Immune-Mediated Necrotizing Myopathies: Current Landscape.

PURPOSE OF REVIEW: Immune-mediated necrotizing myopathy (IMNM), characterized by acute or subacute onset, severe weakness, and elevated creatine kinase levels, poses diagnostic and therapeutic challenges. This article provides a succinct overview of IMNM, including clinical features, diagnostic strategies, and treatment approaches. RECENT FINDINGS: Recent insights highlight the different clinical presentations and therapeutic options of IMNM stratified by autoantibody positivity and type. Additionally, recent findings call into question the reported link between statin use and IMNM. This review synthesizes current knowledge on IMNM, emphasizing its distinct clinical features and challenging management. The evolving understanding of IMNM underscores the need for a comprehensive diagnostic approach that utilizes a growing range of modalities. Early and aggressive immunomodulatory therapy remains pivotal. Ongoing research aims to refine diagnostic tools and therapeutic interventions for this challenging muscle disorder, underscoring the importance of advancing our understanding to enhance patient outcomes.

[Recurrent lymphadenitis was Kikuchi-Fujimoto disease]. / Lymfadenit som mystiskt återkom var Kikuchi­Fujimotos sjukdom.

Kikuchi-Fujimoto disease, or histiocytic necrotizing lymphadenitis, is in most cases a benign disease which affects lymph nodes in the cervical region. Cervical adenopathy and fever are the most common symptoms, and young adults are mostly affected. Lymph node biopsy is the mode of diagnosis with demonstration of paracortical areas of apoptotic necrosis with abundant karyorrhectic debris and a proliferation of histiocytes, plasmacytoid dendritic cells, and CD8+ T cells in the absence of neutrophils. In most cases, the disease is self-limiting but it can be recurrent or evolve to SLE. Treatment varies from symptomatic to more systemic with cortisone and intravenous immunoglobulin.

Skin necrosis after intradermal injection of lyophilized exosome: A case report and a review of the literature.

BACKGROUND: Exosomes have gained attention for their potential in skin rejuvenation. Currently, most exosome products are available for topical administration, and the use of subdermal injection as a route of administration has not been approved. AIMS: The purpose of this case report is to describe a case of skin necrosis that occurred following an intradermal injection of lyophilized exosomes. MATERIALS AND METHODS: We hereby report a case of a middle-aged man who experienced adverse effects after receiving an intradermal injection of lyophilized exosomes. Multiple injections of an exosome product were administered to treat enlarged facial pores. Shortly after the injection, the patient felt pain and noticed several dark red bumps. Three days after injection, the lesions transformed into palpable, painful, non-blanchable purplish papules and nodules, accompanied by central, tiny crusted erosions. The residual product was injected into the upper arm using an intradermal method. Similar lesions also appeared, and a skin biopsy showed necrotic keratinocytes, leukocytoclastic vasculitis, and eccrine necrosis. RESULTS: There are few reports available regarding complications, especially those related to intradermal exosomes. These complications include multiple foreign-body granulomatous reactions at the injection sites. In our case, oral prednisolone was administered for a duration of 7 days. After the treatment, the lesions exhibited notable improvement, eventually leaving post-inflammatory hyperpigmentation. CONCLUSION: Utilizing exosomes through unapproved methods should be avoided due to the possibility of adverse reactions that could cause aesthetic issues.

Imaging diagnosis and differential diagnosis of extraskeletal osteosarcoma.

OBJECTIVE: The aim of this study was to investigate the clinical, imaging and pathological features of extraskeletal osteosarcoma (EOS) and to improve the understanding of this disease and other similar lesions. METHODS: The data for 11 patients with pathologically confirmed extraosseous osteosarcoma, including tumour site and size and imaging and clinical manifestations, were analysed retrospectively. RESULTS: Six patients were male (60%), and 5 were female (40%); patient age ranged from 23 to 76 years (average age 47.1 years). Among the 11 patients, 7 had clear calcifications or ossification with different morphologies, and 2 patients showed a massive mature bone tumour. MRI showed a mixed-signal mass with slightly longer T1 and T2 signals in the tumour parenchyma. Enhanced CT and MRI scans showed enhancement in the parenchyma. Ten patients had different degrees of necrosis and cystic degeneration in the mass, 2 of whom were complicated with haemorrhage, and MRI showed "fluid‒fluid level" signs. Of the 11 patients, five patients survived after surgery, and no obvious recurrence or metastasis was found on imaging examination. One patient died of lung metastasis after surgery, and 2 patients with open biopsy died of disease progression. One patient died of respiratory failure 2 months after operation. 2 patients had positive surgical margins, and 1 had lung metastasis 6 months after operation and died 19 months after operation. Another patient had recurrence 2 months after surgery. CONCLUSION: The diagnosis of EOS requires a combination of clinical, imaging and histological examinations. Cystic degeneration and necrosis; mineralization is common, especially thick and lumpy mineralization. Extended resection is still the first choice for localized lesions. For patients with positive surgical margins or metastases, adjuvant chemoradiotherapy is needed.

Angioinvasive gastrointestinal mucormycosis with duodenal necrosis and perforation in a patient with visceral myopathy.

A 55-year-old woman with a past medical history of visceral myopathy with multiple resultant abdominal surgeries, colectomy caused by severe pseudo-obstruction, and end-stage renal disease on hemodialysis presented with complaints of nausea, abdominal distention, vomiting, and diarrhea for five days. Small-bowel obstruction was identified on CT. She subsequently developed acute gastrointestinal bleeding, anemia, and hemodynamic instability. Extensive gastric ulceration with frank hemorrhage prompted urgent subtotal gastrectomy with multiple intraoperative findings, including a necrotic distal duodenum. Surgical pathology showed invasive fungal infection of the duodenum with perforation, morphologically compatible with invasive mucormycosis. The patient was started on intravenous liposomal amphotericin B, but despite best efforts, the patient ultimately succumbed to this invasive fungal infection in addition to sepsis and multi-organ failure.

[A modified method for percutaneous drainage of acute necrotic collections in patients with infected pancreatic necrosis]. / Modifitsirovannyi metod chreskozhnogo drenirovaniya ostrykh nekroticheskikh skoplenii u patsientov s infitsirovannym pankreonekrozom.

OBJECTIVE: To develop a modified method for percutaneous drainage of acute necrotic collections in patients with infected pancreatic necrosis. MATERIALS AND METHODS: Minimally invasive surgical technologies were used in 74 patients with infected acute necrotic collections at the Krasnodar Regional Clinical Hospital No. 2 between 2017 and 2019. Of these, 59 (79.7%) people underwent percutaneous drainage as a final treatment. In 11 (14.9%) patients, video sequestrectomy through the fistula was additionally used to increase efficiency of percutaneous drainage. RESULTS: PCD in our modification implies delivery of double-lumen drains 26-32 Fr in the same plane to zones of necrosis, their programmed replacement for prevention of obstruction and flexible endoscopy for control of pathological process. Local purulent-necrotic parapancreatitis occurred in 31 (41.9%) patients, widespread parapancreatitis - in 43 (58.1%) patients. There were 339 minimally invasive interventions. Laparotomy was required in 4 (5.4%) patients. Incidence of perioperative complications was 10.6%, mortality - 16.2%. CONCLUSION: A modified percutaneous drainage method may be used as final surgical treatment in 79.7% of patients with infected pancreatic necrosis.

Diagnostic Value Analysis of Multi-Slice Helical Computed Tomography Reconstruction Parameters Combined with 3.0 T Magnetic Resonance in Clear Cell Renal Cell Carcinoma.

OBJECTIVE: The diagnostic value of multi-slice helical computed tomography (MSCT) reconstruction parameters combined with 3.0 T magnetic resonance (MR) in clear cell renal cell carcinoma (CCRCC) was analysed. METHODS: A total of 158 patients with renal tumours were selected in First Hospital in Zibo city from February 2018 to March 2023 for the retrospective study and divided into CCRCC and non-CCRCCs groups according to the final results of pathological diagnosis. MSCT detection and 3.0 T MR detection were performed in both groups for imaging manifestation analysis. The receiver operating characteristic (ROC) curve was used in analysing the clinical efficacy of each single and combined diagnosis. RESULTS: The results of pathological diagnosis showed 115 patients with CCRCC and 43 non-CCRCC patients, accounting for 72.78% and 27.22%, respectively. Patients with CCRCC had higher proportions of calcification, necrosis, cystic degeneration and more pseudocapsules than non-CCRCC patients (p < 0.05). Patients with CCRCC mainly showed peripheral and heterogeneous enhancement, whereas non-CCRCC patients mainly showed homogeneous enhancement, and the difference was significant (p < 0.05). The cortical phase, parenchymal phase and excretion stage had higher computed tomography (CT) values in the CCRCC group (p < 0.001), and no significant difference in the CT value of plain scan phase was found between the groups (p > 0.05). The CCRCC group had obviously higher apparent diffusion coefficient value and incidence of necrosis and cystic degeneration (p < 0.001), lower incidence of haemorrhage (p < 0.05) and distinctly higher cortical enhancement indexes in the cortical phase, parenchymal phase and delay period (p < 0.001). The ROC analysis showed that the area under the curve, specificity, sensitivity and 95% CI of combined detection were higher than those of each single detection. CONCLUSIONS: The combination of MSCT reconstruction parameters and 3.0 T MR has a certain diagnostic value for CCRCC. The combined diagnosis has higher area under the curve, specificity, sensitivity and 95% CI, which can provide effective reference for clinical diagnosis and treatment, with a certain clinical application value.

Differential Diagnosis of Intracranial Malignant Tumors Using MRI Based on Morphological Features and Signal Intensity Ratio of Lesions.

Objective: To investigate the clinical value of conventional MRI morphological features and signal intensity ratio in the differential diagnosis of intracranial malignant tumors (high-grade glioma (HGG), primary central nervous system Lymphoma (PCNSL) and single brain metastasis (BM). Methods: Retrospective analysis of 92 cases of HGG, 27 cases of PCNSL, and 35 cases of BM. MRI data in The General Hospital of Western Theater Command from August 2014 to December 2021, comparative analysis of morphological characteristics of tumors and lesion/normal brain parenchyma signal ratio (lesiontonormal parenchymaratio, LNR), five indexes were included T1WI signal ratio (LNRT1), T2WI signal intensity ratio (LNRT2), T2WI/T1WI signal ratio (LNRT2/T1), T1WI enhanced signal ratio (LNRT1CE) and contrast enhancement ratio (CER). The differential diagnostic performance was also assessed by subject operating characteristic (ROC) curves. Results: HGG, PCNSL, and BM were all seen more frequently in the supratentorial region, More than 50% of HGG mainly showed irregular morphology, intratumoral necrosis, cystic degeneration, peritumoral severe edema, cyclic uneven enhancement after enhancement, PCNSL significantly enhanced the main uniformity, necrosis cyst became rare, BM group showed uneven enhancement, no obvious specificity, and the differences in tumor morphology, peritumor edema, intratumor hemorrhage, necrotic cystic lesions, and enhancement patterns were statistically significant among the three (P < .05). PCNSL LNRT1 and its LNRT1CE (LNRT1: 0.558 ± 0.050, LNRT1CE: 1.637 ± 0.125) were significantly higher than those of HGG (LNRT1: 0.480 ± 0.077, LNRT1CE: 1.425 ± 0.160) and BM (LNRT1: 0.514 ± 0.120, LNRT1CE: 1.375 ± 0.122), while LNRT2 and LNRT2/T1 (LNRT2: 1.389 ± 0.086, LNRT2/T1: 2.511 ± 0.295) were significantly lower than those of HGG (LNRT2: 1.527 ± 0.191, LNRT2/T1: 3.263 ± 0.657), and BM (LNRT2: 1.504 ± 0.089, LNRT2/T1: 3.103 ± 0.830). There was no significant difference in CER among the three groups (P > .05). ROC curve analysis of LNRT1, LNRT2, LNRT1CE, and LNRT2/T1 could be used to discriminate PCNSL from HGG and BM, with LNRT1CE having the largest area under the curve of 0.873, sensitivity of 0.963 and specificity of 0.669. Conclusion: MRI lesion morphological features and signal intensity ratio are important for discriminating HGG from PCNSL and BM. As a quantitative parameter, tumor signal intensity ratio can provide an important supplement for subjective judgment, to improve the accuracy of tumor qualitative diagnosis and differential diagnosis.

[Complex surgical treatment of patients with necrotic soft tissue infections]. / Kompleksnoe khirurgicheskoe lechenie patsientov s nekroticheskimi infektsiyami myagkikh tkanei.

OBJECTIVE: To develop an optimal algorithm for complex treatment of patients with necrotic soft tissue infections (NSTI). MATERIAL AND METHODS: The study included 114 patients with NSTI who were treated between 2016 and 2021. We analyzed treatment outcomes in 2 groups: retrospective (n=43) with traditional approaches to purulent surgery (drainage of necrotic foci, local therapy with iodophores and water-soluble ointments, antibacterial and detoxification therapy, delayed skin grafting) and prospective (n=71) with active surgical treatment and modern algorithm based on a differentiated approach and high-tech methods (vacuum therapy, hydrosurgical treatment of wounds, early skin grafting and extracorporeal hemocorrection). RESULTS: The main group was characterized by shorter phase I of the wound process by 7.1±2.1 days, earlier relief of symptoms of systemic inflammatory response by 4.2±1.4 days, shorter hospital-stay by 7.7±2.2 days and lower mortality by 15%. CONCLUSION: Early surgery and integrated approach including active surgical strategy, early skin grafting and intensive care with extracorporeal detoxification are necessary to improve the outcomes in patients with NSTI. These measures are effective to eliminate purulent-necrotic process, reduce mortality and hospital-stay.

[One-stage reconstructive jejunogastroplasty after previous multiple abdominal surgeries for left-sided diaphragm rupture complicated by gastric incarceration and necrosis]. / Odnomomentnaya rekonstruktivnaya eyunogastroplastika u bol'nogo posle mnozhestvennykh operatsii na bryushnoi polosti po povodu razryva levogo kupola diafragmy s ushchemleniem i nekrozom zheludka.

Acute gastric necrosis is a rare event requiring organ resection. Delayed reconstruction is advisable in patients with peritonitis and sepsis. The most common complication of gastrectomy with reconstruction is failure of esophagojejunostomy and duodenal stump. In case of severe esophagojejunostomy failure, appropriate surgical approach and timing of reconstructive stage should be analyzed. We report one-stage reconstructive surgery in a patient with multiple fistulas after previous gastrectomy. Surgery included reconstructive jejunogastroplasty with jejunal graft interposition. The patient underwent previous several unsuccessful reconstructive procedures complicated by failure of esophagojejunostomy and duodenal stump with external intestinal, duodenal and esophageal fistulas. Nutritional insufficiency, water and electrolyte disorders due to significant loss of proteins and intestinal juice through the drain tubes deteriorated clinical status. Surgical procedures finished reconstruction, provided closure of multiple fistulas and stomas and restored physiological duodenal passage.

Histiocytic sarcoma with spinal necrosis in a dog with progressing non-ambulatory tetraparesis.

Background: Histiocytic sarcoma (HS) is an aggressive malignant neoplasm, and widespread metastasis occurs with a fatal outcome. HS involving the central nervous system is relatively uncommon. Spinal cord necrosis, a very rare condition, could be induced by ischemia or infarction. Here, we report a dog progressing non-ambulatory tetraparesis with spinal cord necrosis caused by HS. Case Description: A 9-year-old male Labrador Retriever was presented with a progressing non-ambulatory tetraparesis. CT imaging revealed lysis of the spinous process of T7 and a ring-shaped lesion surrounding the soft tissue of lung fields. T2-weighted MRI showed the spinous processes of T6 to T8 as hyperintense, and the lesion infiltrated into the T7 vertebra and the spinal cord. After euthanasia, the final diagnosis upon necropsy was HS, which was observed in the lung, spinous process, thoracic cord, and the pulmonary hilar lymph node. Moreover, necrotic spots were spread widely through the thoracic spinal cord. Conclusion: This report outlines a case of canine HS in the lung, spinous process, thoracic cord, and pulmonary hilar lymph node. Ischemic deficit and necrosis of the thoracic spinal cord resulted from the compression of perivascular tumor cells, which rapidly led to progressive tetraparesis. Although the diagnosis was difficult, MRI and CT images helped determine the prognosis. To our knowledge, this is the first case report of canine HS with direct spinal cord involvement associated with spinal necrosis.

A young female case of asymptomatic immune-mediated necrotizing myopathy: a potential diagnostic option of antibody testing for rhabdomyolysis.

Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) immune-mediated necrotizing myopathy (IMNM) is a neuromuscular disorder that presents muscle weakness in proximal extremities and/or the trunk with an elevation of creatine kinase (CK). Young and asymptomatic anti-HMGCR IMNM patients are very rare and a treatment regimen has not been established. The present case, a 17-year-old woman without any muscular symptoms, only showed hyperCKemia that was detected by chance. After close examinations, including a muscle biopsy and antibody search, she was diagnosed as anti-HMGCR IMNM, and initial treatment with methotrexate and continuous intravenous immunoglobulin seemed to be effective. The present case is the unusually young asymptomatic case of anti-HMGCR IMNM. The diagnosis was successfully made, leading to the early introduction of a treatment. Given the course of this case, we believe that the preceding antibody testing is one of the diagnostic option for rhabdomyolysis.

Endoscopic necrosectomy of infected WON in acute necrotising pancreatitis - Development of an effective therapeutic algorithm based on a single-center consecutive patient cohort.

OBJECTIVE: Development of an effective therapeutic algorithm for interventional (minimally invasive) approach to infected walled-off necrosis (WON) in patients with necrotising pancreatitis reflecting real-word data. MATERIAL/METHODS: All consecutive patients who underwent endoscopic necrosectomy for necrotising pancreatitis through a defined study period were enclosed in this retrospective observational case study. The therapeutic approach was analysed for clinical success rate, complication spectrum and rate as well as mortality and compared with data from the literature. Finally, a therapeutic algorithm was derived. RESULTS: From 2004 to 2019, 126 patients with necrotising pancreatitis (median of APACHE II score, 10.5 points) were treated. In 92.9 % of cases (n=117), an infected WON with microbial pathogen detection was found. After a median of 18 days from symptom onset, first intervention was performed (53.2 % as percutaneous drainage with programmed rinsing, 29.4 % as EUS-guided internal drainage). From 2004 to 2010, double pigtail stents were used. Later, lumen-apposing metal stent (LAMS) such as AXIOSTM stent (Boston Scientific, Ratingen, Germany) was preferred. The combined percutaneous and internal drainage was performed in approximately 50 % of subjects.Endoscopic transluminal necrosectomy was performed in 123 patients (97.6 %) at a median of 33 days from symptom onset. Endoscopic percutaneous necrosectomy was conducted in 11.1 % of the individuals. A median number of two endoscopic necrosectomy sessions per patient was necessary for the therapy. The clinical success rate (discharge without surgical intervention) was 82.5 %. The complication rate (bleeding and perforation) and the need for surgery were both 9.5 %. The overall mortality was 8.7 %. CONCLUSION: Therapy of necrotising pancreatitis with infected WON consists of early calculated antibiotic therapy with adequate drainage. Combined external and internal drainages with programmed rinsing seem to improve prognosis, as well as minimise I) : the need for forced necrosectomies (mainly via a transluminal access site) and II) : complication rate as well as, thus, improve outcome.

A propósito de un caso: abordaje y manejo integral de la enfermedad de buerger en Atención Primaria / Regarding A Clinical Case: Comprehensive Approach And Management Buerger's Disease In Primary Care

Las enfermeras especialistas en atención comunitaria tenemos una misión con la salud pública para mejorar la salud de la comunidad. Desde nuestra consulta tenemos la gran responsabilidad de mejorar la salud de nuestros pacientes, desde la prevención y promoción de la salud y siempre con un enfoque centrado en la persona y su empoderamiento. A propósito de un caso clínico aborda la problemática que supone la pandemia del tabaquismo en nuestra sociedad actual y cómo desde nuestra consulta de atención primaria podemos abordar tanto la deshabituación como el seguimiento y tratamiento de las morbilidades asociadas. En este caso, la enfermedad de buerger. (AU)

Community care nurse specialists have a mission with public health to improve the health of the community. From our health center we have the great responsibility of improving the health of our patients, from the prevention and promotion of health and always with a personcentered approach and their empowerment. Regarding a clinical case, it addresses the problems that the smoking pandemic entails in our current society and from our primary care consultation we can address both cessation and follow-up and how to treat associated morbidities. In this case, Buerger's disease. (AU)

The use of MR perfusion parameters in differentiation between glioblastoma recurrence and radiation necrosis.

INTRODUCTION: This study focuses on the challenge of distinguishing between tumour recurrence and radiation necrosis in glioma treatment using magnetic resonance imaging (MRI). Currently, accurate differentiation is possible only through surgical biopsy, which is invasive and may cause additional damage. The study explores non-invasive methods using dynamic susceptibility contrast (DSC) MR perfusion with parameters like relative peak height (rPH) and relative percentage of signal-intensity recovery (rPSR). MATERIAL AND METHODS: Among retrospectively evaluated patients (multicentre study) with an initial diagnosis of the primary and secondary brain tumour, 47 met the inclusion criteria and were divided into two groups, the recurrent glioblastoma (GBM) WHO IV group and the radiation necrosis group, based on MRI of the brain. All patients enrolled into the recurrent GBM group had a second surgical intervention. RESULTS: Mean, minimum and maximum rPH values were significantly higher in the recurrent GBM group than in the radiation necrosis group ( p < 0.001), while rPSR values were lower in the recurrent GBM group than in the radiation necrosis group ( p = 0.011 and p = 0.012). DISCUSSION: This study investigates the use of MR perfusion curve characteristics to differentiate between radiation necrosis and glioblastoma recurrence in post-treatment brain tumours. MR perfusion shows promising potential for distinguishing between the two conditions, but it also has certain limitations. Despite challenges in finding a sufficient cohort size, the study demonstrates significant differences in MR perfusion parameters between radiation necrosis and GBM recurrence. CONCLUSIONS: The results demonstrate the potential usefulness of these DSC perfusion parameters in discriminating between glioblastoma recurrence and radiation necrosis.

Necrosis intestinal, una complicación infrecuente de la nutrición enteral por yeyunostomía / Mesenteric necrosis, a rare complication of feeding jejunostomy

RESUMEN La nutrición enteral es parte importante del soporte vital avanzado en el paciente crítico, y ha demostrado ser más fisiológica, económica y con resultados superiores a la nutrición parenteral. La yeyunostomía para alimentación enteral está indicada cuando no es posible la alimentación por vía oral y está contraindicada la utilización de una sonda nasogástrica o nasoyeyunal de alimentación. Es una vía de alimentación con escasa morbilidad, aunque no está exenta de complicaciones, y algunas de ellas pueden ser graves. Comunicamos un caso de necrosis intestinal vinculado a la alimentación enteral por yeyunostomía en un paciente sometido a una gastrectomía oncológica.

ABSTRACT Enteral nutrition is an important component of advanced life support in the critically ill patient, and has demonstrated to be more physiologic, cheaper and with better results than parenteral nutrition. Jejunostomy for enteral nutrition is indicated when the oral route is impossible and the use of a nasogastric or nasojejunal feeding tube is contraindicated. Although the rate of complications associated with enteral nutrition through jejunostomy is low, they may occur and be serious. We report a case of bowel necrosis associated with a jejunostomy performed for enteral nutrition in a patient who underwent oncologic gastrectomy.

Mimicking the mimicker: Necrotizing sarcoid granulomatosis.

Necrotizing sarcoid granulomatosis (NSG) is a rare disease that shares similarities with pulmonary vasculitides and sarcoidosis. This is a report of two cases of NSG with a review of literature. The first case is a 33-year-old black female with a one-year history of malaise and cough. Lung imaging revealed scattered pulmonary nodules. Histopathology showed multiple necrotizing granulomas without prominent neutrophilic infiltrates. The second case is a 58-year-old white female with a one-year history of fatigue, dyspnea, and ophthalmoplegia on the left eye. Imaging showed multiple pulmonary nodules. Lung biopsy was consistent with NSG. The challenge of the NSG diagnosis is to distinguish it from other mimickers. Pathology often shows necrotizing granulomatous vasculitis, distinguishing it from classical sarcoid. Laboratory markers for vasculitis like neutrophil cytoplasmic antibodies and antibodies against myeloperoxidase and proteinase 3 are negative or only low titers. NSG responds well to immune-suppression, most commonly with glucocorticoids.