scholarly journals On the Nose: Anti-MDA-5 Dermatomyositis Manifesting as Perinasal Swelling

2022 ◽  
pp. 1-5
Author(s):  
Emily Molina ◽  
Lisa Christopher-Stine ◽  
Jemima Albayda
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Early Diagnosis ◽  
Lung Disease ◽  
Immunosuppressive Therapy ◽  
Clinical Presentation ◽  
High Titer ◽  
Sun Exposure ◽  
Initial Presentation ◽  
Increased Risk

The clinical presentation of dermatomyositis (DM) is diverse, with varied phenotypes that may be correlated with specific autoantibodies. The anti-melanoma differentiation-associated gene 5 (MDA5) antibody in DM is associated with an amyopathic phenotype of DM, with several unusual cutaneous manifestation and increased risk for rapidly progressive interstitial lung disease. Initial presentation may be subtle, but early diagnosis is key to initiation of proper immunosuppressive therapy. In this report, we describe perinasal edema and erythema as a presenting complaint of anti-MDA5 DM in an otherwise healthy 40-year-old woman. The edema began shortly after heavy sun exposure and was followed by painful papules in her hands and arthritis within a few weeks. She was found to have high titer of anti-CCP and anti-MDA5, and thus was diagnosed with DM and rheumatoid arthritis overlap. A CT chest, abdomen, and pelvis showed patchy ground-glass and interstitial opacities in bilateral lower lobes consistent with mild interstitial lung disease without evidence of malignancy. Perinasal cutaneous findings and arthralgias improved with initiation of prednisone. To our knowledge, this is the first report of perinasal edema as a presenting symptom for DM and should raise suspicion for MDA-5 disease.

scholarly journals Predictors of mortality in rheumatoid arthritis-associated interstitial lung disease

2015 ◽  
Vol 47 (2) ◽  
pp. 588-596 ◽  
Author(s):  
Joshua J. Solomon ◽  
Jonathan H. Chung ◽  
Gregory P. Cosgrove ◽  
M. Kristen Demoruelle ◽  
Evans R. Fernandez-Perez ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
High Resolution Computed Tomography ◽  
Predictors Of Mortality ◽  
Risk Of Death ◽  
Increased Risk ◽  
Over Time

Interstitial lung disease (ILD) is a common pulmonary manifestation of rheumatoid arthritis. There is lack of clarity around predictors of mortality and disease behaviour over time in these patients.We identified rheumatoid arthritis-related interstitial lung disease (RA-ILD) patients evaluated at National Jewish Health (Denver, CO, USA) from 1995 to 2013 whose baseline high-resolution computed tomography (HRCT) scans showed either a nonspecific interstitial pneumonia (NSIP) or a “definite” or “possible” usual interstitial pneumonia (UIP) pattern. We used univariate, multivariate and longitudinal analytical methods to identify clinical predictors of mortality and to model disease behaviour over time.The cohort included 137 subjects; 108 had UIP on HRCT (RA-UIP) and 29 had NSIP on HRCT (RA-NSIP). Those with RA-UIP had a shorter survival time than those with RA-NSIP (log rank p=0.02). In a model controlling for age, sex, smoking and HRCT pattern, a lower baseline % predicted forced vital capacity (FVC % pred) (HR 1.46; p<0.0001) and a 10% decline in FVC % pred from baseline to any time during follow up (HR 2.57; p<0.0001) were independently associated with an increased risk of death.Data from this study suggest that in RA-ILD, disease progression and survival differ between subgroups defined by HRCT pattern; however, when controlling for potentially influential variables, pulmonary physiology, but not HRCT pattern, independently predicts mortality.

scholarly journals Is incident rheumatoid arthritis interstitial lung disease associated with methotrexate treatment? Results from a multivariate analysis in the ERAS and ERAN inception cohorts

BMJ Open ◽  
2019 ◽  
Vol 9 (5) ◽  
pp. e028466 ◽  
Author(s):  
Patrick Kiely ◽  
A D Busby ◽  
E Nikiphorou ◽  
K Sullivan ◽  
D A Walsh ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Secondary Outcome ◽  
Primary Analysis ◽  
Early Ra ◽  
Increased Risk ◽  
Extended Analysis ◽  
Specific Factors ◽  
New Diagnosis

ObjectivesTo assess predictive factors for rheumatoid arthritis interstitial lung disease (RA-ILD) in two early rheumatoid arthritis (RA) inception cohorts with a focus on methotrexate (MTX) exposure.DesignMulticentre prospective early RA inception cohort studies; the early RA study (ERAS) and the early RA network (ERAN).SettingSecondary care, ERAS nine centres, ERAN 23 centres in England, Wales and Ireland.ParticipantsPatients with new diagnosis of RA, n=2701. Standardised data including demographics, drug therapies and clinical outcomes including the presence of RA-ILD were collected at baseline, within 3–6 months, at 12 months and annually thereafter.Primary and secondary outcome measuresPrimary outcome was the association of MTX exposure on RA-ILD diagnosis. Secondary outcomes were the association of demographic, comorbid and RA-specific factors on RA-ILD diagnosis and the association of MTX exposure on time to RA-ILD diagnosis.ResultsOf 92 eligible ILD cases, 39 occurred in 1578 (2.5%) MTX exposed and 53 in 1114 (4.8%) non-MTX exposed cases. The primary analysis of RA-ILD cases only developing after any conventional synthetic disease-modifying antirheumatic drug treatment (n=67) showed MTX exposure not to be associated with incident RA-ILD (OR 0.85, 95% CI 0.49 to 1.49, p=0.578) and a non-significant trend for delayed ILD diagnosis (OR 0.54, 95% CI 0.28 to 1.06, p=0.072). In an extended analysis including RA-ILD cases present at RA diagnosis (n=92), MTX exposure was associated with a significantly reduced risk of incident RA-ILD (OR 0.48, 95% CI 0.3 to 0.79, p=0.004) and longer time to ILD diagnosis (OR 0.41, 95% CI 0.23 to 0.75, p=0.004). Other independent baseline associations with incident RA-ILD were higher age of RA onset, ever smoking, male gender, rheumatoid nodules and longer time from first RA symptom to first outpatient visit.ConclusionsMTX treatment was not associated with an increased risk of RA-ILD diagnosis. On the contrary, evidence suggested that MTX may delay the onset of ILD.

Rheumatoid arthritis-associated interstitial lung disease – an underestimated, complex and interdisciplinary problem in everyday clinical practice

2020 ◽  
Vol 13 (4) ◽  
pp. 431-441
Author(s):  
Sebastian Majewski ◽  
Joanna Makowska
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Symptoms ◽  
Future Research ◽  
Clinical Aspects ◽  
Scientific Review ◽  
Disease Modifying Drugs ◽  
Risk Of Death ◽  
Increased Risk

Rheumatoid arthritis (RA) is a chronic, systemic inflammatory connective tissue disease affecting 0.5–1% of the general population. Interstitial lung disease (ILD) is a serious complication of RA, leads to a deterioration in the quality of life, increases the risk of hospitalization and premature death. The clinical course of RA-associated ILD (RA-ILD) varies from interstitial lesions involving little areas of the lung which do not lead to clinical symptoms, to progressive interstitial lesions that may lead to acute respiratory exacerbation, development of respiratory failure, and death. The main risk factor for the development of ILD in the course of RA, apart from older age and male sex, is the high activity of the underlying disease. Effective treatment of RA, aimed at the goal of remission, may therefore be a preventive method for the development of RA-ILD. Due to non-specific symptoms, the diagnosis of RA-ILD is often overlooked. Despite significant morbidity and increased risk of death due to RA-ILD, currently, we do not have any guidelines on the management of this clinical situation. Moreover, the problem is even more complicated due to the possibility of potential pneumotoxicity of many disease-modifying drugs and their unclear effectiveness regarding lung involvement in RA. Taken together, optimal management of a patient with RA-ILD is a clinical challenge. There is an urgent need to clarify several clinical aspects concerning the early diagnosis, monitoring, establishing indications for treatment, and choice of appropriate therapeutic management. In this work, we performed a scientific review of the current state of knowledge on RA-ILD and indicated the directions of future research needed aiming at improving patients’ care.

scholarly journals Adolescent-onset anti-MDA5 antibody-positive juvenile dermatomyositis with rapidly progressive interstitial lung disease and spontaneous pneumomediastinum: a case report and literature review

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Tsz-Wing Yeung ◽  
Kai-Ning Cheong ◽  
Yu-Lung Lau ◽  
Kei-Chiu Niko Tse
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Immunosuppressive Therapy ◽  
High Mortality ◽  
Inflammatory Myopathy ◽  
Unknown Etiology ◽  
Rapid Progression ◽  
Muscle Involvement ◽  
Spontaneous Pneumomediastinum ◽  
Increased Risk

Abstract Background Dermatomyositis with positive anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody has a distinct phenotype associated with small hand joint arthritis, mucocutaneous ulceration, palmar papules and less muscle involvement. It is also associated with increased risk of rapidly progressive interstitial lung disease (RP-ILD) and has a high mortality rate in adults. There is evidence that cases complicated with spontaneous pneumomediastinum (PNM) have an increase in mortality. While most of the evidence for this rare disease is derived from the adult literature, we report a case diagnosed in an adolescent complicated with both RP-ILD and PNM with a good outcome after aggressive immunosuppressive therapy. Our case also illustrates the potential challenges in diagnosis of this condition in the setting of non-specific clinical manifestations, the need for a high index of suspicion, and the importance of testing for myositis-specific antibodies (MSA) early to aid in diagnosis given the risk of rapid progression in these patients. Case presentation A 16-year-old Chinese female presented with fever and cough for 1 day, and finger swelling for 3 weeks. Physical examination revealed arthritis of fingers and wrists, ulcers and palmar papules over fingers, hyperpigmentation of interphalangeal joints, and rash over the neck. The diagnosis of dermatomyositis was made 1 month later with the onset of malar rash, Gottron’s papules, calcinosis and myalgia. The diagnosis was supported by the presence of anti-MDA5 antibody and evidence of inflammatory myopathy on magnetic resonance imaging. In retrospect, she already had interstitial lung disease at first presentation manifested as cough and opacity on chest radiograph, which was later confirmed with chest computed tomography. She was treated according to adult guidelines with steroid and calcineurin inhibitor. Her disease was resistant to initial therapy and was complicated by RP-ILD and spontaneous PNM. Intensive immunosuppressive therapy including cyclophosphamide and rituximab were required to induce remission. Conclusions Recognition of distinct clinical features of anti-MDA5 antibody-positive dermatomyositis and testing for MSA is crucial in patients with skin ulceration and abnormal pulmonary findings of unknown etiology, as prompt diagnosis with early aggressive treatment and anticipation of complications could make a difference in the outcome of this disease with high mortality.

scholarly journals Rheumatoid Arthritis Management for General Internists

2016 ◽  
Vol 8 ◽  
Author(s):  
Mitchell Uh MD FRCPC ◽  
David Collins MD FRCPC ABIM
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Joint Destruction ◽  
Morbidity And Mortality ◽  
Inflammatory Disorder ◽  
General Internists ◽  
Canadian Population ◽  
Increased Risk ◽  
Significant Disability

Rheumatoid arthritis (RA) is a chronic systemic inflammatory disorder that causes progressive joint destruction, deformity, and significant disability. In addition to arthritis, RA is associated with numerous extra-articular features with attendant morbidity and mortality. These include but are not limited to ocular inflammation, interstitial lung disease, pleuropericardial disease, vasculitis, accelerated atherosclerosis, and increased risk of lymphoma. The prevalence of RA in the Canadian population is approximately 1%.

scholarly journals Lifestyle and clinical risk factors for incident rheumatoid arthritis-associated interstitial lung disease

2020 ◽  
pp. jrheum.200863
Author(s):  
Vanessa L. Kronzer ◽  
Weixing Huang ◽  
Paul F. Dellaripa ◽  
Sicong Huang ◽  
Vivi Feathers ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Risk Factors ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Functional Status ◽  
Area Under The Curve ◽  
Clinical Risk Factors ◽  
Logistic Regression Models ◽  
Clinical Risk ◽  
Increased Risk

Objective To determine the association between novel lifestyle factors on risk of rheumatoid arthritis-associated interstitial lung disease (RA-ILD), define the threshold at which smoking increases RA-ILD risk, and calculate the degree to which known lifestyle and clinical factors predict RA-ILD. Methods This nested case-control study matched incident RA-ILD cases to RA non-ILD controls on age, sex, RA duration, rheumatoid factor, and time from exposure assessment to RA-ILD. Exposures included education, body mass index (BMI), smoking, anti-cyclic citrullinated peptide, race, joint erosions, rheumatoid nodules, C-reactive protein (CRP), disease activity score, functional status, disease-modifying anti-rheumatic drug use, and glucocorticoid use. Odds ratios (OR) for each exposure on risk of RA-ILD were obtained from logistic regression models. Area under the curve (AUC) was calculated based all lifestyle and clinical exposures. Results We identified 84 incident RA-ILD cases and 233 matched controls. After adjustment, obesity, high-positive CRP (≥10 mg/L), and poor functional status (MDHAQ ≥1) were associated with increased risk of RA-ILD (OR 2.42, 95% confidence interval [CI] 1.11-5.24 vs. normal BMI; OR 2.61, 95% CI 1.21-5.64 vs. CRP <3mg/L; OR 3.10, 95% CI 1.32-7.26 vs. MDHAQ <0.2). Smoking 30 pack-years or more was strongly associated with risk of RA-ILD compared to nonsmokers (OR 6.06, 95% CI 2.72-13.5). Together, lifestyle and clinical risk factors for RA-ILD had an AUC of 0.79 (95% CI 0.73-0.85). Conclusion Obesity, CRP, functional status, and extensive smoking may be novel risk factors for RA-ILD, useful for RA-ILD risk assessment and prevention. The overall ability to predict RA-ILD remains modest.

scholarly journals The Multifaceted Aspects of Interstitial Lung Disease in Rheumatoid Arthritis

2013 ◽  
Vol 2013 ◽  
pp. 1-13 ◽  
Author(s):  
Lorenzo Cavagna ◽  
Sara Monti ◽  
Vittorio Grosso ◽  
Nicola Boffini ◽  
Eva Scorletti ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Disease Process ◽  
Tnf Alpha ◽  
Increased Risk ◽  
Nonspecific Interstitial Pneumonia ◽  
Citrullinated Peptide

Interstitial lung disease (ILD) is a relevant extra-articular manifestation of rheumatoid arthritis (RA) that may occur either in early stages or as a complication of long-standing disease. RA related ILD (RA-ILD) significantly influences thequoad vitamprognosis of these patients. Several histopathological patterns of RA-ILD have been described: usual interstitial pneumonia (UIP) is the most frequent one, followed by nonspecific interstitial pneumonia (NSIP); other patterns are less commonly observed. Several factors have been associated with an increased risk of developing RA-ILD. The genetic background plays a fundamental but not sufficient role; smoking is an independent predictor of ILD, and a correlation with the presence of rheumatoid factor and anti-cyclic citrullinated peptide antibodies has also been reported. Moreover, bothexnovooccurrence and progression of ILD have been related to drug therapies that are commonly prescribed in RA, such as methotrexate, leflunomide, anti-TNF alpha agents, and rituximab. A greater understanding of the disease process is necessary in order to improve the therapeutic approach to ILD and RA itself and to reduce the burden of this severe extra-articular manifestation.

scholarly journals High-titer rheumatoid factor seropositivity predicts mediastinal lymphadenopathy and mortality in rheumatoid arthritis-related interstitial lung disease

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Albina Tyker ◽  
Iazsmin Bauer Ventura ◽  
Cathryn T. Lee ◽  
Rachel Strykowski ◽  
Nicole Garcia ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Lung Function ◽  
Lung Disease ◽  
Rheumatoid Factor ◽  
Cox Regression ◽  
Mediastinal Lymph Node ◽  
High Titer ◽  
Mediastinal Lymphadenopathy ◽  
High Morbidity

AbstractRheumatoid arthritis-related interstitial lung disease (RA-ILD) is a common connective tissue disease-related ILD (CTD-ILD) associated with high morbidity and mortality. Although rheumatoid factor (RF) seropositivity is a risk factor for developing RA-ILD, the relationship between RF seropositivity, mediastinal lymph node (MLN) features, and disease progression is unknown. We aimed to determine if high-titer RF seropositivity predicted MLN features, lung function impairment, and mortality in RA-ILD. In this retrospective cohort study, we identified patients in the University of Chicago ILD registry with RA-ILD. We compared demographic characteristics, serologic data, MLN size, count and location, and pulmonary function over 36 months among patients who had high-titer RF seropositivity (≥ 60 IU/ml) and those who did not. Survival analysis was performed using Cox regression modeling. Amongst 294 patients with CTD-ILD, available chest computed tomography (CT) imaging and serologic data, we identified 70 patients with RA-ILD. Compared to RA-ILD patients with low-titer RF, RA-ILD patients with high-titer RF had lower baseline forced vital capacity (71% vs. 63%; P = 0.045), elevated anti-cyclic citrullinated peptide titer (122 vs. 201; P = 0.001), CT honeycombing (50% vs. 80%; P = 0.008), and higher number of MLN ≥ 10 mm (36% vs. 76%; P = 0.005). Lung function decline over 36 months did not differ between groups. Primary outcomes of death or lung transplant occurred more frequently in the high-titer RF group (HR 2.8; 95% CI 1.1–6.8; P = 0.028). High-titer RF seropositivity was associated with MLN enlargement, CT honeycombing, and decreased transplant-free survival. RF titer may be a useful prognostic marker for stratifying patients by pulmonary disease activity and mortality risk.

scholarly journals Methotrexate and rheumatoid arthritis associated interstitial lung disease

2020 ◽  
pp. 2000337
Author(s):  
Pierre-Antoine Juge ◽  
Joyce S. Lee ◽  
Jessica Lau ◽  
Leticia Kawano-Dourado ◽  
Jorge Rojas Serrano ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Case Control Study ◽  
Meta Analysis ◽  
High Resolution Computed Tomography ◽  
Analysis Techniques ◽  
Discovery Sample ◽  
Increased Risk ◽  
Control Study

Question addressed by the studyMethotrexate (MTX) is a key anchor drug for rheumatoid arthritis (RA) management. Fibrotic interstitial lung disease (ILD) is a common complication of RA. Whether MTX exposure increases the risk of ILD in patients with RA is disputed. We aimed to evaluate the association of prior MTX use with development of RA-ILD.MethodsThrough a case-control study design with discovery and international replication samples, we examined the association of MTX exposure with ILD in 410 patients with chronic fibrotic ILD associated with RA (RA-ILD) and 673 patients with RA without ILD. Estimates were pooled over the different samples using meta-analysis techniques.ResultsAnalysis of the discovery sample revealed an inverse relationship between MTX exposure and RA-ILD (adjusted odds ratio [OR], 0.46; 95% confidence interval [CI], 0.24–0.90; p=0.022), which was confirmed in the replication samples (pooled adjusted OR, 0.39; 95% CI, 0.19–0.79; p=0.009). The combined estimate using both the derivation and validation samples revealed an adjusted OR of 0.43 (95% CI, 0.26–0.69; p=0.0006). MTX ever users were less frequent among patients with RA-ILD compared to those without ILD, irrespective of chest high resolution computed tomography pattern. In patients with RA-ILD, ILD detection was significantly delayed in MTX ever users compared to never users (11.4±10.4 years and 4.0±7.4 years, respectively; p<0.001).Answer to the QuestionOur results suggest that MTX use is not associated with an increased risk of RA-ILD in patients with RA, and that ILD was detected later in MTX treated patients.

Sign in / Sign up

Export Citation Format

Share Document