Inflammatory Myositis Research Articles

PET-MRI in idiopathic inflammatory myositis: a comparative study of clinical and immunological markers with imaging findings

BackgroundWe sought to determine the utility of PET-MRI in diagnosing Idiopathic Inflammatory Myositis (IIM), and look for association between FDG uptake and clinical, pathological and laboratory parameters.MethodsA retrospective, observational study was conducted on IIM patients having positive serum autoantibodies and who underwent PET-MRI (3-Tesla SIEMENS Biograph MR scanner) between 2017 and 2021. Thirty patients who underwent PET-MRI to detect systemic metastasis without muscle involvement formed the control group.ResultsIn the IIM cohort, female: male sex ratio was 1.73, mean age at diagnosis was 40.33 years, and the mean duration of illness was 7 months. 33.33% of patients had severe limb weakness. Mi2B (43.33%), Mi2A (43.33%), PL-7(10%), PL-12(6.67%), SRP (16.67%), Tif1gamma (3.33%), NxP2 (3.33%), Ro-52(40%), PM-Scl, U1-RNP, ANA (26.67%) were the serum autoantibodies identified. Using SUV max Ratio to quantify FDG uptake, PET-MRI showed a sensitivity of 100% with 93.3% specificity in diagnosing IIM.FDG uptake was maximum in proximal lower limb region followed by proximal upper limb. Multivariate regression analysis showed that the severity of muscle weakness, serum Mi2B antibody positivity and serum creatinine kinase levels had a significant positive correlation with FDG uptake (value of 0.005, 0.043, 0.042, respectively for whole-body FDG uptake). FDG uptake also showed good correlation with histopathological features and muscle MRI, but there was no significant association with treatment response. Three female patients in our cohort had primary malignancy involving the breast, uterus, and cervix.ConclusionsPET-MRI is a promising diagnostic modality for IIM. PET-MRI reflects the severity of muscle inflammation, showing good association with various clinical/laboratory parameters, histopathology, and muscle MRI. Parameters associated with severe muscle inflammation in PET-MRI—clinical severity of muscle weakness, Mi2B positivity, and serum creatine kinase levels—may be used as clinical/laboratory markers of disease severity in IIM. PET-MRI has the added advantage of detection of systemic malignancy.

41 Twenty years’ experience with juvenile dermatomyositis among Libyan children

BackgroundJuvenile inflammatory myositis are systemic autoimmune diseases of unknown aetiology that are characterized by inflammation of skeleton, muscles, skin, and internal organs. Studies from different regions have reported different incidence and age at onset suggesting that the clinical and demographic features may differ by race and geographic regions. This study aims to describe the characteristics of disease among Libyan children who have been treated in the main rheumatology unit covering most of the population in LibyaObjectivesTo describe the demographic and clinical features of patients with juvenile dermatomyositisTo determine the outcome of children with juvenile dermatomyositis and factors affecting the outcome.Patients and methodsThis is a retrospective descriptive study conducted by reviewing patient records diagnosed with juvenile dermatomyositis from 5/2000–2/2022.ResultsTwenty-one patients were included, female to male ratio was 6:1. Mean age at disease onset was 7.7 ± 2.8 years and their mean follow-up period was 4.36 ± 3.2 years. Most of the patients (14.7%) presented before 1 month of starting symptoms. Most of the patients (13,0.9%), were diagnosed as dermatomyositis, 2 (9.5%) as polymyositis, 2 (9.5%) as amyopathic myositis, and 4 (19%) as overlap syndrome. Family History of dermatomyositis was positive in 4 (19%) of patients and family history of other autoimmune diseases was positive in 5 (23.8%) patients.Around half of the patients had monocyclic disease course (11, 52.4%), 5 patients (23.8%) had polycyclic disease course and other 5 patients (23.8%) had chronic persistent disease course.MRI proximal muscles and EMG were used for diagnosis in 10 patients (48%) and 16 (76.1%) respectively, and muscle biopsy was used in 2 patients to confirm the diagnosis.One overlap patient had severe lung fibrosis on CT scan chest with restrictive lung disease and 4 patients (19%) had restrictive lung disease with normal CT scan chest.Methyleprednisolone pulses were used in 10 patients (50%) with 3 (15%) who required more than one pulse of intravenous prednisolone. Immunoglobulin was used in 12 (60%) of the patients. All patients needed oral prednisolone and methotrexate was used in 15 (75%) of the patients. Other drugs used were Azathioprine in 3 overlap patients, cyclophosphamide and Mycophenolate mofetil in one patient with overlap syndrome. Hydroxychroroquine was used in 6 (30%) of the patients.In the last visit, 12 patients (60%) were in remission, 7 (58%) of them presented before 1 month of disease onset. Fifty-eight percent of the females and 66.7% of the males were in remission. Five patients (25%) had short stature, 3 (15%) had chronic cutaneous changes, 1 (5%) had arrythmia, 2 (10%) had calcinosis, 2 (10%) had osteoporosis, and one patient died.ConclusionA female predominance was noted with age at presentation comparable to other studies.The most frequent manifestations were skin manifestations and musculoskeletal features were the second most frequent symptoms.Patients has low rate of complications with low rate of calcinosis and mortality and none of them had gastrointestinal manifestations.Males were more likely to be in remission. 41 Table 1summarizes the main clinical features at presentation:FeatureNumber and percentageSkin changes17 (81%)Facial oedema1 (4.8%)Hands and feet oedema1 (4.8%)Fever4 (19%)Generalized fatigability12 (57.1%)Arthritis11 (52.4%)Arthralgia1 (4.8%)Muscle weakness15 (71%)Dysphonia4 (19%)Contractures2 (9.5%)

ANTI-JO-1 ANTIBODY-ASSOCIATED CLINICALLY AMYOPATHIC DERMATOMYOSITIS WITH RAPIDLY PROGRESSIVE INTERSTITIAL LUNG DISEASE

ANTI-JO-1 ANTIBODY-ASSOCIATED CLINICALLY AMYOPATHIC DERMATOMYOSITIS WITH RAPIDLY PROGRESSIVE INTERSTITIAL LUNG DISEASE

VP.44 Juvenile Anti-PM Scl75 myositis with necrosis, phagocytosis, and endomysial fibrosis

The most common myositis are usually divided in two groups: the autoimmune myositis or idiopathic inflammatory myopathies (dermatomyositis, immune-mediated necrotizing myopathy, antisynthetase syndrome, and inclusion body myositis), and the overlap myositis, manifesting with inflammatory myositis with extramuscular symptoms. Most children present dermatomyositis but other types of juvenile myositis may be a diagnostic challenge. A 3 years and 11 months old girl presented subacute muscle weakness with profound hypotonia since 2 years and 6 months of age. No Gottron´s papules or heliotrope rash were observed. Magnetic resonance imaging demonstrated muscle STIR hyperintensity. Her muscle biopsy showed necrosis, phagocytosis, endomysial fibrosis, lymphohistiocytic endomysial infiltrate, strong membrane attack complex (MAC/C5b9) deposition in the sarcolemma, and weak MHC-I (HLA-ABC) sarcolemmal expression. Electron microscopy revealed some fibres with numerous autophagic vacuoles, and endothelial cells presented no tubuloreticular inclusions. On her last follow-up at the age of 10 years old, she presented good response to immunosuppressive treatment with stable muscle strength. Her serological profile, performed after treatment, revealed Anti-PM Scl75 antibodies. The appropriate diagnosis and treatment was of utmost importance for muscle function preservation.

VP.45 Clinical features of anti-mitochondrial M2 antibody-positive myositis: Case series of 17 patients

To clarify the prevalence, clinical features, treatment outcome, and severity determinants of anti-mitochondrial M2 antibody-positive myositis (AMA-M2-positive myositis). Clinical data for AMA-M2-positive myositis patients who were treated at Hokkaido University Hospital and other participating facilities from 2012 to 2021 were retrospectively evaluated. Of a total 182 patients with inflammatory myositis, 17 patients (13 females and 4 males) were AMA-M2-positive (mean age, 57.4 years; SD, 9.4 years). The median duration before diagnosis was 24 months (interquartile range [IQR]: 12-85), indicating a trend of delayed diagnosis of this disease. Weakness mainly involving paravertebral muscles, weight loss, respiratory failure, and cardiac complications were typical symptoms, but the course and severity of each symptom varied from case to case. Twelve patients (71%) had vital capacity percentage (%VC) of less than 80% and 4 patients needed ventilator support. The average body mass index (BMI) at diagnosis was 19.6 (SD, 4.1). Thirteen patients (76%) had cardiac complications and 6 patients showed lower left ventricular ejection fraction (LVEF) less than 50%. A strong correlation was found between %VC and mRS scores (R=0.68, p=0.002), indicating a trend for the degree of respiratory dysfunction to be linked to a decline in ADL. The correlation between %VC and BMI was also strong (R=0.68, p=0.002), indicating that weight loss can be an indicator of potential progression of respiratory failure. Fourteen patients received immunotherapy and CK level improved within a few months. %VC and mRS scores also improved significantly. We found significant correlations of %VC with BMI and mRS scores in AMA-M2-positive myositis patients. Immunotherapy improved the CK level, respiratory dysfunction, and ADL. We propose that CK level, %VC and BMI should be monitored as disease indicators in treatment of AMA-M2-positive myositis.

S3283 A Tough Pill to Swallow: A Case Series of Statin-Induced Necrotizing Autoimmune Myopathy Manifesting as Dysphagia and Transaminitis

Introduction: Statins are commonly used to prevent cardiovascular disease. However, a small subset of patients may develop autoimmune myopathy, a form of inflammatory myositis characterized by proximal muscle weakness and in some cases, dysphagia. We present two cases highlighting statin-induced necrotizing autoimmune myopathy (SINAM), a rare entity affecting roughly 2 cases per 1 million people. Case Description/Methods: Two Hispanic men, aged 60 and 71, presented with dysphagia and transaminitis. They reported gradual onset of proximal muscle weakness and weight loss and were taking atorvastatin 20mg and 80mg, respectively. Case 1: Labs revealed ALP 58 IU/L, AST 238 U/L, ALT 407 U/L, CK 7228 U/L, normal CRP, ESR, and HMG CoA reductase (HMGCR) antibodies 257 U/mL. EGD was unrevealing. MRI of the pelvis/femur showed patchy, symmetric intramuscular edema in leg muscles (Figure 1). Muscle biopsy revealed myonecrosis consistent with immune-mediated necrotizing myopathy. Case 2: Labs revealed ALP 79 IU/L, AST 571 U/L, ALT 373 U/L, CK 8392 U/L, CRP 19.2 mg/L, ESR 67 mm/hr, and HMGCR antibodies 367 U/mL. Muscle biopsy was not pursued. Both cases revealed negative viral hepatitis, ANA, myositis panel, smooth muscle Ab, LKM-1 Ab, total IgG, ferritin, anti-mitochondrial Ab, and alpha-1 antitrypsin. Both patients were started on high-dose steroids. Case 1 patient had minimal improvement and was subsequently started on mycophenolate and rituximab. He remains under close monitoring. Case 2 patient had persistent severe weakness and dysphagia, therefore intravenous immunoglobulin (IVIG) and mycophenolate were added. However, dysphagia persists despite a consistent decrease in muscle enzymes. He remains hospitalized and is pending PEG tube placement. (Figure) Discussion: SINAM is a rare variant of idiopathic inflammatory myopathy characterized by proximal muscle weakness and myofiber necrosis after statin exposure. Diagnosis is made with positive antibodies to HMGCR and may avoid the need for muscle biopsy in the appropriate clinical context. Cessation of statin and initiation of glucocorticoids are first-line treatments. However, many cases are refractory to steroid monotherapy. Other therapies such as IVIG and immunosuppressants serve a role in refractory disease. Alternative lipid-lowering agents should be used, and statins must not be rechallenged. These cases emphasize that muscle weakness, dysphagia and transaminitis should be evaluated diligently as early recognition can lead to initiation of life-saving treatment.Figure 1.: A-D. MRI pelvis/femur showing bilateral patchy and symmetric intramuscular edema in leg muscles.

P.91 Double seropositive inflammatory myositis with anti-PL-7 and anti-Mi-2 antibodies

P.91 Double seropositive inflammatory myositis with anti-PL-7 and anti-Mi-2 antibodies

RARE CASE OF FULMINANT OF INTERSTITIAL LUNG DISEASE SECONDARY TO IDIOPATHIC INFLAMMATORY MYOSITIS

RARE CASE OF FULMINANT OF INTERSTITIAL LUNG DISEASE SECONDARY TO IDIOPATHIC INFLAMMATORY MYOSITIS

P60 A case of autoimmune inflammatory myositis in a patient with SLC29A3 spectrum disorder

Introduction/BackgroundThe SLC29A3 gene encodes the equilibrative nucleoside transporter hENT3. SLC29A3 spectrum disorder, an autosomal recessive disorder caused by mutations in this gene, is a rare multisystem disorder resulting in cutaneous manifestations (hyperpigmented, hypertrichotic and indurated patches of skin) and a variety of systemic features, with variable phenotypic expression. Along this spectrum are H syndrome and pigmented hypertrichotic dermatosis with insulin-dependent diabetes (PHID) syndrome. The majority of cases reported in the literature have been in patients of Arab descent. We report a case of a patient with SLC29A3 mutation who later developed features of autoimmune disease and myositis.Description/MethodAn 11-year-old girl with an established diagnosis of SLC29A3 mutation, dysosteosclerosis, camptodactyly and type 1 diabetes mellitus presented with a three-week history of intermittent fever, myalgia, muscle weakness and cervical lymphadenopathy. She had a non-specific rash over her eyelids, interphalangeal joints of both hands, torso and limbs. Her movements were restricted due to myalgia. She had no evidence of arthritis. Her creatine kinase was elevated (>1400 units/litre) and MRI of her thighs revealed evidence of myositis not akin to Juvenile Dermatomyositis distribution. An extensive investigation screen showed no evidence of infections. The bone marrow was hypocellular but there was no evidence of malignancy or histiocytosis. An autoinflammatory disease was suspected and the patient was treated with three pulses of IV methylprednisolone, to which she initially responded well but soon relapsed. Anakinra was trialled but this failed as the patient’s daily fevers persisted and her muscle weakness continued to worsen. Methotrexate was commenced and did not result in the desired reduction in prednisolone dose, so the patient was started on 4-weekly infliximab infusions. Following commencement of infliximab, the patient’s fever and rash resolved; her energy levels and muscle power improved and her muscle enzymes began to normalise. Repeat MRI showed her myositis was in remission. Infliximab was stopped after 13 doses due to side effects at the end of the infusions (tachycardia, hypertension and pyrexia). In addition, it was found that she had developed antibodies against infliximab and measured serum drug levels were low. Methotrexate was eventually stopped. The patient is now off all rheumatological treatment (as of February 2019) and she has not had reactivation of any of her symptoms.Discussion/ResultsThe patient we report had insulin-dependent diabetes mellitus, dysosteosclerosis and camptodactyly, three systemic features associated with SLC29A3 mutation. She presented concomitantly with signs of autoinflammatory disease and myositis. While up to 25% of patients with SLC29A3 mutation develop autoinflammatory features, to our knowledge, this is the first reported case of myositis in a patient with this mutation. Both the inflammatory features and myositis resolved following treatment with infliximab infusions. A number of case reports have described successful treatment of inflammation in SLC29A3 mutation with IL-6 inhibitors such as tocilizumab. The complete remission demonstrated in this case following infliximab infusions points to TNF-α inhibition as another potential therapeutic target. It is also interesting to note that our patient is now off all rheumatological treatment, which contrasts with other case reports in the literature where the patients have typically required maintenance immunomodulation therapy to remain asymptomatic. This case may therefore represent a new clinical subtype of SLC29A3 mutation with an autoimmune inflammatory myositis, rather than autoinflammatory as initially suspected, that carries a better prognosis. Further case reports of myositis in SLC29A3 mutation would help to build a clearer picture of its clinical presentation, prognosis and response to biologic therapies.Key learning points/Conclusion1. SLC29A3 spectrum disorder is a rare multisystem disorder that has only recently been recognised. By presenting this case report we hope to increase awareness of this condition and network with other clinicians who have encountered inflammatory disease associated with SLC29A3 mutation, either in their clinical practice or research.2. Up to a quarter of patients with SLC29A3 mutation present with autoinflammatory disease.3. We report on the first case of autoimmune inflammatory myositis in a patient with SLC29A3 mutation, to our knowledge.4. Our patient demonstrated complete remission of her inflammatory disease following treatment with infliximab, which may represent a new clinical subtype of SLC29A3 mutation with an autoimmune, rather than autoinflammatory, myositis that carries a better prognosis.

OA17 An evolving case of Anti PM/Scl Anti body associated Aggressive Systemic Sclerosis

OA17 An evolving case of Anti PM/Scl Anti body associated Aggressive Systemic Sclerosis

Clinical Characteristics, Therapeutics, and Treatment Outcomes of Adult Patients with Anti-Melanoma Differentiation-Associated gene 5 Dermatomyositis: A Single-Center Experience from South India

Introduction: To study the clinical characteristics and treatment outcomes of patients with anti-melanoma differentiation-associated gene 5 (MDA5) dermatomyositis (DM). Methods: This was a retrospective study done between 2019 and 2021 in a tertiary care center in South India. All consecutive patients, presenting to the adult rheumatology department, classified as idiopathic inflammatory myositis (IIM), and positive for anti-MDA5 antibodies, were included in the study. Baseline characteristics of anti-MDA5 patients were compared with the data of non-MDA5 patients over the last 10 years. Clinical, biochemical, and treatment responses were assessed on follow-up. Complete and partial responders were identified using predefined criteria. Factors predicting mortality were determined by logistic regression analysis. Data were analyzed using the Statistical Package for the Social Sciences (SPSS) version 25. Results: A total of 29 adult patients of IIM were positive for anti-MDA5 antibody during the study period. The mean (±standard deviation) age of the patients was 40.3 (±13.02) years with a female:male ratio of 1.4:1. Panniculitis, calcinosis, palmar papules, and ulcerated Gottron’s were the specific cutaneous manifestations, seen in 3 (10%), 7 (24%), 4 (13.4%), and 7 (24%) patients, respectively. A total of 14 patients (48.1%) had clinically amyopathic DM and 17 patients (60.7%) had interstitial lung disease (ILD) diagnosed by high-resolution computerized tomography scan, of which organizing pneumonia was the predominant pattern. Complete response was seen in 10 patients (43.4%), while a partial response was seen in 8 patients (34.7%). Five patients died on follow-up, accounting for mortality of 21%. Age >50 years was significantly associated with mortality (P = 0.025). Gottron’s sign (P < 0.001), panniculitis (P < 0.001), calcinosis (P < 0.001), cutaneous ulcerations (P < 0.001), inflammatory arthritis (P < 0.001), and ILD (P < 0.02) were present more commonly in the anti-MDA5 IIM group, whereas myopathy (P < 0.001), elevated creatine phosphokinase (P < 0.001), and lactate dehydrogenase (P < 0.001) were more frequently present in the non-anti-MDA5 IIM group (n = 421). Conclusions: Anti-MDA5 DM represents a distinct and unique subset of IIM with characteristic clinical manifestations. Elderly age is a poor prognostic factor of mortality.

Inclusion body myositis in an older patient

Inclusion body myositis (IBM) is a condition also referred to as sporadic IBM. It is a rare variant of a broader group of diseases described under the banner of inflammatory myositis. In general, myositis/myositides tend to present with a varying cluster of symptoms such as muscular weakness, aches and/or pain. Myositis has been linked to multiple causes or triggers, but generally share the common feature (present to a variable extent) of inflammatory changes within skeletal muscle tissue. In addition, some associations (e.g. Sjogren’s disease and systemic lupus erythematosus – SLE) have been reported for IBM. Some medication classes (e.g. statins and fibrates) may present a risk to certain individuals developing drug-associated myopathies. IBM can be challenging to diagnose, and may be mistaken for many other causes of muscle weakness e.g. polymyositis. Furthermore, IBM tends to affect muscles asymmetrically, and runs a typically progressive and chronic course. Consequently, over time IBM may result in significant functional impairment, activity limitation, and participation restriction. This case report describes an older woman with a clinical diagnosis of probable IBM complicated with oesophageal dysmotility. She presented to hospital with progressive dysphagia, breathlessness, and a productive cough for which treatment was started for aspiration pneumonia. The report considers some broader ‘principles of management’ of a chronic myositis. In the current absence of a definitive curative treatment, we also discuss some realistic and practical pharmacological treatment options that may be used for the selective care of patients presenting with an incurable chronic myositis such as IBM.

A Case of Lamotrigine-Induced Inflammatory Myositis

We describe a 21-year-old woman with bipolar disorder who started taking lamotrigine 2 months ago who presented with fever and weakness. Physical examination revealed an erythematous rash over the right thenar eminence, as well as proximal muscle weakness in bilateral upper and lower extremities. Magnetic resonance imaging of the lower extremities showed patchy bilateral enhancing myositis. Right quadriceps muscle biopsy revealed inflammatory myopathy. Subsequent bone marrow biopsy demonstrated hemophagocytic lymphohistiocytosis. The patient started a prednisone taper for suspected inflammatory myositis and achieved full remission. This is one of the first descriptions of lamotrigine inducing an autoimmune response presenting as an inflammatory myositis.

Is the Rheumatoid Arthritis Impact of Disease (RAID) score a meaningful instrument for other inflammatory rheumatic diseases? A cross-sectional analysis of data from the German National Database

ObjectiveTo analyse the performance of the rheumatoid arthritis impact of disease (RAID) score in patients with ankylosing spondylitis, polymyalgia rheumatica, systemic lupus erythematosus, primary Sjögren’s syndrome, idiopathic inflammatory myositis and...

Treating Autoimmune-Related Interstitial Lung Disease With B Cell Depletion.

Autoimmune rheumatic diseases may affect vital organs with lung involvement being severe and difficult to treat manifestation. Systemic sclerosis (SSc) commonly affects the lung in the form of interstitial lung disease (ILD). ILD may be also seen in patients with rheumatoid arthritis (RA), Sjögren's syndrome (SS), systemic lupus erythematosus (SLE), inflammatory myositis (IM), antisynthetase syndrome (AS), and the ANCA-associated vasculitides (AAV). Rituximab (RTX) is an anti-CD20 B lymphocyte depleting mAb, often administered in the treatment of autoimmune rheumatic diseases. Although RTX is an off-label treatment for CTD–ILD, there are numerous reports providing data that is effective in improving both pulmonary function tests (PFTs) and chest computed tomography findings consistent with ILD. There are retrospective uncontrolled studies that assess RTX as a treatment of ILD in autoimmune diseases. These studies, apart from one, do not include patients with AAV-ILD. In SSc-ILD, in particular, there are both controlled and uncontrolled studies displaying encouraging results following B cell depletion. In addition, a number of retrospective uncontrolled studies and fewer prospective studies evaluate RTX in connective tissue diseases CTD–ILD. Although RTX is an approved treatment for AAV there are scarce only data focusing on patients with AAV-ILD specifically. The results of a handful of studies comparing treatment of CTD-ILD with RTX to treatment with other agents are in favor of RTX. Results from large, still ongoing controlled trials are awaited to ascertain RTX effects in ILD encountered in autoimmune rheumatic diseases. We review herein the results of the different RTX trials in patients with autoimmune disease–associated with ILD. Despite the heterogeneity of these studies, RTX may be considered an alternative and safe but still off-label treatment for patients with refractory CTD–ILD.

INFLAMMAGING IN MUSCLE: THE MISSING LINK BETWEEN SARCOPENIA AND IDIOPATHIC INFLAMMATORY MYOPATHIES

Ageing is associated with a progressive decline in muscle mass and quality. Inflammaging, chronic low-grade inflammation is a major causative as well as maintenance factor in age-related disorders. Idiopathic inflammatory myopathies or myositis also exhibit a chronic stage of inflammation caused by various immune and non-immune-mediated processes. This review draws parallels between the mechanisms of inflammaging, sarcopenia, and myositis, and their possible interconnection.
 We searched literature on information about myositis, sarcopenia, ageing, inflammaging, and senescence to draw parallels between the mechanisms linking myositis, sarcopenia, and inflammaging. Further, we discuss the evidence base to support that the process of senescence is hastened in an inflamed muscle [1].

Cardiovascular Abnormalities in Juvenile Dermatomyositis: A Scoping Review for the Clinical Rheumatologists.

Juvenile dermatomyositis (JDM) is a common form of inflammatory myositis in children. Vasculopathy and endothelial dysfunction play significant roles in the pathogenesis of JDM. Cardiac involvement in JDM is often underestimated, and it may be a potential indicator of poor prognosis. Cardiac dysfunction in JDM can occur both in the acute and chronic stages of the disease. Amongst the acute complications, acute congestive heart failure (CHF), myocarditis, arrhythmia, and complete heart block are common. However, these remain unrecognized due to a lack of overt clinical manifestations. Increased rates of cardiovascular abnormalities have been noted with anti-SRP and anti-Jo 1 auto-antibody positivity. Long-term follow-up studies in JDM have shown an increased prevalence of hypertension, atherosclerosis, coronary artery disease, and metabolic syndrome in adolescence and adulthood. Monitoring of body-mass index, blood pressure, and laboratory evaluation of fasting glucose and lipid profile may help in identifying metabolic syndrome in children with JDM. Steroid-sparing agents, daily exercise, and a healthy diet may reduce such long-term cardiac morbidities. Current use of multimodality imaging such as stress-echocardiography, contrast-enhanced echocardiography, cardiac magnetic resonance imaging, and positron emission tomography has increased the diagnostic yield of subclinical heart disease during acute and chronic stages of JDM. This review elaborates on different aspects of cardiac dysfunction in JDM. It also emphasizes the importance of cardiac screening in long-term follow-up of children with JDM.

Neurological Complications of Dengue Fever.

Purpose of ReviewTo discuss the neurological complications of dengue virus (DENV) infection and their pathogenesis.Recent FindingsInclude recognition of the four different serotypes of DENV and their epidemiology as well as recognition of the expanded dengue syndrome encompassing multisystem involvement in the severe form of the disease including involvement of the central nervous system (CNS). DENV is a neurotropic virus with the ability to infect the supporting cells of the CNS. Neural injury during the acute stage of the infection results from direct neuro-invasion and/or the phenomenon of antibody-dependent enhancement, resulting in plasma leakage and coagulopathy. Immune mechanisms have been implicated in the development of the delayed neurological sequelae through molecular mimicry. A myriad of neurological syndromes has been described as a result of the involvement of the CNS, the peripheral nervous system (PNS), or both.SummaryNeurological manifestations in DENV infection are increasingly being recognized, some of which are potentially fatal if not treated promptly. DENV encephalopathy and encephalitis should be considered in the differential diagnosis of other acute febrile encephalopathies, autoimmune encephalitides, and in cases of encephalopathy/encephalitis related to SARS-CoV2 infection, especially in dengue-endemic areas. Acute disseminated encephalomyelitis (ADEM) may be occasionally encountered. Clinicians should be knowledgeable of the expanded dengue syndrome characterized by the concurrent compromise of cardiac, neurological, gastrointestinal, renal, and hematopopoietic systems. Isolated cranial nerve palsies occur rather uncommonly and are often steroid responsive. These neuropathies may result from the direct involvement of cranial nerve nuclei or nerve involvement or may be immune-mediated. Even if the diagnosis of dengue is confirmed, it is absolutely imperative to exclude other well-known causes of isolated cranial nerve palsies. Ischemic and hemorrhagic strokes may occur following dengue fever. The pathogenesis may be beyond the commonly observed thrombocytopenia and include cerebral vasculitis. Involvement of ocular blood vessels may cause maculopathy or retinal hemorrhages. Posterior reversible encephalopathy syndrome (PRES) is uncommon and possibly related to dysregulated cytokine release phenomena. Lastly, any patient developing acute neuromuscular weakness during the course or within a fortnight of remission from dengue fever must be screened for acute inflammatory demyelinating polyneuropathy (AIDP), hypokalemic paralysis, or acute myositis. Rarely, a Miller–Fisher-like syndrome with negative anti-GQ1b antibody may develop.

The Utility of Myositis Specific Antibodies in Clinical Practice.

Fifteen myositis-specific antibodies have been described and characterized over the past 40 years. Approximately two thirds of patients with idiopathic inflammatory myositis have a myositis-specific antibody and only rarely more than one. Assays to detect them are now widely available within clinical practice. We describe the original description and clinical phenotype of the myositis-specific antibodies, forming the antisynthetase syndrome group, anti-MDA-5 and rapidly progressive interstitial lung disease, anti-SRP/HMGCR and necrotizing myositis, anti-TIF-1γ/NXP-2 and malignancy, anti-SAE and esophageal disease, and anti-Mi-2 and classic dermatomyositis skin disease. Clinical practice is likely to be refined, with diagnosis and classification of the idiopathic inflammatory myositides based primarily on myositis-specific antibody, rather than directed by muscle histology or the broader clinical characteristics of polymyositis and dermatomyositis. All patients newly presenting with idiopathic inflammatory myositis should be routinely screened for myositis-specific antibodies. A positive result will usefully provide diagnostic and prognostic information, guide selection of therapy, and prompt surveillance for potential organ involvement and other features, such as cancer, throughout the disease course.

Predictors of Immunogenic Response to the BNT162b2 mRNA COVID-19 Vaccination in Patients with Autoimmune Inflammatory Rheumatic Diseases Treated with Rituximab.

Treatment with rituximab (RTX) blunts SARS-CoV-2 vaccination-induced humoral response. We sought to identify predictors of a positive immunogenic response to the BNT162b2 mRNA vaccine in patients with autoimmune inflammatory rheumatic diseases (AIIRD) treated with RTX (AIIRD-RTX). We analyzed 108 AIIRD-RTX patients and 122 immunocompetent controls vaccinated with BNT162b2 mRNA participating in a multicenter vaccination study. Immunogenicity was defined by positive anti-SARS-CoV-2 S1/S2 IgG. We used a stepwise backward multiple logistic regression to identify predicting factors for a positive immunogenic response to vaccination and develop a predicting calculator, further validated in an independent cohort of AIIRD-RTX BNT162b2 mRNA vaccinated patients (n = 48). AIIRD-RTX patients who mounted a seropositive immunogenic response significantly differed from patients who did not by a lower number of RTX courses (median (range) 3 (1–10) vs. 5 (1–15), p = 0.007; lower cumulative RTX dose (mean ± SD) 6943.11 ± 5975.74 vs. 9780.95 ± 7240.12 mg, p = 0.033; higher IgG level prior to last RTX course (mean ± SD), 1189.78 ± 576.28 vs. 884.33 ± 302.31 mg/dL, p = 0.002), and extended interval between RTX treatment and vaccination, 469.82 ± 570.39 vs. 162.08 ± 160.12 days, p = 0.0009, respectively. Patients with ANCA-associated vasculitis and inflammatory myositis had a low likelihood of a seropositive immunogenic response compared to patients with rheumatoid arthritis, odds ratio (OR) 0.209, 95% confidence interval (CI) 0.046–0.96, p = 0.044 and OR 0.189, 95% CI 0.036–0.987, p = 0.048, respectively. Based on these findings, we constructed a calculator predicting the probability of a seropositive immunogenic response following BNT162b2 mRNA vaccination which performed with 90.5% sensitivity, 59.3% specificity, and 63.3% positive and 88.9% negative predictive values. In summary, the predicting calculator could guide clinicians for optimal timing of BNT162b2 mRNA vaccination in AIIRD-RTX patients.