scholarly journals NCMP-23. PEMBROLIZUMAB-ASSOCIATED CD8+ VASCULITIC MONONEURITIS MULTIPLEX IN A PATIENT WITH MESOTHELIOMA: FIRST CASE REPORT

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii127-ii127
Author(s):  
Michaela Baldauf ◽  
Kapauer Monika ◽  
Jörger Markus ◽  
Flatz Lukas ◽  
Regulo Rodriguez ◽  
...  
Keyword(s):  
Case Report ◽  
Checkpoint Inhibitors ◽  
Axonal Neuropathy ◽  
Immunosuppressive Treatment ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
High Dose ◽  
Mononeuritis Multiplex ◽  
First Case ◽  
The Right

Abstract INTRODUCTION Immunotherapy, especially with immune checkpoint inhibitors (ICPI), has increasingly become an attractive treatment modality for various types of cancers. However, many patients develop ICPI-associated autoimmune adverse events such as pneumonitis, colitis or rarely neurological syndromes. Large and medium vessel vasculitis haS only occasionally been reported. Here we report the first case of ICPI-associated mononeuritis multiplex in a patient with malignant mesothelioma, caused by a histological proven small vessel vasculitis. CASE REPORT A 61-year old female developed subacute progressive painful and asymmetric sensorimotor deficits on distal extremities. Electrophysiologically, signs of a severe axonal neuropathy of both legs and the right arm were found, and swellings of the corresponding nerves were seen upon nerve ultrasound exam. The clinical and electrophysiological findings were reminiscent of mononeuritis multiplex. Laboratory work up including CSF examination was normal. More than two years prior to developing peripheral nerve deficits, the patient had been diagnosed with malignant pleural mesothelioma and treated with the anti-PD1 monoclonal antibody pembrolizumab on progression after chemotherapy. Biopsy of the right sural nerve revealed a small vessel vasculitis with a lymphocyte predominance of CD8+ T cells over CD4+ T as well as B lymphocytes. Despite discontinuation of pembrolizumab and immunosuppressive treatment (high dose methylprednisone, cyclophosphamide) complemented by opioid therapy, painful allodynia persisted. CONCLUSION ICPI-associated autoimmune disorders also include small vessel vasculitis with rare phenotypes such as mononeuritis multiplex. Further studies are required to improve our understanding of the link between ICPIs, and the pathogenic process leading to vasculitis, as well as to optimize treatment options for those rare diseases.

P08.01 Immune-Related Acute Motor Axonal Neuropathy: A Small Case Series and Review of the Literature

2021 ◽  
Vol 23 (Supplement_2) ◽  
pp. ii26-ii26
Author(s):  
Y Pina ◽  
N Tran ◽  
P Forsyth ◽  
S Mokhtari ◽  
E Peguero
Keyword(s):  
Checkpoint Inhibitors ◽  
Axonal Neuropathy ◽  
Case Series ◽  
Standard Of Care ◽  
Cancer Center ◽  
High Dose ◽  
Acute Motor Axonal Neuropathy ◽  
First Case ◽  
Oncological Patients ◽  
Solid Malignancies

Abstract BACKGROUND Immunotherapy have revolutionized cancer treatment in the past decade, with a significant increased survival in patients with solid tumors. However, the use of immune checkpoint inhibitors (ICIs) has been associated with a growing number of neurotoxicities, some of which can be fatal if not recognized and treated promptly. Some of these neurotoxicities include very uncommon syndromes like Acute Motor Axonal Neuropathy (AMAN). Herein we present four oncological cases of patients who underwent immunotherapy and developed AMAN. METHODS Four patients were diagnosed with immune-related AMAN between 2017 and 2000 at H. Lee Moffitt Cancer Center. The patients were treated with standard of care and currently follow up in clinic. RESULTS We describe four oncological patients who developed a motor axonal neuropathy (i.e., AMAN) confirmed on nerve conduction studies following 2 cycles of immunotherapy, including a 28 year old woman with melanoma brain metastasis and a 50 year old woman with renal cell carcinoma both treated with ipilimumab and nivolumab, a 32 year old man with Hodgkin lymphoma who was treated with nivolumab and brentuximab, and a 77 year old woman with renal urothelial cancer who was treated with pembrolizumab and cabozantinib. All four patients were promptly recognized as having immune-related neurotoxicity (irNs), were promptly treated (i.e., high dose steroids +/- IVIG +/- other immunomodulators), and significantly improved and have remained stable. CONCLUSION This is the first case series of patients with AMAN following two cycles of immunotherapy, who were successfully treated. It is crucial to develop a better understanding of the irNs associated with ICIs, including those rare conditions that are difficult to diagnose and treat, as the utilization of these immunomodulating therapies continues to increase and expand to include other solid malignancies. Neurologists should be involved early on in any case of suspected irN to assist in the management of these complicated patients and a swift work up should be initiated for timely diagnosis and treatment.

NCMP-21. IMMUNE-RELATED ACUTE MOTOR AXONAL NEUROPATHY: A SMALL CASE SERIES AND REVIEW OF THE LITERATURE

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi151-vi151
Author(s):  
Yolanda Pina ◽  
Nam Tran ◽  
Neha Verma ◽  
Michael Vogelbaum ◽  
Peter Forsyth ◽  
...  
Keyword(s):  
Checkpoint Inhibitors ◽  
Axonal Neuropathy ◽  
Case Series ◽  
Standard Of Care ◽  
Cancer Center ◽  
High Dose ◽  
Acute Motor Axonal Neuropathy ◽  
First Case ◽  
Oncological Patients ◽  
Solid Malignancies

Abstract BACKGROUND Immunotherapy revolutionized cancer treatment in the past decade, with a significant increased survival in patients with solid tumors. However, immune checkpoint inhibitors (ICIs) have been associated with a growing number of neurotoxicities, some of which can be fatal if not recognized and treated promptly. Some of these neurotoxicities include very uncommon syndromes like Acute Motor Axonal Neuropathy (AMAN). Herein we present four oncological cases of patients who underwent immunotherapy and developed AMAN. METHODS Four patients were diagnosed with immune-related AMAN between 2017 and 2000 at H. Lee Moffitt Cancer Center. Patients were treated with standard of care. RESULTS We describe four oncological patients who developed a motor axonal neuropathy (i.e., AMAN) confirmed on nerve conduction studies following 2 cycles of immunotherapy, including a 28 year old woman with melanoma brain metastasis and a 50 year old woman with renal cell carcinoma both treated with ipilimumab and nivolumab, a 32 year old man with Hodgkin lymphoma who was treated with nivolumab and brentuximab, and a 77 year old woman with renal urothelial cancer who was treated with pembrolizumab and cabozantinib. All four patients were promptly recognized as having immune-related neurotoxicity (irNs), were promptly treated (i.e., high dose steroids +/- IVIG +/- other immunomodulators), and significantly improved and have remained stable. CONCLUSION This is the first case series of patients with AMAN following two cycles of immunotherapy, who were successfully treated. It is crucial to develop a better understanding of irNs, including those rare conditions that are difficult to diagnose and treat, as the utilization of these immunomodulating therapies continues to expand to include other solid malignancies. Neurologists should be involved early on in any case of suspected irN to assist in the management of these complicated patients and a swift work up should be initiated for timely diagnosis and treatment.

Progressive multifocal leukoencephalopathy in systemic lupus erythematosus managed with pembrolizumab: A case report with literature review

Lupus ◽  
2021 ◽  
pp. 096120332110354
Author(s):  
Ting-Yuan Lan ◽  
Yan-Siou Chen ◽  
Chiao-Feng Cheng ◽  
Sin-Tuan Huang ◽  
Chieh-Yu Shen ◽  
...  
Keyword(s):  
Case Report ◽  
Viral Load ◽  
Progressive Multifocal Leukoencephalopathy ◽  
Jc Virus ◽  
Checkpoint Inhibitors ◽  
Cell Activity ◽  
Response To Treatment ◽  
High Dose ◽  
Effective Response ◽  
First Case

Progressive multifocal leukoencephalopathy (PML) is one of the rare but lethal infectious complication in patients with SLE, manifesting progressive central nervous demyelination caused by JC virus (JCV). There have been no effective antiviral agents so far; however, immune checkpoint inhibitors (ICI) have been demonstrated as potential treatments by reinvigorating antiviral T-cell activity against JC virus. To date, sixteen PML cases treated with anti-PD-1 have been reported; however, there was no report addressing the use of ICI in patients with concomitant PML and rheumatic disease, possibly due to the concern for possible autoimmune disease flare-up. In addition, treatment outcomes of these ICI-treated cases were heterogeneous. Experiences from these cases suggested that high disease burden, JC viral load in CSF, and severe immunosuppression status at baseline may predict poor response to treatment. Our case, a 62-year-old woman with long-standing SLE, turned out to have a delayed but effective response to prolonged ICI treatment despite of her high JC viral load and immunosuppressed status caused by high-dose steroid and rituximab. To our knowledge, this is the first case report with SLE complicated with PML clinically improved by pembrolizumab treatment without consequent immune related adverse events (irAE). Considering the lethal nature of PML and absence of effective medication, ICI is a reasonable consideration in patients with SLE and progressive PML.

Eosinophilic vasculitis: an inhabitual and resistant manifestation of a vasculitis

VASA ◽  
2010 ◽  
Vol 39 (4) ◽  
pp. 344-348 ◽  
Author(s):  
Jandus ◽  
Bianda ◽  
Alerci ◽  
Gallino ◽  
Marone
Keyword(s):  
Skin Biopsy ◽  
Intravenous Iron ◽  
Small Vessel Vasculitis ◽  
High Dose ◽  
Raynaud Phenomenon ◽  
The Third ◽  
Left Elbow ◽  
Right Hand ◽  
The Right ◽  
Trunk Skin

A 55-year-old woman was referred because of diffuse pruritic erythematous lesions and an ischemic process of the third finger of her right hand. She was known to have anaemia secondary to hypermenorrhea. She presented six months before admission with a cutaneous infiltration on the left cubital cavity after a paravenous leakage of intravenous iron substitution. She then reported a progressive pruritic erythematous swelling of her left arm and lower extremities and trunk. Skin biopsy of a lesion on the right leg revealed a fibrillar, small-vessel vasculitis containing many eosinophils.Two months later she reported Raynaud symptoms in both hands, with a persistent violaceous coloration of the skin and cold sensation of her third digit of the right hand. A round 1.5 cm well-delimited swelling on the medial site of the left elbow was noted. The third digit of her right hand was cold and of violet colour. Eosinophilia (19 % of total leucocytes) was present. Doppler-duplex arterial examination of the upper extremities showed an occlusion of the cubital artery down to the palmar arcade on the right arm. Selective angiography of the right subclavian and brachial arteries showed diffuse alteration of the blood flow in the cubital artery and hand, with fine collateral circulation in the carpal region. Neither secondary causes of hypereosinophilia nor a myeloproliferative process was found. Considering the skin biopsy results and having excluded other causes of eosinophilia, we assumed the diagnosis of an eosinophilic vasculitis. Treatment with tacrolimus and high dose steroids was started, the latter tapered within 12 months and then stopped, but a dramatic flare-up of the vasculitis with Raynaud phenomenon occurred. A new immunosupressive approach with steroids and methotrexate was then introduced. This case of aggressive eosinophilic vasculitis is difficult to classify into the usual forms of vasculitis and constitutes a therapeutic challenge given the resistance to current immunosuppressive regimens.

Chronic relapsing axonal neuropathy: A first case report

1995 ◽  
Vol 37 (1) ◽  
pp. 112-115 ◽  
Author(s):  
Elisabeth Chroni ◽  
Susan M. Hall ◽  
Richard A. C. Hughes
Keyword(s):  
Case Report ◽  
Axonal Neuropathy ◽  
First Case

scholarly journals Acute Tubulointerstitial Nephritis in a Patient on Anti-Programmed Death-Ligand 1 Triggered by COVID-19: A Case Report

2021 ◽  
Vol 8 ◽  
pp. 205435812110147
Author(s):  
Dimitry Buyansky ◽  
Catherine Fallaha ◽  
François Gougeon ◽  
Marie-Noëlle Pépin ◽  
Jean-François Cailhier ◽  
...  
Keyword(s):  
Case Report ◽  
Severe Acute Respiratory Syndrome ◽  
Tubulointerstitial Nephritis ◽  
Checkpoint Inhibitor ◽  
Checkpoint Inhibitors ◽  
High Dose ◽  
Programmed Death Ligand 1 ◽  
Acute Tubulointerstitial Nephritis ◽  
Programmed Death ◽  
Kidney Involvement

Rationale: Immune checkpoint inhibitors are monoclonal antibodies used in the treatment of various types of cancers. The downside of using such molecules is the potential risk of developing immune-related adverse events. Factors that trigger these autoimmune side effects are yet to be elucidated. Although any organ can potentially be affected, kidney involvement is usually rare. In this case report, we describe the first known instance of a patient being treated with an inhibitor of programmed death-ligand 1 (anti-PD-L1, a checkpoint inhibitor) who develops acute tubulointerstitial nephritis after contracting the severe acute respiratory syndrome coronavirus 2. Presenting concerns of the patient: A 62-year-old patient, on immunotherapy treatment for stage 4 squamous cell carcinoma, presents to the emergency department with symptoms of lower respiratory tract infection. Severe acute kidney injury is discovered with electrolyte imbalances requiring urgent dialysis initiation. Further testing reveals that the patient has contracted the severe acute respiratory syndrome coronavirus 2. Diagnosis: A kidney biopsy was performed and was compatible with acute tubulointerstitial nephritis. Interventions: The patient was treated with high dose corticosteroid therapy followed by progressive tapering. Outcomes: Rapid and sustained normalization of kidney function was achieved after completion of the steroid course. Novel findings: We hypothesize that the viral infection along with checkpoint inhibitor use has created a proinflammatory environment which led to a loss of self-tolerance to renal parenchyma. Viruses may play a more important role in the pathogenesis of autoimmunity in this patient population than was previously thought.

scholarly journals Vasculitis and Breast Cancer: Mind the Hint

2021 ◽  
pp. 550-560
Author(s):  
Miguel Esperança-Martins ◽  
Vasco Evangelista ◽  
Salomão Fernandes ◽  
Raquel Almeida
Keyword(s):  
Breast Cancer ◽  
Ductal Carcinoma ◽  
Small Vessel ◽  
Alveolar Haemorrhage ◽  
Small Vessel Vasculitis ◽  
Diffuse Alveolar Haemorrhage ◽  
Anca Associated Vasculitis ◽  
First Case ◽  
Circulating Antibodies ◽  
Perinuclear Anca

Diffuse alveolar haemorrhage related to an anti-neutrophil cytoplasmic antibody (ANCA)-associated small vessel vasculitis is an extremely rare form of presentation of breast cancer. Here we report the case of a 77-year-old woman with a histological diagnosis of a papillary ductal carcinoma of the breast presenting with a diffuse alveolar haemorrhage secondary to a perinuclear ANCA-associated vasculitis. To our knowledge, this is the first case ever reported of a diffuse alveolar haemorrhage related to an ANCA-associated small vessel vasculitis as a form of presentation of breast cancer. The therapeutic approach of this paraneoplastic vasculitis included the use of corticosteroids and plasmapheresis, a very useful technique to remove endothelial aggressors (circulating antibodies) as a strategy to earn time for a proper therapeutic decision specifically directed for disease modification, but that can also be associated with several severe adverse effects, which are illustrated in our case.

scholarly journals Case Report: Bow Hunter's Syndrome Caused by Compression of Extracranially Originated Posterior Inferior Cerebellar Artery

2021 ◽  
Vol 12 ◽  
Author(s):  
Noriya Enomoto ◽  
Kenji Yagi ◽  
Shunji Matsubara ◽  
Masaaki Uno
Keyword(s):  
Case Report ◽  
Posterior Inferior Cerebellar Artery ◽  
Head Rotation ◽  
Radiographic Examination ◽  
Posterior Decompression ◽  
First Case ◽  
Cerebellar Artery ◽  
Cervical Approach ◽  
The Right ◽  
Hunter's Syndrome

Bow hunter's syndrome (BHS) is most commonly caused by compression of the vertebral artery (VA). It has not been known to occur due to an extracranially originated posterior inferior cerebellar artery (PICA), the first case of which we present herein. A 71-year-old man presented with reproducible dizziness on leftward head rotation, indicative of BHS. On radiographic examination, the bilateral VAs merged into the basilar artery, and the left VA was predominant. The right PICA originated extracranially from the right VA at the atlas–axis level and ran vertically into the spinal canal. During the head rotation that induced dizziness, the right PICA was occluded, and a VA stenosis was revealed. Occlusion of the PICA was considered to be the primary cause of the dizziness. The patient underwent surgery to decompress the right PICA and VA via a posterior cervical approach. Following surgery, the patient's dizziness disappeared, and the stenotic change at the right VA and PICA improved. The PICA could be a causative artery for BHS when it originates extracranially at the atlas–axis level, and posterior decompression is an effective way to treat it.

scholarly journals Patient re-presents to the clinic with reactive arthritis in the right hip after covid-19 infection: A case report

2021 ◽  
Author(s):  
Kamyar Shokraee ◽  
Soroush Moradi ◽  
Tahereh Eftekhari ◽  
Rasoul Shajari ◽  
Maryam Masoumi
Keyword(s):  
Case Report ◽  
Reactive Arthritis ◽  
Respiratory Symptoms ◽  
Gastrointestinal Infection ◽  
Case Presentation ◽  
Sexually Transmitted ◽  
Methyl Prednisolone ◽  
First Case ◽  
The Right

Abstract Background: SARS-COV-2, first reported in December 2019, usually presents with respiratory symptoms but can have various other manifestations and sequelae. One of the rare complications of COVID-19 infection is Reactive Arthritis. This complication is more likely to occur following sexually transmitted or gastrointestinal infection.Case presentation: Herein, we report a 58 years old woman hospitalized following COVID-19 infection and was discharged after a week. She consequently presented to the clinic ten days after her discharge, complaining of walking difficulties and radiating pain in her right hip. After ultrasound and MRI, she was diagnosed with reactive arthritis inflammation in the hip’s neck. Other known microorganisms responsible for reactive arthritis were ruled out before attributing it to the earlier COVID-19 infection. She reached remission after being treated using a combination of indomethacin and depot methyl-prednisolone for 14 days. Conclusion: To our knowledge, this is the first case of reactive arthritis caused by SARS-COV-2 in the hip. Further attention should be paid to symptoms occurring after an episode of infection with COVID-19 in order to expand our understanding of the disease and the symptoms with which it can manifest.

Eosinophilic granulomatosis with polyangiitis: understanding the disease and its management

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_3) ◽  
pp. iii84-iii94 ◽  
Author(s):  
Giorgio Trivioli ◽  
Benjamin Terrier ◽  
Augusto Vaglio
Keyword(s):  
B Cells ◽  
Granulomatosis With Polyangiitis ◽  
Clinical Presentation ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Mononeuritis Multiplex ◽  
Persistent Symptoms ◽  
Tissue Eosinophilia ◽  
Eosinophilic Granulomatosis

Abstract Eosinophilic granulomatosis with polyangiitis is characterized by asthma, blood and tissue eosinophilia and small-vessel vasculitis. The clinical presentation is variable, but two main clinic-pathologic subsets can be distinguished: one hallmarked by positive ANCA and predominant ‘vasculitic’ manifestations (e.g. glomerulonephritis, purpura and mononeuritis multiplex) and the other by negative ANCA and prominent ‘eosinophilic’ manifestations (e.g. lung infiltrates and cardiomyopathy). The pathogenesis is not fully understood but probably results from the interplay between T and B cells and eosinophils. Eosinophilic granulomatosis with polyangiitis must be differentiated from several conditions, including hypereosinophilic syndromes and other small-vessel vasculitides. The overall survival is good; however, patients frequently relapse and have persistent symptoms. The recently developed monoclonal antibodies targeting B cells and eosinophilopoietic cytokines such as IL-5 are emerging as valid alternatives to conventional immunosuppressive therapies. In this review, we discuss the essential features of eosinophilic granulomatosis with polyangiitis, with particular respect to the most relevant issues concerning clinical presentation and management.

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