ABSTRACT
Background/Aims Myasthenia gravis (MG) is an antibody-mediated autoimmune disease targeting proteins at the postsynaptic membrane of the neuromuscular junction. MG is thought to occur in genetically susceptible individuals following an environmental trigger. SARS-CoV-2 infection has been associated with new-onset autoimmune disease, new-onset MG, and exacerbations of pre-existing MG, with molecular mimicry between SARS-CoV-2 epitopes and autoantigen-induced autoreactivity thought to be part of the underlying mechanism. We report a case of newonset ocular MG following first dose Pfizer-BioNTech BNT162b2 SARS-COV2 vaccination which was referred to rheumatology as suspected mononeuritis multiplex. Methods A 53-year-old man of East Asian ethnicity presented to the emergency department (ED) with sudden onset diplopia and left lateral gaze restriction 7 days after receiving his first dose of the Pfizer-BioNTech BNT162b2 SARS-COV2 vaccination. He had longstanding myopia and dry eyes but no other medical history, no regular medications or significant family history. He was a current smoker, with a 50-pack year history. He did not drink alcohol or use any recreational drugs. He was found to have an isolated left VI cranial nerve (CN) palsy with an otherwise normal ocular and physical examination. Blood tests were unremarkable apart from raised cholesterol, and he was discharged with a suspected self-limiting microvascular CN lesion. Three weeks later he presented to ED with worsening diplopia, increasingly restricted eye movements, headache, nausea, vomiting and blurred vision. Ophthalmology assessment noted new right sided CN III and VI palsy, persistent left CN VI palsy, and vertical diplopia in all fields of gaze. Neurological and physical examination were normal. Bloods including an autoimmune screen were unremarkable. SARS-CoV-2 Spike antibodies were positive consistent with SARS-CoV-2 vaccination but not infection. Intracranial and thoracic imaging were unremarkable. He was referred to and seen by both rheumatology and neurology as a case of suspected mononeuritis multiplex. Results A diagnosis of ocular MG was confirmed with positive serum acetylcholine receptor antibodies, and he was started on prednisolone, and pyridostigmine to good effect. Daily forced vital capacity (FVC) showed no respiratory muscle involvement, and nerve conduction studies and electromyography were normal, excluding secondary generalisation. Conclusion A review of the literature found 14 reported cases of new-onset MG all within 4 weeks following SARS-CoV-2 vaccine. Whilst these cases provide interesting insights into the pathogenesis of autoimmune conditions such as MG, they are not epidemiological studies to inform vaccine safety. Ultimately, current evidence suggests that the risks of SARS-COV-2 infection outweigh the risk of vaccine-related adverse events, therefore we suggest clinicians should be aware of potential new-onset autoimmune conditions, but support the safety of SARSCOV2 vaccination. Further, research into possible immunological mechanisms behind this phenomenon, including identifying potential epitopes inducing molecular mimicry, could help establish the likelihood of a causative link.
ABSTRACT
Background: The world is currently rocking to and fro in the midst of the COVID-19 viral storm and vaccinations have played a pivotal role in calming this.Although COVID-19 vaccines have been thoroughly assessed and studied before being rolled out to the general population, there have been reports of post vaccination complications in limited number of subjects strongly associated with COVID-19 vaccinations[1]. Objectives: To report a case of severe ANCA associated vasculitis after COVID-19 vaccination. Methods: A case report and discussion. Results: In view of this, we report the case of a 77 year old caucasian male who developed severe ANCA associated vasculitis (AAV) after two doses of Astra-Zeneca vaccine and one booster dose of Pfzer COVID-19 booster. He presented with acute onset infammatory arthritis with mononeuritis multiplex with bilateral foot drop and left radial and ulnar nerve forearm weakness in typical asymmetrical pattern two weeks after the Pfzer vaccination. He had a raised MPO-ANCA titre of 66 IU/ml, C-reactive protein of 131mg/L and reactive thrombocytosis of 458 X 10 9/L. Nerve conduction study confrmed mononeuritis multiplex in the bilateral peroneal nerves and left radial and ulnar nerve. A total body CT had excluded malignancy and paraneoplastic associations and Gullian-Barre diagnosis was also excluded. The patient was treated with 3 days of intravenous methylprednisolone 1g daily then given intravenous Rituximab 1g, two weeks apart. He is currently undergoing rehabilitation in view of the vasculitic neuropathy from his diagnosis. Conclusion: Diagnosis of AAV is often delayed or missed by other medical specialties due to its varied presentation. AAV should be suspected in a patient with paraesthesia/weakness in keeping with mononeuritis multiplex or other peripheral neuropathy in the absence of an alternative explanation (e.g. diabetes,B12 defciency) and in particular with a wrist or foot drop.Exposure to certain drugs and substances of abuse such as cocaine, hydralazine and propylthiouracil has been implicated with AAV.While short-term side effects of COVID-19 vaccine resemble those of other vaccines, long-term side effects remain unknown[2]. Rare side effects continue to surface as millions of people receive COVID-19 vaccines around the world.
ABSTRACT
Case report-IntroductionCOVID-19 pandemic affected medical practise significantly and caused difficulties in accessing necessary investigations at the appropriate time. As of March 2020, NHS England issued measures to redirect staffs and resources in preparation for the rising cases of coronavirus. As a result of this, non-urgent tests/treatments were put on hold. We present a new case of EGPA admitted to our district general hospital during the COVID-19 pandemic to highlight the challenges faced. The diagnosis was reached based on clinical judgment in the absence of some confirmatory tests as well as the decision of starting immunosuppressant treatment during the pandemic.Case report-Case descriptionA 41-years-old lady with a background of well-controlled asthma, presented with five days history of paraesthesia and swelling in both legs. She also reported mild pleuritic chest pain, which radiated to her left arm. Physical examination revealed left foot drop. She had reduced sensation on the L5-S1 dermatomal distribution with absent ankle reflex and reduced knee reflex of her left leg. Her left calf was swollen and tender. The rest of her examination was unremarkable.Baseline blood revealed raised WCC of 19.3 with significant eosinophilia (10). CRP and ESR were 135 mg/L and 48mm/hr, respectively. Electrocardiogram showed new T-wave inversion in the anterolateral leads with significantly raised troponin levels. There was ground glass appearance in both lungs, keeping with suspected COVID-19 and no evidence of pulmonary embolus was found on CTPA. MRI spine confirmed no evidence of cauda equina compression. Deep vein thrombosis was also excluded with US doppler.She was treated as myocarditis and pneumonia secondary to probable COVID-19 infection. Echocardiogram revealed severe LVSD (EF < 35%) with no LV hypertrophy. Three days later, she became acutely breathless and required high flow oxygen. New bilateral basal crackles were found on auscultation. Her antibiotic regimes were escalated to intravenous infusion.A revised CT report suggested the findings may correlate with eosinophilic pneumonia or EGPA. MRI of lower legs proved muscular oedema in bilaterally, which was suggestive of myositis with fasciitis. There was no significant change on the thigh musculature. CK level was slightly elevated (403 IU/L). Urinalysis was positive for blood (3+). Given the strong clinical suspicion of EPGA, a decision to start high dose steroid therapy was made, despite the pending immunology results. After the third dose of the methylprednisolone, pulsed cyclophosphamide was started along with high dose oral prednisolone. The patient was discharged home following significant clinical improvement.Case report-DiscussionThis patient has fulfilled 4 out of 6 criteria of ACR 1990 classification for EGPA, which are eosinophilia, bronchial asthma, mononeuritis multiplex and pulmonary infiltrates on radiological images. However, in the context of current pandemic, these changes on chest CT findings could also be suggestive of COVID-19 pneumonitis.At present, there is no reliable test for COVID-19. Even though RT-PCR testing has been the gold standard for diagnosing suspected cases, the clinical sensitivity and specificity of these tests are variable. A negative test may not rule out infection. In our case, the patient was tested twice at separate times to rule out the possibility of COVID-19 infection.During the pandemic, there is extremely limited access to some confirmatory tests. We were not able to perform nerve conduction studies on our patient as the service was suspended, instead, we sought neurologist's review to confirm the mononeuritis multiplex. We also sought advice from haematologist to rule out the possibility of hyper-eosinophilic syndrome as bone marrow biopsy was unavailable. The screen for atypical pneumonia, aspergillosis, viruses, and tuberculosis were negative. By excluding the alternative diagnoses related to eosinophilia, we concluded that this was likely to be a case of first presentation EGPA.Our next obstacle was intr ducing remission-induction regimens during COVID-19 pandemic. BSR does not recommend starting new treatment due to the increased risk of infection. We had to weigh out the benefits and risks of initiating immunosuppression. Our patient was made aware of the potential risks involved which include severe infection with COVID-19. She was also shifted to a side room with strict infection control precautions and PCP prophylaxis prescribed before starting pulsed methylprednisolone and cyclophosphamide. Fortunately, her neurological symptoms resolved after three days of steroid therapy. Eosinophils count dropped within 1 day to zero, after the first dose of IV methylprednisolone.Case report-Key learning pointsDespite the rising cases of COVID-19 infection, it is essential to keep an open mind and consider alternative diagnosis if a patient did not respond to conventional treatment. As EGPA and COVID-19 pneumonia share similar clinical and radiological presentation, clinical judgement is essential when making the diagnosis as the treatments for both conditions are vastly different. When EGPA is suspected, a multidisciplinary team should be involved in the evaluation of different organ involvements as well as ruling out other causes of eosinophilia. The role of specialists' inputs is extremely important in reaching the diagnosis, especially with limited access to the usual confirmatory tests due to reduced services during the pandemic.In addition, when there is an increased risk of infection such as during the COVID-19 pandemic, it is essential to weigh up the benefits and risks of commencing immunosuppressant treatment carefully. Patients need to be involved in the decision-making process as well as take precautions to minimise the risk of infection. The decision to start remission induction regimes should not be delayed if there is a presence of life or organ threatening disease manifestations in EGPA patients. Our patient has had a life-threatening disease because of multi-organ involvements (cardiac, pulmonary, and neurological systems).