scholarly journals Combination Therapy with Rituximab, Tofacitinib and Pirfenidone in a Patient with Rapid Progressive Interstitial Lung Disease (RP-ILD) Due to MDA5 Antibody-Associated Dermatomyositis: A Case Report

Medicina ◽  
2021 ◽  
Vol 57 (12) ◽  
pp. 1358
Author(s):  
Tsai-Hung Yen ◽  
Chih-Wei Tseng ◽  
Kao-Lun Wang ◽  
Pin-Kuei Fu
Keyword(s):  
Interstitial Lung Disease ◽  
Combination Therapy ◽  
Lung Disease ◽  
Poor Prognosis ◽  
Clinical Symptoms ◽  
Organizing Pneumonia ◽  
Laboratory Examination ◽  
Sustainable Improvement ◽  
Respiratory Conditions ◽  
Radiographic Changes

Anti-melanoma differentiation-associated protein 5 (MDA5)-positive rapidly progressive interstitial lung disease (RP-ILD) is associated with poor prognosis, and the most effective therapeutic intervention has not been established. Herein we report a case of a 45-year-old female patient who presented with myalgia, Gottron’s papules with ulceration, and dyspnea on exertion which became aggravated within weeks. Laboratory examination and electromyography confirmed myopathy changes, and a survey of myositis-specific antibodies was strongly positive for anti-MDA5 antibody. High-resolution chest tomography suggested organizing pneumonia with rapidly progressive changes within the first month after diagnosis of the disease. Anti-MDA5-associated dermatomyositis with RP-ILD was diagnosed. Following combination therapy with rituximab, tofacitinib and pirfenidone, clinical symptoms, including cutaneous manifestation, respiratory conditions and radiographic changes, showed significant and sustainable improvement. To our knowledge, this is the first reported case of anti-MDA5-associated dermatomyositis with RP-ILD successfully treated with the combination of rituximab, tofacitinib, and pirfenidone.

scholarly journals Interstitial lung disease has a poor prognosis in rheumatoid arthritis: results from an inception cohort

Rheumatology ◽  
2010 ◽  
Vol 49 (8) ◽  
pp. 1483-1489 ◽  
Author(s):  
G. Koduri ◽  
S. Norton ◽  
A. Young ◽  
N. Cox ◽  
P. Davies ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Poor Prognosis ◽  
Inception Cohort

MDA-5 dermatomyositis complicated by interstitial lung disease and cutaneous ulcers: successful treatment with corticosteroids, mycophenolate mofetil and intravenous immunoglobulin

2020 ◽  
Vol 13 (9) ◽  
pp. e236431
Author(s):  
Kelli Stager ◽  
Leanna Wise
Keyword(s):  
Mycophenolate Mofetil ◽  
Interstitial Lung Disease ◽  
Early Diagnosis ◽  
Lung Disease ◽  
Intravenous Immunoglobulin ◽  
Poor Prognosis ◽  
Aggressive Treatment ◽  
Cutaneous Manifestations ◽  
Severity Of Disease ◽  
Aggressive Management

Antimelanoma differentiation-associated gene 5 (MDA-5) dermatomyositis is a subtype of dermatomyositis that is associated with rapidly progressive interstitial lung disease (RP-ILD), as well as with a variety of cutaneous manifestations. Patients with MDA-5 dermatomyositis tend to have a poor prognosis that is often attributed to the high rates of concurrent RP-ILD. Given the severity of disease, early diagnosis and aggressive management is pivotal. We present a case of a 40-year-old woman diagnosed with MDA-5 dermatomyositis who presented with weakness, painful cutaneous ulcerations and interstitial lung disease. She was treated with monthly intravenous Ig (IVIg), weight-based prednisone and mycophenolate mofetil (MMF). After approximately 2 years of treatment, her interstitial lung disease remains stable and she has had significant improvement in weakness and cutaneous ulcerations. Our case provides evidence for early and aggressive treatment of MDA-5 dermatomyositis with a combination of weight-based prednisone, MMF and IVIg.

scholarly journals Combination Therapy with Nintedanib and Sarilumab for the Management of Rheumatoid Arthritis Related Interstitial Lung Disease

2020 ◽  
Vol 2020 ◽  
pp. 1-4 ◽  
Author(s):  
Caterina Vacchi ◽  
Andreina Manfredi ◽  
Giulia Cassone ◽  
Carlo Salvarani ◽  
Stefania Cerri ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Combination Therapy ◽  
Lung Disease ◽  
Therapeutic Approach ◽  
Negative Impact ◽  
Case Series ◽  
Joint Involvement ◽  
Sustained Remission ◽  
Systemic Inflammatory Disease

Rheumatoid arthritis (RA) is a chronic, systemic, inflammatory disease characterized by joint and extra-articular involvement. Among them, interstitial lung disease (ILD) is one of the most common and severe extra-articular manifestations, with a negative impact on both therapeutic approach and overall prognosis. ILD can occur at any point of the natural history of RA, sometimes before the appearance of joint involvement. Since no controlled studies are available, the therapeutic approach to RA-ILD is still debated and based on empirical approaches dependent on retrospective studies and case series. Here, we report the case of a 75-year-old patient affected by RA complicated by ILD successfully treated with a combination therapy of an antifibrotic agent, nintedanib, and an inhibitor of IL-6 receptor, sarilumab. We obtained a sustained remission of the joint involvement and, simultaneously, a stabilization of the respiratory symptoms and function, with a good safety profile. To date, this is the first report describing a combination therapy with nintedanib and a disease-modifying antirheumatic drug (DMARD) for the management of RA complicated by ILD. Future prospective studies are needed to better define efficacy and safety of this approach in the treatment of these subjects.

The Pulmonary Histopathology of Anti-KS Transfer RNA Synthetase Syndrome

2015 ◽  
Vol 139 (1) ◽  
pp. 122-125 ◽  
Author(s):  
Frank Schneider ◽  
Rohit Aggarwal ◽  
David Bi ◽  
Kevin Gibson ◽  
Chester Oddis ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Connective Tissue Disorder ◽  
Transfer Rna ◽  
Organizing Pneumonia ◽  
Antisynthetase Syndrome ◽  
Pulmonary Manifestations

Context The clinical spectrum of the antisynthetase syndromes (AS) has been poorly defined, although some frequently present with pulmonary manifestations. The anti-KS anti–asparaginyl-transfer RNA synthetase syndrome is one in which pulmonary interstitial lung disease is almost always present and yet the histopathologic spectrum is not well described. Objective To define the morphologic manifestations of pulmonary disease in those patients with anti-KS antiasparaginyl syndrome. Design We reviewed the connective tissue disorder registry of the University of Pittsburgh and identified those patients with anti-KS autoantibodies who presented with interstitial lung disease and had surgical lung biopsies. Results The 5 patients with anti-KS antisynthetase syndrome were usually women presenting with dyspnea and without myositis, but with mechanic's hands (60%) and Raynaud phenomenon (40%). They most often presented with a usual interstitial pneumonia pattern of fibrosis (80%), with the final patient displaying organizing pneumonia. Conclusions Pulmonary interstitial lung disease is a common presentation in patients with the anti-KS–antisynthetase syndrome, who are often women with rather subtle or subclinical connective tissue disease, whereas the literature emphasizes the nonspecific interstitial pneumonia pattern often diagnosed clinically. Usual interstitial pneumonia and organizing pneumonia patterns of interstitial injury need to be added to this clinical differential diagnosis.

scholarly journals Multidisciplinary problems of rheumatology and pulmonology

2017 ◽  
Vol 89 (5) ◽  
pp. 4-9
Author(s):  
L P Ananyeva
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Symptoms ◽  
Serological Test ◽  
Systemic Disease ◽  
Connective Tissue Diseases ◽  
Lung Damage ◽  
Lung Involvement ◽  
Pulmonary Manifestations

Damage to the respiratory system worsens prognosis and increases mortality in all systemic immunoinflammatory rheumatic diseases (SIRDs). Leading pulmonary manifestations in each disease are different, so the typical picture of each nosological entity is very peculiar. In all SIRDs, the disease starts with lung involvement in 10—20% of cases, preceding the expanded clinical presentation of a systemic disease. At a certain stage, lung damage may be the only manifestation of the disease or predominate in its clinical picture, determining the severity of a condition. Among the patients referred to consultation with a pulmonologist for interstitial lung disease, about 15% have manifestations of SIRDs that is confirmed by a special survey. The paper discusses the diagnostic features of SIRDs with lung involvement, as well as indications for consultation with a rheumatologist. The key clinical symptoms indicating a possible rheumatic disease in patients with lung involvement are considered. Evidence for the main circulating autoantibodies identifiable in high titers in patients with SIRDs in a serological test is given and its positivity is interpreted. The issues associated with the term «undifferentiated connective tissue diseases-associated interstitial lung disease» are discussed; the expediency of using the term «interstitial pneumonia with autoimmune signs (AIS)» is emphasized. The clinical characteristics of and classification criteria for AIS are presented. The prognosis and outcomes of interstitial pneumonias of varying etiologies, as well as the importance of the multidisciplinary approach to managing patients with SIRDs and lung involvement, which improves the quality of diagnosis, adequacy of treatment, and quality of life, are dealt with.

scholarly journals THE CASE OF THE DEBUT OF SYSTEMIC SCLERODERMA IN A PATIENT AGED 10

2018 ◽  
Vol 25 (3) ◽  
pp. 162-166
Author(s):  
А. V. BURLUTSKAYA ◽  
N. V. SAVELYEVA ◽  
A. V. STATOVA
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Large Range ◽  
Clinical Symptoms ◽  
Joint Damage ◽  
Skin Lesions ◽  
Systemic Scleroderma ◽  
Pathogenetic Therapy ◽  
Systemic Manifestations

Aim. To show the diversity and severity of clinical symptoms, a large range of diagnostic search, as well as difficulties in therapy in a 10-year-old patient with systemic manifestations of juvenile scleroderma.Materials and methods. A retrospective analysis of the anamnetic data, the course of the disease, laboratory and instrumental data, as well as the ongoing therapy of a 10-year-old patient with juvenile systemic scleroderma was carried out.Results. The patient was diagnosed with multiple typical sclerodermic foci of irregular shape, with different stages of lesion (edema, induration and atrophy). Joint damage was noted in the form of pseudoarthritis interphalangeal joints of hands with the formation of sclerodactily and right ankle joint. As a result of computed tomography of chest, signs of interstitial lung disease by the type of fibrous alveolitis have been revealed. After verification of the diagnosis, the patient was prescribed basic therapy: methylprednisolone and cyclophosphane.Conclusion. A clinical case of the debut of juvenile systemic scleroderma with rapidly progressive, widespread skin lesions, involvement of the musculoskeletal system and the development of fibrous alveolitis is presented. Due to intensive and long-term pathogenetic therapy (glucocorticoids, cyclophosphamide), the patient's condition was stabilized and the progression of interstitial lung disease was stopped.

scholarly journals Australasian Interstitial Lung Disease Registry (AILDR): Objectives, Design and Rationale of a Bi-National Prospective Database

2020 ◽  
Author(s):  
Irene Moore ◽  
Jeremy Wrobel ◽  
Jessica Rhodes ◽  
Qi Lin ◽  
Susanne Webster ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Best Practice ◽  
Epidemiological Data ◽  
National Registry ◽  
Second Phase ◽  
Pilot Phase ◽  
Treatment Pathways ◽  
Progressive Respiratory Failure ◽  
Respiratory Conditions

Abstract Background: Interstitial Lung Disease (ILD) is a group of respiratory conditions affecting the lung interstitium often associated with progressive respiratory failure. There is increasing recognition of the need for improved epidemiological data to help determine best practice and improve standardisation of care. The Australasian ILD Registry (AILDR) is a bi-national registry of patients with all ILD subtypes designed to establish a clinically meaningful database reflecting real world practice in Australasia with an objective to improve diagnostic and treatment pathways through research and collaboration. Methods: AILDR is a prospective observational registry recruiting patients attending ILD clinics at centres around Australia and New Zealand. Core and non-core data are stored on a secure server. The pilot phase was launched in 2016 consisting of four sites in Australia. Currently in its second phase a further 16 sites have been recruited, including three in New Zealand.Results: A total of 1061 participants were consented during the pilot phase. Baseline data demonstrated a mean age 68.3±12.5 (SD) years, mean FVC (%predicted) 79.1±20.4 (SD), mean DLCO (%predicted) 58.5±17.9 (SD) and nadir exertional SpO2 (%) 91±6.9 (SD). Idiopathic pulmonary fibrosis (31%) and connective-tissue disease related ILD (21.7%) were the two most common subtypes. Baseline demographics and physiology were not significantly different across the four centres.Conclusion: AILDR is an important clinical and research tool providing a platform for epidemiological data that will prove essential in promoting understanding of a rare cohort of lung disease and provide foundations for our aspiration to standardise investigation and treatment pathways of ILD across Australasia.

Sign in / Sign up

Export Citation Format

Share Document