IgG4 related lung disease- a rare and novel mimic of malignancy and infections-a case series of three patients with a brief review of updated literature.

IgG4-related lung disease is an extremely rare and novel entity which is still poorly understood. We reviewed the 16 patients diagnosed with IgG4-related disease from October 2014 through December 2019 at our institution. The three cases that showed pulmonary involvement are included in this series. Of these, two patients had cavitary lung disease and developed aspergilloma and chronic cavitating aspergillosis after a prolonged course of steroid therapy, and one had isolated pulmonary nodule and ground glass opacity. We reviewed the updated literature and briefly described disease epidemiology, clinical characteristics, diagnostic approaches, and management strategies for IgG4-related lung disease.

A Novel Multi-faceted Course Blueprint to Support Outcome-based Holistic Surgical Education: The Integrated Generation 4 Model (iG4).

BACKGROUND/AIM: We present a novel multi-faceted, internationally adaptable course curriculum blueprint, which provides holistic surgical education at the undergraduate level. MATERIALS AND METHODS: The Integrated Generation 4 (iG4) course (Essential Skills in the Management of Surgical Cases - ESMSC Marathon course) curriculum consists of four essential learning components: core skills-based learning, case-based discussions, basic science workshops and soft-skills. These are all clustered in a specialty-led network architecture. Every cluster consists of modules from the four learning cores, while network nodes are modules that are mutually shared by more than one clusters. RESULTS: We produced a standardized blueprint of 50 modules based on the 4 learning cores, covering 9 surgical specialties. This resulted in a curriculum map where every module is described using 3 parameters: χ axis (skills component), y axis (knowledge component), z axis (specialty component). CONCLUSION: iG4 proof of concept sets the ground for a novel, reproducible and standardised effort to produce a portfolio of undergraduate surgical skills serving the vision of holistic surgical education.

Retroperitoneal fibrosis: A literature review. / Fibrosis retroperitoneal. Revisión de la literatura.

OBJECTIVES: Retroperitoneal fibrosis (RPF) is an uncommon disease due an inflammatory condition and deposit of fibrotic tissue that involves the retroperitoneal area over the lower four lumbar vertebrae. Very few epidemiologic studies exist that accurately characterize the incidence and prevalence of the disease. MATERIALS AND METHODS: A review of the English language literature was performed using the MEDLINE combining the keywords: "retroperitoneal fibrosis", "Ormond´ disease", "IG4 related disease". Additionally, hand search of bibliographies of included studies and previous reviews was also performed to include additional information. RESULTS: RPF develops insidiously, because the initial symptoms are non-specific. Pain is the most common presenting symptom. Various radiological diagnostic methods are used in the diagnosis of retroperitoneal fibrosis. Contrast-enhanced computerized tomography (CT) is a useful method for diagnosing retroperitoneal pathologies. Magnetic resonance imaging (MRI) is an important radiological method especially in the diagnosis of fibrotic tissue and in the examination of the retroperitoneal organ relation with fibrous tissue. Nuclear imaging is also a method used in renal function evaluation and patient follow-up. Various medical and surgical treatments would be used in the treatment of retroperitoneal fibrosis. In general, immunosuppressive agents such as corticosteroids, tamoxifen, azothiopurine, cyclophosphamide, cyclosporine, progesterone, mycophenolate mofetil are used in medical treatment. Surgical treatment methods are recommended in cases where medical treatment is not efficient. CONCLUSION: Unfortunately, despite a recent surge in the number of publications on this topic, a few progress has been made in our understanding of the classification, pathophysiology, and, most importantly, the most appropriate treatment for this disease.

OBJETIVO: La fibrosis retroperitoneal (FPR) es una enfermedad poco común debido a una afección inflamatoria y depósito de tejido fibrótico que afecta el área retroperitoneal sobre las cuatro vértebras lumbares inferiores. Existen muy pocos estudios epidemiológicos que caractericen con precisión la incidencia y la prevalencia de la enfermedad. El objetivo de esta revisión es ofrecer una visión general de las características clínicas, los métodos de diagnóstico, los tratamientos y su eficacia en los pacientes con FPR.MATERIALES Y MÉTODOS: Se realizó una revisión de la literatura en idioma inglés utilizando las bases de datos MEDLINE, combinando las palabras clave: "fibrosis retroperitoneal", "enfermedad de Ormond", "enfermedad relacionada con IG4". Además, la búsqueda manual de bibliografías de estudios incluidos y revisiones anteriores también se realizó para incluir información adicional. RESULTADOS: La FRP se desarrolla insidiosamente, ya que los síntomas iniciales son inespecíficos. El dolor es el síntoma de presentación más común. Varios métodos de diagnóstico radiológico se utilizan en el diagnóstico de la fibrosis retroperitoneal. La tomografía computarizada (TC) con contraste es un método útil para diagnosticar patologías retroperitoneales. La resonancia magnética (RM) es un método radiológico importante, especialmente en el diagnóstico de tejido fibrótico y en la valoración de la relación del órgano retroperitoneal con el tejido fibroso. La imagen nuclear también es un método utilizado en la evaluación de la función renal y el seguimiento de los pacientes. Varios tratamientos médicos y quirúrgicos se usarían en el tratamiento de la fibrosis retroperitoneal. En general, los agentes inmunosupresores como los corticosteroides, el tamoxifeno, la azatioprina, la ciclofosfamida, la ciclosporina, la progesterona y el micofenolato mofetilo se usan en el tratamiento médico. Los métodos de tratamiento quirúrgico se recomiendan en casos donde el tratamiento médico no es eficiente.CONCLUSIÓN: Desafortunadamente, a pesar de un aumento reciente en el número de publicaciones sobre este tema, se ha avanzado poco en nuestra comprensión de la clasificación, la fisiopatología y, lo que es más importante, el tratamiento más adecuado para esta enfermedad.

Intracranial Inflammatory Pseudotumor Associated with Idiopathic Hypertrophic Pachymeningitis Mimicking Malignant Tumor or High-Grade Meningioma.

BACKGROUND: Idiopathic hypertrophic pachymeningitis (IHP) is a rare clinical disease characterized by inflammatory fibrosis, which causes diffuse thickening of the dura mater. The inflammatory fibrosis will be locally invasive in nature but will be characterized by a benign histological appearance, known as an inflammatory pseudotumor. We present the case of a patient with an infiltrative lesion involving the right frontal convexity diagnosed as IHP, which had been suspected to be a malignant tumor or high-grade meningioma from the preoperative radiological findings. CASE DESCRIPTION: A 59-year-old man was admitted to our hospital because of a temporary loss of consciousness. Contrast-enhanced magnetic resonance imaging (MRI) of the brain showed a 30-mm × 12-mm mass located in the right frontal convexity. The lesion had an isointensity to high-intensity signal with moderate periregional edema on T2-weighted MRI, and homogeneous enhancement, including a necrotic portion with a long dural tail along the right frontal convexity, after contrast-enhanced MRI. Thus, our preliminary diagnosis was high-grade meningioma or a malignant tumor. We decided to surgically remove the tumor. Intraoperatively, the lesion appeared as a dural-based yellowish mass with partial infiltration of the cortex. Histopathological examination of the lesion revealed thickened meninges with marked fibroinflammatory changes. The inflammatory changes extended into the underlying brain parenchyma and were centered in the perivascular spaces. The lesion showed abundant lymphoplasmacytic infiltration with fibrosis. Immunohistochemistry revealed mixed T and B lymphocytes and plasma cells. Only a small number of IgG4-positive cells were identified. From these findings, we finally concluded that the diagnosis was IHP. The patient did not receive any further steroid therapy, because the patient had no evidence of systemic autoimmune disease. A follow-up brain MRI scan was performed 6 month after surgery, which revealed no recurrence of the lesion. CONCLUSIONS: Surgical treatment can be the first treatment option when the lesion is not localized to a critical portion of the brain. Thus, it might be possible to arrive at a definitive diagnosis histologically and determine additional treatment strategies. Also, if the surgeons are confident that the IHP has been removed completely, additional steroid therapy might not be necessary.

IgG4-Related disease simulating paraneoplastic syndrome: Role of 18FDG PET/CT imaging.

Immunoglobulin G4 (IgG4)-related systemic disease (IgG4-RSD) is a new systemic entity associated with autoimmune pancreatitis (AIP). Other organ involvements take the form of sclerosing cholangitis, sclerosing cholecystitis, sclerosing sialadenitis, retroperitoneal fibrosis, and interstitial nephritis. Recently, lung diseases related to IgG4 have been described to occur with or without other organ involvement. These diseases include interstitial lung disease (ILD), pulmonary inflammatory pseudotumor, and lymphomatoid granulomatosis. Most of these cases occur in combination with AIP, which also appears to have a general preponderance for males. The true incidence of IgG4-related ILD and the incidence of AIP are unknown. Here, we describe a case of a 53-year-old gentleman who presented with weight loss, fever, loose motions, altered sensorium, and persistent low hemoglobin, for which he was referred for 18-fluorodeoxyglucose positron emission tomography/computed tomography scan to diagnose probable underlying occult malignancy/paraneoplastic syndrome. It revealed features suggestive of IgG4 disease involving the pancreas and lungs, which was confirmed subsequently.