scholarly journals Severe Disabling Myalgia as an Initial Presentation of Polyarteritis Nodosa

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
H. M. Senarathna ◽  
C. L. Fonseka ◽  
H. A. S. Perera ◽  
P. U. T. De Silva ◽  
T. P. Weerarathna
Keyword(s):  
Polyarteritis Nodosa ◽  
Early Recognition ◽  
Specific Treatment ◽  
Oral Prednisolone ◽  
Pulse Therapy ◽  
Initial Presentation ◽  
Foot Drop ◽  
Clinical Feature ◽  
High Dose ◽  
Ct Angiogram

Background. Polyarteritis nodosa (PAN) is a form of necrotizing vasculitis affecting medium or small blood vessels with multiorgan involvement. Although myalgia is a clinical feature of PAN, severe disabling myalgia as the initial presentation is rarely noted. Case Presentation. We present a case of 54-year-old male with recently detected chronic kidney disease admitted with progressive severe disabling muscular pains predominantly over calves with constitutional symptoms for seven weeks. He was weak to mobilize out of the bed. Later, he developed a vasculitic rash, unilateral ulnar claw, and bilateral foot drop. His skin and muscle biopsies showed evidence of vasculitis. His renal and mesenteric artery CT angiogram revealed stenosed segment of the celiac artery without evidence of visible aneurysms elsewhere. He completed six cycles of intravenous cyclophosphamide pulse therapy with high-dose oral prednisolone with good response. With continuation of aggressive immunosuppression and rehabilitation for five months, the patient improved and was able to walk without support. Conclusion. Musculoskeletal predominant PAN, even though rare, needs to be considered in patients presenting with disabling muscle pain and weakness. These features may herald over days to months along with constitutional symptoms before other systems getting affected. Early recognition of such symptoms and initiating specific treatment would be important for better outcomes.

scholarly journals Polyarteritis nodosa with perirenal hematoma due to the rupture of a renal artery aneurysm

2020 ◽  
Author(s):  
Taro Miyagawa ◽  
Yasunori Iwata ◽  
Megumi Oshima ◽  
Hisayuki Ogura ◽  
Koichi Sato ◽  
...  
Keyword(s):  
Polyarteritis Nodosa ◽  
Coil Embolization ◽  
Artery Aneurysm ◽  
Oral Prednisolone ◽  
Good Health ◽  
Pulse Therapy ◽  
Arterial Aneurysm ◽  
Laboratory Findings ◽  
Perirenal Hematoma ◽  
Intravenous Methylprednisolone Pulse

Abstract We present the case of a 67-year-old man in good health with perirenal hematoma due to a ruptured arterial aneurysm in the kidney. The patient developed weight loss, muscle weakness, multiple mononeuropathy, hypertension, anemia, renal insufficiency, and multiple lacuna infarctions about a month ago. He was admitted to the hospital due to worsening of his symptom. After admission, severe right-flank pain suddenly occurred; he was then transferred to our hospital. Renal angiography revealed bilateral multiple microaneurysms, and the patient was diagnosed with polyarteritis nodosa based on the clinical, radiographic, and histological findings. We performed selective coil embolization to the ruptured aneurysm and administered oral prednisolone along with intravenous methylprednisolone pulse therapy. Cyclophosphamide pulse therapy was also given. The treatment improved clinical and laboratory findings and achieved clinical remission. Selective coil embolization to the bleeding aneurysm of polyarteritis nodosa was minimally invasive and promptly effective. Immunosuppressants proved useful in the regulation of disease activity and the aneurysm.

scholarly journals A rare case of childhood polyarteritis nodosa successfully treated with etanercept

2019 ◽  
Vol 7 (7) ◽  
pp. 2837
Author(s):  
Pragathi Kamath ◽  
Bela Verma ◽  
Tejasi Sawant
Keyword(s):  
Polyarteritis Nodosa ◽  
Systemic Vasculitis ◽  
Vascular Inflammation ◽  
Oral Prednisolone ◽  
Skin Lesions ◽  
Pulse Therapy ◽  
Tnf Alpha ◽  
Membrane Expression ◽  
Etanercept Treatment ◽  
Methyl Prednisolone

Childhood Polyarteritis Nodosa (CPAN) a rare and often fatal disease tends to be more common in individuals of Asian descent. Previously it was referred to as Infantile PAN. Polyarteritis Nodosa (PAN) is a systemic autoimmune vasculitis characterized by necrotizing inflammatory lesions of the medium-sized and small muscular arteries, preferentially at vessel bifurcations, resulting in formation of microaneurysms, aneurysmal ruptures with hemorrhage, thrombosis and consequently organ ischemia or infarction. It usually appears in middle or older age without gender predilection. PAN shows variety of symptoms, including general symptoms, neurological, skin, renal and gastrointestinal involvement. In particular skin lesions characterized by multiple firm waxy papules, subcutaneous nodules, livedo reticularis, ulcers and gangrene are observed in 25%-60% of patients with PAN. The etiology of systemic vasculitis is yet unknown. However, dysregulation and/or enhanced expression of pro-inflammatory substances may be involved in pathogenesis of these diseases. Tumour necrosis factor (TNF) alpha is a pro-inflammatory cytokine produced primarily by cells of macrophage-monocyte lineage which may directly participate in vascular inflammation as well as in endothelial cell death via apoptosis. In addition, TNF-alpha may play a role in neutrophil priming inducing membrane expression of Proteinase-3 or Myeloperoxidase which are subsequently recognized by ANCA-associated vasculitis (AAV). Author report a case of 14 months old male child with complaints of fever, gangrenous changes of ear lobes, tip of nose and toes, seizures, altered sensorium and hypertension. Initially Injectable Methyl Prednisolone pulse therapy was started followed by oral Prednisolone. After initiation of Etanercept treatment, his symptoms improved dramatically. Sensorium improved, skin ulcers healed faster, and gangrenous changes were arrested, fever subsided and child started accepting oral feeds.

Pancreaticoduodenal artery aneurysm associated with polyarteritis nodosa presenting as massive upper gastrointestinal bleeding

2021 ◽  
Vol 14 (11) ◽  
pp. e247359
Author(s):  
Juan Carlos Reyes Abon ◽  
Marc Paul Jose Lopez ◽  
A'Ericson Berberabe ◽  
Kenan Jared Cinco
Keyword(s):  
Polyarteritis Nodosa ◽  
Artery Aneurysm ◽  
Pulse Therapy ◽  
High Dose ◽  
Pancreaticoduodenal Artery ◽  
True Aneurysm ◽  
Risk Of Rupture ◽  
Pancreaticoduodenal Artery Aneurysm ◽  
Gastrointestinal Tract Bleeding ◽  
Cyclophosphamide Pulse

The pancreaticoduodenal arteries are rare sites for true aneurysm formation, but these may develop in association with occlusion of the coeliac circulation, degenerative conditions or inflammatory vascular disorders. These have a high risk of rupture regardless of size or other factors. One identified cause is polyarteritis nodosa (PAN), which is an autoimmune necrotising vascular condition that affects small-sized and medium-sized arteries. We report a case of a 40-year-old man with massive gastrointestinal tract bleeding from a ruptured pancreaticoduodenal artery aneurysm secondary to PAN. This was managed with emergent open aneurysm ligation followed by high-dose corticosteroids and cyclophosphamide pulse therapy. Only three other cases of PAN-associated pancreaticoduodenal artery aneurysms have been reported in the literature.

High-Dose Corticosteroid Pulse Therapy Increases the Survival Rate in COVID-19 Patients at Risk of Cytokine Storm

2020 ◽  
Author(s):  
Miguel Ángel López-Zúñiga ◽  
Aida Moreno-Moral ◽  
Ana Ocaña-Granados ◽  
Francisco Padilla-Moreno ◽  
Alba María Castillo-Fernández ◽  
...  
Keyword(s):  
At Risk ◽  
Survival Rate ◽  
Pulse Therapy ◽  
High Dose ◽  
Cytokine Storm ◽  
High Dose Corticosteroid ◽  
Patients At Risk ◽  
Corticosteroid Pulse Therapy ◽  
Dose Corticosteroid

scholarly journals Cortical Excitability across the ALS Clinical Motor Phenotypes

2021 ◽  
Vol 11 (6) ◽  
pp. 715
Author(s):  
Thanuja Dharmadasa
Keyword(s):  
Motor Cortex ◽  
Cortical Excitability ◽  
Phenotypic Variability ◽  
Specific Treatment ◽  
Underlying Disease ◽  
Selective Vulnerability ◽  
Clinical Feature ◽  
Cortical Hyperexcitability ◽  
Wide Range

Amyotrophic lateral sclerosis (ALS) is characterized by its marked clinical heterogeneity. Although the coexistence of upper and lower motor neuron signs is a common clinical feature for most patients, there is a wide range of atypical motor presentations and clinical trajectories, implying a heterogeneity of underlying pathogenic mechanisms. Corticomotoneuronal dysfunction is increasingly postulated as the harbinger of clinical disease, and neurophysiological exploration of the motor cortex in vivo using transcranial magnetic stimulation (TMS) has suggested that motor cortical hyperexcitability may be a critical pathogenic factor linked to clinical features and survival. Region-specific selective vulnerability at the level of the motor cortex may drive the observed differences of clinical presentation across the ALS motor phenotypes, and thus, further understanding of phenotypic variability in relation to cortical dysfunction may serve as an important guide to underlying disease mechanisms. This review article analyses the cortical excitability profiles across the clinical motor phenotypes, as assessed using TMS, and explores this relationship to clinical patterns and survival. This understanding will remain essential to unravelling central disease pathophysiology and for the development of specific treatment targets across the ALS clinical motor phenotypes.

scholarly journals Clinical presentation and immunological features of Post-Malaria Neurologic Syndrome: a case report and review of literature

2020 ◽  
Vol 19 (1) ◽  
Author(s):  
Nadia Castaldo ◽  
Carlo Tascini ◽  
Paola Della Siega ◽  
Maddalena Peghin ◽  
Davide Pecori
Keyword(s):  
Unmet Need ◽  
Specific Treatment ◽  
Rare Condition ◽  
Acute Disseminated Encephalomyelitis ◽  
Intravenous Immunoglobulins ◽  
Neurological Involvement ◽  
Systematic Research ◽  
High Dose ◽  
Microbiological Analysis ◽  
Randomized Controlled Studies

Abstract Background Malaria still represents a major health threat, in terms of both morbidity and mortality. Complications of malaria present a diversified clinical spectrum, with neurological involvement leading to the most serious related-conditions. The authors recently encountered a case of a 60-year old Italian man presenting with confusion, language disturbances and Parkinson-like syndrome 3 weeks after complete remission from severe Plasmodium falciparum cerebral malaria. Chemical and microbiological analysis revealed aseptic meningitis, diffuse encephalitis and abnormal immune-activation. Re-infection and recrudescence of infection were excluded. Further analysis excluded paraneoplastic and autoimmune causes of encephalitis. A diagnosis of Post-Malaria Neurological Syndrome (PMNS) was finally formulated and successfully treated with high dose of steroids. Methods A systematic research of current literature related to PMNS was performed. Results 151 cases of PMNS were included, the majority of which occurred after severe P. falciparum infections. Four main clinical pattern were identified: 37% of the cases presented as “classical” PMNS, 36% presented as delayed cerebellar ataxia (DCA), 18% resembled acute inflammatory demyelinating polyneuropathy (AIDP), and 8% presented as acute disseminated encephalomyelitis (ADEM)-like form. Differentiation between different forms was not always simple, as clinical and radiological findings frequently overlap. Overall, in almost all of the tested cases, cerebrospinal fluid was found pathological; EEG revealed nonspecific encephalopathy in 30% of classical PMNS and 67% ADEM; imaging tests were found abnormal in 92% of ADEM-like forms. Pathogenesis remains unclear. An autoimmune mechanism is the most corroborated pathogenic hypothesis. Overall, the majority of PMNS cases revert without specific treatment. In most severe forms, high dose steroids, intravenous immunoglobulins, and plasmapheresis have been shown to improve symptoms. Conclusions PMNS is a disabling complication of malaria. The overall incidence is not known, due to frequent misdiagnosis and under-reporting. Pathogenesis is not also fully understood, but rapid response to immune-modulating treatment along with similarities to auto-immune neurological disease, strongly support a dysregulated immunological genesis of this condition. The lack of randomized controlled studies regarding therapeutic approaches is a major unmet need in this setting. A systematic collection of all the PMNS cases would be desirable, in order to increase awareness of this rare condition and to prospectively investigate the most appropriate management.

Sudden onset blindness treated with pulse steroid therapy in a patient with microscopic polyangiitis

Open Medicine ◽  
2011 ◽  
Vol 6 (5) ◽  
pp. 631-633
Author(s):  
Yoshiro Horai ◽  
Tomoya Miyamura ◽  
Karin Shimada ◽  
Soichiro Takahama ◽  
Rumi Minami ◽  
...  
Keyword(s):  
Steroid Therapy ◽  
Microscopic Polyangiitis ◽  
Oral Prednisolone ◽  
Sudden Onset ◽  
High Dose ◽  
Pulse Steroid Therapy ◽  
Ocular Manifestations ◽  
Dose Oral ◽  
The Right ◽  
Pulse Steroid

AbstractWe report a 71-year-old male with microscopic polyangiitis (MPA) who developed sudden-onset, progressive, bilateral visual loss associated with a relapse of MPA symptoms. The patient was referred to our hospital, and treated with intravenous pulse steroid therapy and high-dose oral prednisolone. Although the right eye remained vision deficient, visual acuity in the left eye recovered. Ocular manifestations of MPA are quite uncommon. This case emphasizes the necessity of early detection and initiation of prompt therapy where ocular manifestations of MPA occur.

Ranscriptome Analysis Reveals the Molecular Mechanism of Yiqi Rougan Decoction in Reducing CCl4-induced Liver Fibrosis in Rats

2021 ◽  
Author(s):  
Yu Xiong ◽  
Jinyuan Hu ◽  
Chen Xuan ◽  
Jiayu Tian ◽  
Kaiyue Tan ◽  
...  
Keyword(s):  
Protein Kinase ◽  
Liver Fibrosis ◽  
Liver Injury ◽  
Molecular Mechanism ◽  
Treatment Strategies ◽  
Specific Treatment ◽  
Mitogen Activated Protein Kinase ◽  
Signalling Pathways ◽  
High Dose ◽  
Peroxisome Proliferator

Abstract BackgroundLiver fibrosis develops from various chronic liver diseases, and there is currently a lack of specific treatment strategies. Yiqi Rougan decoction (YQRG) is a traditional Chinese medicine that has shown durative effects in the treatment of liver fibrosis; however, the mechanism associated with YQRG-related improvements in liver fibrosis remains to be experimentally determined. This study evaluated the therapeutic effect of YQRG on carbon tetrachloride (CCl4)-induced liver fibrosis in rats and its molecular mechanism. MethodsWe used low-, medium-, and high-dose YQRG to treat CCl4-induced liver fibrosis in rats, followed by assessment of liver injury and fibrosis according to liver appearance, body weight, liver mass index, histopathologic examination, and serum testing. Additionally, we performed transcriptome analysis using RNA-sequencing (RNA-seq) technology, including cluster, Gene Ontology (GO), and pathway analyses, to identify differentially expressed genes (DEGs), and protein and gene expression were detected by immunofluorescence (IFC), western blot, and real-time quantitative PCR. ResultsThe results showed that YQRG effectively alleviated CCl4-induced liver injury and fibrosis in rats, including observations of improved liver function, decreased activity of hepatic stellate cells (HSCs), and decreased extracellular matrix (ECM) deposition. Moreover, we identified downregulated and upregulated DEGs in the model group relative to the control and YQRG-treated groups, with GO analysis revealing their enrichment in biological processes, such as endoplasmic reticulum stress (ERS), apoptosis, and autophagy. Furthermore, pathway analysis showed that YQRG treatment downregulated the mitogen-activated protein kinase (MAPK) and phosphoinositide 3-kinase/Akt (PI3K/AKT) signalling pathways and upregulated other signalling pathways, including those related to peroxisome proliferator-activated receptors(PPAR) and AMP-activated protein kinase(AMPK), with these finding subsequently verified experimentally. ConclusionThese findings showed that YQRG improved CCl4-induced liver fibrosis through multiple mechanisms and pathways, offering critical insight into the YQRG-related therapeutic mechanism and promoting further research into its potential application.

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