Atypical Kawasaki Disease after COVID-19 Vaccination: A New Form of Adverse Event Following Immunization

Kawasaki disease (KD) is a medium-vessel vasculitis that is typically presented during childhood; fewer than 100 cases of KD have been reported worldwide in adult patients who met the criteria according to the American College of Rheumatology. This study presents the case of an 18-year-old patient with no previous history of any disease, who presented atypical KD with liver and kidney dysfunction, with a good response to intravenous immunoglobulin therapy. The symptoms began 22 days after the application of the COVID-19 vaccine (nonreplicating viral vector Vaxzevria), and other conditions were ruled out. The term Adverse Events Following Immunization (AEFI)encompasses all the reactions that follow the application of any vaccine with no necessary causal relationship and can be due to the vaccine product, quality of the vaccine, immunization errors, or anxiety or just happen to be coincident events. These reactions should be reported so that clinicians can identify compatible cases and consider that the presentation of this disease, despite being atypical, can be manifested in adult patients. Likewise, case reports are an important basis for the pharmacovigilance of vaccines.

Rituximab and intravenous immunoglobulin treatment in PM/Scl antibody-associated disease: case-based review

Autoantibodies to the 75-kDa and 100-kDa subunits of the PM/Scl nucleolar protein complex are associated with an overlap syndrome, manifesting with clinical features of systemic sclerosis and idiopathic inflammatory myopathy. We describe the diverse clinical features in a series of 4 cases with anti-PM/Scl-75 and/or anti-PM/Scl-100 antibodies, including severe proximal muscle weakness, oesophageal dysfunction, respiratory weakness requiring mechanical ventilation, Raynaud’s, calcinosis cutis, sclerodactyly and critical digital ischaemia. Despite the severity of striated and oesophageal muscle weakness, all patients responded very well to immune suppression, and calcinosis cutis in one case regressed substantially. We highlight the efficacy of Rituximab and intravenous immunoglobulin therapy (IVIg) in these cases, enabling return to normal muscle function within six months. Rituximab was preferentially chosen for cases with hyper-gammaglobulinemia and multiple autoantibodies in addition to anti-PM/Scl, and IVIg was utilised for cases where a rapid onset of effect was required, such as severe ventilator-dependent respiratory muscle weakness and oesophageal dysfunction.

A Case Report on Post COVID -19 Vaccine Associated Guillain–Barré Syndrome

Guillain–Barré syndrome (GBS) is an autoimmune demyelinating illness in which a patient’s immune system attacks and cause deterioration of peripheral nervous system leading to progressive paralysis and polyneuropathy. The exact cause of the GBS is unclear but the main mechanism of behindis the demyelination of nerves especially the motor, sensory, and autonomic nerves which can be triggered by any immunologic or infectious agent. The infectious agent elicits the humoral and cellular mediated immune response due to their molecular mimicry in which the antibodies created against the infection matches with the proteins on the nerve. The characteristic features of Guillain–Barré syndrome are ascending flaccid paralysis, paresthesia, impairment of muscle reflexes, respiratory failureetc. The GBS is diagnosed via nerve conduction studies, lumbar puncture (Cerebrospinal fluid analysis), electromyography, Brighton criteria. Treatments like intravenous immunoglobulin therapy, plasma exchange can ease the symptoms and reduce the duration of the illness. This case report focusing on a 43-year-old female patient admitted seeking ventilatory support for respiratory distress caused by Guillain–Barré Syndrome in a tertiary hospital. Patient had developed limb weakness with ascending paralysis along with facial weakness within a couple of weeks after receiving the COVID -19 vaccination (COVISHIELD)one month back. Patient underwent nerve conduction study and routine monitoring of vital parameters. After conservative management with physiotherapy, ventilation, intravenous immunoglobulins and prophylaxis for pain and DVT patient gradually started improving the muscle power and was discharged to continue the rehabilitation care at home.

Evaluation of Intravenous Immunoglobulin G treatment in Outpatients Rheumatology Practice

Objective: Intravenous immunoglobulin is an alternative therapeutic agent that can be used off-label in many autoimmune rheumatological diseases. The aim of this study is to evaluate the autoimmune rheumatological diseases characteristics in which intravenous immunoglobulin therapy is used and the efficacy and safety of this therapy. Methods and Methods: We performed a retrospective review of 133 patients with autoimmune rheumatological disease who received at least 1 course of intravenous immunoglobulin treatment at Hacettepe University Rheumatology Outpatient Clinic between January 2013 and December 2020. The autoimmune rheumatological disease demographic and clinical features, organ involvements, treatment phases (primary-secondary or infection), treatment responses and adverse effects were evaluated. Results: A total of 79% (n=105) patients were female and the mean±SD age was 45.5±16.9 years. The most common underlying rheumatic diseases were systemic lupus erythematosus (35%, n=47) and dermatomyositis/polymyositis (35%, n=47). Intravenous immunoglobulin therapy was most commonly used for resistant/relapsed myositis and haematological involvement. The median (IQR) intravenous immunoglobulin treatment course was 6.5 (13) and the duration of intravenous immunoglobulin treatment was 10.8 (24) months. Although it is used as second-line therapy in 77% of patients, complete clinical response was observed in 32% and partial response in 47%. There was a significant reduction in the median (IQR) steroid doses (methylprednisolone or equivalent dose) patients received from baseline after intravenous immunoglobulin treatment [30 (33) vs 8 (12), p<0.0001]. It was observed that the use of conventional disease-modifying antirheumatic drugs decreased after intravenous immunoglobulin treatment and the use of rituximab increased. Adverse effects associated with intravenous immunoglobulin treatment (10%) and discontinuation (4%) were found to be very low. Conclusion: Intravenous immunoglobulin treatment was commonly given in systemic lupus erythematosus and dermatomyositis/polymyositis patients because of hematological involvement and resistant/relapsed myositis in our study, respectively. Although it is mainly the second-line treatment, two-thirds of the patients achieved a complete/partial response. Side effects and related discontinuation due to intravenous immunoglobulin treatment are very few.

Severe neonatal autoimmune thrombocytopenia secondary to maternal Evans syndrome

Evans syndrome is a rare and chronic autoimmune disease seen in both paediatric and adult age groups. We present a case of severe thrombocytopenia in a neonate born to a mother with Evans syndrome who showed no response to intravenous immunoglobulin therapy initially and improved after treatment with methylprednisolone.

A Kobayashi- és a Kawanet-pontrendszer prediktív értéke Kawasaki-kóros betegeink immunglobulin-rezisztenciája és kardiológiai szövődményei szempontjából.

Összefoglaló. Bevezetés: A Kawasaki-szindróma immunvasculitis, amely kezeletlenül kardiológiai szövődményekhez vezethet. A korai intravénás immunglobulin-terápia mérsékli a szövődményeket, de az esetek 10–20%-a rezisztens a kezelésre. Ennek előrejelzésére világszerte számos rizikóbecslő pontrendszert használnak. Célkitűzés: A Kobayashi- és a Kawanet-pontrendszer prediktív értékének vizsgálata betegeink intravénás immunglobulin-rezisztenciája és kardiológiai szövődményei vonatkozásában. Tudomásunk szerint ez az első magyarországi vizsgálat, amely Kawasaki-szindróma esetében pontrendszerek prediktív értékét méri fel. Módszer: Retrospektív pilotvizsgálatunkban kigyűjtöttük a 2005. január és 2020. április között Kawasaki-szindróma miatt ápolt betegeink adatait. Mindegyiküknél Kobayashi-, illetve Kawanet-pontot számoltunk, valamint megvizsgáltuk azok specificitását, szenzitivitását az intravénás immunglobulin-rezisztencia, illetve a kardiológiai szövődmények előrejelzése szempontjából. A Kobayashi-pontrendszerben 4, a Kawanet-pontrendszerben pedig 2 pont vagy annál magasabb érték jelez rizikót. Eredmények: Kawasaki-szindrómát 28 gyereknél véleményeztünk, 13 esetben észleltünk mérsékelt, 4 esetben súlyos szövődményt. 4 betegünk bizonyult intravénás immunglobulinra rezisztensnek. A rezisztencia szempontjából a Kobayashi-pontrendszer alacsony szenzitivitást (25%), illetve magas specificitást (91,6%), míg a Kawanet-pontrendszer mérsékelt szenzitivitást (50%) és specificitást (50%) mutatott. A szövődmények szempontjából hasonló eredményeket kaptunk, Kobayashi-pontrendszer: szenzitivitás: 17%; specificitás: 100%, illetve Kawanet-pontrendszer: szenzitivitás: 47%; specificitás: 45%. Következtetés: A legtöbb, nem ázsiai országban készült tanulmányhoz hasonlóan az intravénás immunglobulin-rezisztencia előrejelzésében a Kobayashi-pontrendszer vizsgálatunkban sem bizonyult hatékonynak. Ezzel szemben, magasabb szenzitivitása miatt, a Kawanet-pontrendszer intravénás immunglobulin-rezisztenciát előre jelző hatékonyságát érdemes lenne nagyobb esetszámban vizsgálni a hazai populációban is. A kardiológiai szövődmények előrejelzésére egyik pontrendszer sem bizonyult alkalmasnak. Orv Hetil. 2021; 162(47): 1885–1890. Summary. Introduction: Kawasaki disease is an immunovasculitis, which, without treatment, leads to cardiac complications. Early intravenous immunoglobulin therapy moderates complications, however, 10–20% of patients are resistant to the therapy. Numerous risk score systems are used worldwide to predict this. Objective: To assess the predictive value of the Kobayashi and Kawanet score systems regarding intravenous immunoglobulin resistance and cardiac complications in our department’s patient cohort. To our best knowledge, this is the first study in Hungary, which examines the predictive value of score systems in the case of Kawasaki disease. Method: In our study, we identified the patients treated for Kawasaki disease between January 2005 and April 2020. In each case, we calculated both the Kobayashi and the Kawanet score, and we examined their specificity and sensitivity regarding the prediction of intravenous immunoglobulin resistance and cardiac complications. In the Kobayashi score system, values above 4, in the Kawanet score system, values above 2 signal risk. Results: We identified 28 patients with Kawasaki disease. We observed moderate complications in 13, severe complications in 4 cases. 4 of our patients were resistant to intravenous immunoglobulin therapy. Regarding intravenous immunoglobulin resistance in our patient cohort, we detected low sensitivity (25%) and high specificity (91.6%) in the case of Kobayashi score, and moderate sensitivity (50%) and specificity (50%) in the case of Kawanet score. Regarding complications, we found similar results in the case of Kobayashi (sensitivity: 17%; specificity: 100%) and the Kawanet (sensitivity: 47%; specificity: 45%) score system. Conclusion: Similarly to the majority of non-Asian studies, we found the Kobayashi score system ineffective in predicting intravenous immunoglobulin resistance. However, due to its higher sensitivity, the predictive value of the Kawanet score system regarding intravenous immunoglobulin resistance is worth examining in a larger patient population in Hungary. Regarding the prediction of cardiac complications, both score systems were found to be ineffective. Orv Hetil. 2021; 162(47): 1885–1890.

Study on Guillain-Barré Syndrome in A Tertiary Level Military Hospital

Introduction: Guillain-Barre Syndrome (GBS) is an acute, frequently severe and fulminant polyradiculopathy that is autoimmune in nature and that causes acute neuromascular failure. The condition is quite common in Bangladesh. GBS is an autoimmune and post-infectious immune disease. Objectives: To see the different presentation and outcome of GBS in combined military hospital (CMH) Dhaka. Materials and Methods: This was a retrospective observational study conducted on all the GBS patients admitted in the Neurology Ward of CMH Dhaka from January 2005 to July 2010. Total 25 patients clinical and laboratory data including CSF analysis, electrophysiological study data were collected from patients’ case sheet. Results: Among the 25 GBS patients male was 22 (88%) and female 03(12%) and most common age group affected was 31-40 years comprising of 09(36%) patients. The most common types of GBS patients were acute inflammatory demyelinating polyneuropathy (AIDP) 17(68%) patients and 10(40%) patients were found to have history of upper respiratory tract infection (URTI). Albuminocytological dissociation was found in 20(80%) patients in CSF study. Intravenous immunoglobulin therapy was given to 13(52%) patients, of them 09(36%) patient needed mechanical ventilation; rest 12(48%) patients were treated conservatively. The final outcome was full recovery 22(88%) patients, 02(8%) patients had residual disability and only one patient died after 2 years of GBS. Conclusion: GBS is an important cause of peripheral neuropathy. Patient should be monitored carefully because a significant number of patients ultimately require mechanical ventilation for respiratory failure which may be of sudden onset. JAFMC Bangladesh. Vol 16, No 2 (December) 2020: 44-46

TMOD-20. CASE REPORT: THE ODYSSEY TO THE RIGHT DIAGNOSIS. SURPRISING GLIOBLASTOMA MIMICS

A 57-year-old female presented at the emergency room with acute onset aphasia but unremarkable cCT (incl. angiography and perfusion). Suspecting an ischemic stroke, she received thrombolysis with quick recovery of aphasia. Afterwards, myoclonic jerks of the face and right arm occurred leading to anticonvulsant therapy. Follow-up cMRI surprisingly demonstrated swollen T2w-hyperintense and Gd-enhancing left limbic, temporal and frontal lobes. Suspected herpes-simplex-encephalitis was treated with aciclovir despite unremarkable CSF results (no pleocytosis, no BBB disruption, negative HSV-PCR) on day 2 and follow-up (day 5). Due to persisting cognitive deficits, autoimmune limbic encephalitis was suspected, and intravenous immunoglobulin therapy was added. Three weeks later, she experienced new neurological symptoms (weakness, blurred vision, vomiting, headache). Follow-up brain MRI demonstrated a massive increase of multifocal Gd-enhancing lesions. Partial resection revealed the diagnosis of an IDH-wildtype glioblastoma (GB). Next generation oncogene panel testing demonstrated a GOPC-ROS1 fusion which is rarely found in GB. Due to the gliomatosis-like infiltration of both hemispheres, radiotherapy was deemed to be too toxic. Instead, she received two cycles of lomustine in absence of a MGMT-promotor methylation. Two months later cMRI showed a symptomatic second multilocular progression. 2nd-line therapy with a ROS-inhibitor was rejected, whereupon she died five weeks later. Our case is in several aspects peculiar: It demonstrates that rare GB-mimics (i.e. HSV- and autoimmune limbic encephalitis) can only be ruled out in a fast manner by brain biopsy. Watchful waiting may neglect fast progression of GB leading to the inability to provide optimal treatment (i.e. radiotherapy). Thrombolysis is strictly contraindicated in primary brain tumors, but was unharmful in our case most probably to the early tumor stage without relevant neoangiogenesis. Rare genetic abnormalities like ROS1-fusions which are reported mostly in childhood glioblastoma may be present and serve as a therapeutic target also in adult GB.