and low levels of antibodies due to a lack of B cells, and while it's rare, renal issues can occur.
  • This article presents two cases of twin brothers with XLA who experienced respiratory infections and renal complications, with varying treatment responses due to financial constraints.
  • The study emphasizes the importance of genetic testing and immune profiling in diagnosing XLA in boys with recurring infections, as well as the need for regular monitoring for kidney issues in these patients.
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  • * A study conducted at two hospitals identified that younger patients (under 25 years), those with initial renal manifestations, and those on low-dose prednisolone are more likely to experience flare-ups.
  • * The research highlighted a strong gender bias, with 95% of the SLE patients being female, and most of the clinical symptoms included musculoskeletal, dermatologic, and constitutional issues.
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  • * It highlights the phenomenon of "long COVID," which involves persistent symptoms that can affect physical health, cognitive abilities, and mental well-being, presenting challenges for healthcare providers.
  • * The discussion includes ongoing research aimed at understanding long COVID's causes and exploring treatment options like medication, physical therapy, and psychological support, stressing the importance of a comprehensive strategy in addressing these complex health issues.
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Introduction: Dermatologic manifestations of diseases in solid organ transplant recipients are common due to long-term immunosuppression.

Case Presentation: We present the case of a 63-year-old man with a kidney transplant who exhibited subcutaneous nodules on lower extremities, cytopenia, and asymptomatic pulmonary infiltrate. Through a skin biopsy and 16S ribosomal RNA (rRNA) sequencing, was identified.

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  • Acquired reactive perforating collagenosis (ARPC) is a rare skin condition where abnormal collagen fibers penetrate the outer skin layer and is often linked to health issues like diabetes or kidney disease.
  • A case study presented a 67-year-old man who developed ARPC after experiencing trauma, despite having no other underlying health changes.
  • Diagnosis involved various tests that confirmed ARPC with eosinophilia, highlighting the need for healthcare providers to consider trauma as a potential trigger when diagnosing this rare condition.
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  • This review examines the links between generalized pustular psoriasis (GPP) and various systemic diseases, indicating that GPP has far-reaching health impacts beyond skin issues.
  • It emphasizes the role of specific cytokines, like IL-17 and IL-36, in GPP's pathophysiology and their potential contributions to complications in cardiovascular, liver, kidney, lung, and skeletal health.
  • The findings suggest a need for a holistic treatment approach that considers both skin and systemic health challenges associated with GPP to improve patient care and outcomes.
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Glomerulonephritis in primary biliary cholangitis in China: an important complication.

Clin Rheumatol

August 2024

Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No.1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China.

Article Synopsis
  • The study investigates the relationship between primary biliary cholangitis (PBC) and glomerulonephritis, finding that glomerulonephritis occurs in about 3% of PBC patients.
  • Out of 330 PBC patients studied, various types of glomerulonephritis were identified, including membranous nephropathy, which was the most common.
  • The research highlights the importance of screening for PBC in patients with glomerulonephritis to prevent serious complications like cirrhosis, as PBC can often be asymptomatic at first.
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  • Systemic lupus erythematosus (SLE) is an autoimmune disease that predominantly affects women, with ongoing research into why this gender disparity exists and how ethnicity may affect the disease's clinical features.
  • A study of 89 childhood-onset SLE patients revealed that males had more instances of serositis and elevated anti-DNA antibodies, while females experienced more hair loss and lower average hemoglobin levels.
  • Despite differences in treatment, renal involvement appeared more commonly in males, indicating a need for further exploration of gender-specific manifestations and treatments in cSLE cases.
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The additional role of anti-nucleosome antibodies in the prediction of renal damage in systemic lupus erythematosus based on CSTAR (XXV).

Lupus

August 2024

Department of Rheumatology, Peking Union Medical College Hospital (PUMCH), Peking Union Medical College & Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.

Article Synopsis
  • This study investigates how anti-nucleosome antibodies impact organ damage in patients with Systemic Lupus Erythematosus (SLE), highlighting their importance alongside anti-dsDNA antibodies.
  • Among 2,481 SLE patients, a significant percentage showed positive anti-nucleosome antibodies, with higher rates of lupus nephritis and new organ damage over an average follow-up of 4.31 years.
  • The findings suggest that both antibody types are independent predictors of renal damage, and their combination may enhance the accuracy of predicting organ damage in SLE patients.
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Article Synopsis
  • * There are two main types of scleroderma: limited cutaneous SSc and diffuse cutaneous SSc, both of which can lead to serious issues in vital organs including respiratory, cardiac, renal, and ocular systems.
  • * Ocular complications from scleroderma can affect both the front (anterior) and back (posterior) of the eye, causing issues like dry eye syndrome, eyelid changes, and abnormalities in the retinal blood vessels and optic nerve.
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Objectives: To analyse the new evidence (2018-2022) for the management of systemic lupus erythematosus (SLE) to inform the 2023 update of the European League Against Rheumatism (EULAR) recommendations.

Methods: Systematic literature reviews were performed in the Medline and the Cochrane Library databases capturing publications from 1 January 2018 through 31 December 2022, according to the EULAR standardised operating procedures. The research questions focused on five different domains, namely the benefit/harm of SLE treatments, the benefits from the attainment of remission/low disease activity, the risk/benefit from treatment tapering/withdrawal, the management of SLE with antiphospholipid syndrome and the safety of immunisations against varicella zoster virus and SARS-CoV2 infection.

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Article Synopsis
  • Lupus nephritis (LN) is a serious condition related to systemic lupus erythematosus, with up to 30% of patients progressing to end-stage kidney disease (ESKD), making kidney function preservation critical through early treatment.
  • Voclosporin, a recently approved calcineurin inhibitor, has shown promising results in studies AURORA 1 and AURORA 2, demonstrating significant reductions in proteinuria and long-term efficacy in preserving kidney function among LN patients.
  • The Institute for Clinical and Economic Review (ICER) assessed the cost-effectiveness of voclosporin, finding it to be cost-effective at around $149,260 per quality-adjusted life-year (QALY), highlighting the
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Unexpected discovery of renal mass in a patient with granulomatosis with polyangiitis: accidental or inevitable?

Clin Exp Rheumatol

April 2024

Department of Rheumatology and Clinical Immunology, Chinese Academy of Medical Sciences & Peking Union Medical College; National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology; State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital (PUMCH); Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.

Article Synopsis
  • Granulomatosis with polyangiitis (GPA) is a rare autoimmune disorder affecting mainly the respiratory system and kidneys, causing symptoms like sinusitis and rapidly progressive kidney disease.
  • The disorder can also impact other body parts, including skin, eyes, heart, joints, and the nervous system.
  • The text discusses two hospital cases featuring atypical symptoms like fever and renal masses, outlining the clinical progression and supporting literature on these unusual manifestations.
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  • * An online survey completed by 280 adults with FD revealed common symptoms like fatigue, pain, and hearing issues, with many finding their symptoms bothersome and difficult to manage.
  • * Despite receiving treatment, nearly half of respondents experienced temporary symptom worsening and only a fraction communicated these changes to their doctors, with some receiving adjustments to their treatment as a result.
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Challenges of siblings with tuberous sclerosis showing various manifestations and severe complications.

Radiol Case Rep

June 2024

Department of Pediatrics, Fatmawati General Hospital, Jl. RS. Fatmawati Raya, Num4, RT.4/RW.9, West Cilandak, District Cilandak, South Jakarta, Special Capital District of Jakarta. Postal Code: 12430.

Article Synopsis
  • Tuberous Sclerosis Complex (TSC) is a rare genetic disorder affecting the central nervous system and other organs, with varying clinical presentations as shown in a case series of two siblings diagnosed with TSC.
  • The first case details a 13-year-old girl with renal angiolipomas and brain tubers, while the second case describes her 6-year-old brother with a subependymal giant cell astrocytoma and renal abnormalities.
  • The study emphasizes the importance of early diagnosis and intervention, dermatological screening, multiple imaging techniques, and family counseling to manage TSC effectively and prevent complications that could negatively impact quality of life.
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Minocycline is a tetracycline commonly used for several dermatological diseases. Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare but severe adverse event which can be caused by minocycline. An 18-year-old male patient developed fever, acute rash, pharyngeal pain, lymphadenopathy, hematologic abnormalities, increased creatinine level, elevated liver enzyme levels, and splenomegaly 4 weeks after the oral treatment of minocycline, 100 mg daily, for acne.

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