Anti-synthetase syndrome masquerading as recurrent pneumonia.

Anti-synthetase syndrome (ASS) is a rare inflammatory myopathy with a wide variety of clinical presentations. ASS-related interstitial lung disease (ASS-ILD) presents with rapid onset and progression, which could often be confused with other more common acute processes such as pneumonia, especially when ILD can be the sole manifestation. A woman in her 50s presented with recurrent dyspnoea for 2 months requiring multiple hospital admissions, and each time, she was diagnosed with multifocal pneumonia and treated with antibiotics. On admission, the evaluation revealed a markedly elevated creatine kinase level at 3258 U/L and a CT scan of the chest revealed worsening scattered ground-glass opacities. Given the concern for ILD as the cause of antibiotic failure, she underwent bronchoscopy with bronchoalveolar lavage which revealed non-specific interstitial pneumonia. A subsequent myositis panel revealed a positive anti-Jo-1 antibody, and she was diagnosed with ASS-ILD. She completed a course of intravenous immunoglobulin and methylprednisolone and experienced significant clinical improvement with the resolution of hypoxaemia and improved polyarthralgia.ASS could often be misdiagnosed as other more common acute lung processes, as a clinically subtle course can escape detection given its rarity, as well as its non-specific and highly variable presentations. This case highlights the importance of early suspicion and consideration of performing specific autoantibody testing when evaluating patients with a suspicion of undifferentiated autoimmune condition.

Inflammatory Myopathies.

PURPOSE OF REVIEW: This article outlines the salient clinical, serologic, electrophysiologic, imaging, and histopathologic findings and treatment options for the idiopathic inflammatory myopathies, including those related to immune checkpoint inhibitors and SARS-CoV-2. RECENT FINDINGS: The classification of idiopathic inflammatory myopathies has improved with the integration of myositis-specific antibodies and histopathologic findings. Characteristic features of immune checkpoint inhibitor-related myositis have been identified, allowing early recognition and treatment of the syndrome. The COVID-19 pandemic has had a profound impact on the care of patients with idiopathic inflammatory myopathies, and several mechanisms of virus-related muscle injury have been proposed. SUMMARY: A comprehensive evaluation including clinical examination, EMG, imaging, antibody testing, muscle biopsy, and cancer screening, when appropriate, can lead to an earlier accurate diagnosis and an individualized treatment approach for patients with idiopathic inflammatory myopathies.

Rituximab-induced hypogammaglobulinaemia in patients affected by idiopathic inflammatory myopathies: a multicentre study.

OBJECTIVES: Rituximab (RTX) is an anti-CD20 chimeric monoclonal antibody recommended as off-label treatment in patients with idiopathic inflammatory myopathies (IIM). The present study aimed to evaluate changes in immunoglobulin (Ig) levels during RTX-treatment and their potential association with infections in a cohort of IIM patients. METHODS: Patients evaluated in the Myositis clinic belonging to the Rheumatology Units of Siena, Bari and Palermo University Hospitals, and treated for the first time with RTX were enrolled. Demographic, clinical, laboratory and treatment variables, including previous and concomitant immunosuppressive drugs and glucocorticoid (GC) dosage were analysed before (T0) and after 6 (T1) and 12 (T2) months of RTX treatment. RESULTS: Thirty patients (median age, IQR 56 (42-66); 22 female) were selected. During the observational period, low levels of IgG (<700 mg/dl) and IgM (<40 mg/dl) occurred in 10% and 17% of patients, respectively. However, no one showed severe (IgG<400 mg/dl) hypogammaglobulinaemia. IgA concentrations were lower at T1 than T0 (p=0.0218), while IgG concentrations were lower at T2 compared to those at baseline (p=0.0335). IgM concentrations were lower at T1 and T2 than T0 (p<0.0001), as well at T2 than T1 (p=0.0215). Three patients suffered major infections, two others had paucisymptomatic COVID-19, one suffered from mild zoster. GC dosages at T0 were inversely correlated with IgA T0 concentrations (p=0.004, r=- 0.514). No correlation was found between demographic, clinical and treatment variables and Ig serum levels. CONCLUSIONS: Hypogammaglobulinaemia following RTX is uncommon in IIM and is not related to any clinical variables, including GC dosage and previous treatments. IgG and IgM monitoring after RTX treatment does not seem useful in stratifying patients who require closer safety monitoring and prevention of infection, due to the lack of association between hypogammaglobulinaemia and the onset of severe infections.

Positive myositis-specific autoantibodies during COVID-19: a case report.

Viral infection is known to be a trigger of autoimmune diseases. Numerous cases of coronavirus disease 2019 (COVID-19) with autoimmune manifestations have been reported and several authors have highlighted the relationship between severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and autoimmune diseases. Autoimmune myopathies being one of these manifestations. A 27-year-old diabetic woman was admitted for management of acido-ketosis decompensation of her diabetes secondary to SARS-CoV-2 infection. During hospitalization, she developed muscle weakness and increased creatine kinase levels, which led us to assay the autoimmunity pattern which became positive for myositis-specific autoantibodies against single recognition particle (anti-SRP). The patient was treated with intense hydration with clinical and biological improvement and anti-SRP disappeared two months later. Positive myositis auto-antibodies are one of the autoimmune complications that could be seen during and after the SARS-CoV-2 infection.

Necrotizing autoimmune myositis following coronavirus disease 2019 infection: a case report.

BACKGROUND: Severe acute respiratory syndrome coronavirus 2 may be associated with late-onset necrotizing myositis, mimicking autoimmune inflammatory myositis; however, the exact underlying pathogenesis of severe acute respiratory syndrome coronavirus 2-induced myositis is still unclear. CASE PRESENTATION: Herein, we report a rare case of necrotizing autoimmune myositis in a 67-year-old middle eastern male following coronavirus disease 2019 infection, who presented with muscle weakness. The patient had positive anti-NXP2. The diagnosis of necrotizing autoimmune myositis was made according to muscle weakness, increased liver enzymes, electromyography and nerve conduction velocity results, and muscle biopsy. The patient underwent a full malignancy evaluation, which was unremarkable, and was discharged in relatively well condition with a daily dose of 1 mg/kg prednisolone and azathioprine 150 mg (2 mg/kg). CONCLUSION: Our report highlights the already known possible protracted sequence of coronavirus disease 2019 infection and the potential for delayed-onset necrotizing myositis.

Antisynthetase syndrome sine myositis presenting as severe acute respiratory failure.

A female in her late 40s presented to the emergency department during the COVID-19 pandemic with shortness of breath, fever and productive cough following a recent diagnosis of bilateral non-massive pulmonary emboli. She had elevated inflammatory markers and her chest X-ray revealed bilateral infiltrates. Her SARS-CoV-2 PCR was negative, and she was treated for community-acquired pneumonia. However, despite treatment she rapidly deteriorated and developed severe respiratory failure, requiring mechanical ventilation.On further investigation, she tested positive for anti-Jo-1 antibodies and a diagnosis of antisynthetase syndrome sine myositis was made. This led to successful treatment with high dose corticosteroids and intravenous immunoglobulin.This case highlights an uncommon presentation of a rare condition, as well as the benefits of working in a multidisciplinary team on the intensive care unit.

Successful treatment of COVID-19 induced neutrophilic myositis with intravenous immunoglobulin and corticosteroids: a case report.

Neutrophilic myositis (NM) is an inflammatory disorder predominantly characterized by neutrophilic infiltration in the muscles, which is suggested to be an extracutaneous manifestation of neutrophilic dermatosis (ND). NM is a rare disorder which has been occasionally reported in association with hematologic and inflammatory disorders. This case report describes a 45-year-old woman who presented with gradual muscle weakness developed after coronavirus disease 2019 (COVID-19) infection. Electromyography and nerve conduction velocity findings were compatible with inflammatory myopathy and muscle biopsy revealed neutrophilic infiltration. She was successfully treated with intravenous immunoglobulin, prednisolone 1 mg/kg/day and azathioprine 150 mg/day. In conclusion, the COVID-19 infection itself and the drugs used to treat it can cause a number of muscle disorders. Awareness of muscular involvement in COVID-19 infected patients is important for early diagnosis and appropriate treatment.

Case report of COVID-19 in an elderly patient: could SARS-CoV2 trigger myositis?

Though the exact etiology of autoimmune diseases still remains not completely known, there are various factors which are known to contribute to be trigger of autoimmune diseases. Viral infection is known to be among the other. It is known as the infection from severe acute respiratory syndrome coronavirus (SARS-CoV) and Middle East respiratory syndrome coronavirus (MERS-CoV) can be an autoimmune trigger, so, we suppose that SARS-Coronavirus (SARS-CoV-2) could be as well. Several authors have highlighted the temporal consequence between SARS-CoV-2 and autoimmune diseases. In this case report we described a patient admitted for COVID-19 pneumonia with completely negative autoimmunity at admission who developed major pulmonary interstitial disease. During the hospitalization the weaning difficulties from oxygen led us to the repetition of autoimmunity pattern which became positive (both during hospitalization then after two months from dismission) with marked positivity for specific antibodies for myositis even after the patient's infectious healing. In the follow-up, the patient continued to have asthenia and muscle weakness despite steroid therapy. She is still in follow-up and will be further evaluated over time. Can we therefore think that in this case the development of autoimmunity can persist beyond the infectious phase and determine over time the development of a real autoimmune myositis?