Long-term experience with rituximab in anti-synthetase syndrome-related interstitial lung disease

Andersson, Helena; Sem, Marthe; Lund, May Brit; Aaløkken, Trond Mogens; Günther, Anne; Walle-Hansen, Ragnhild; Garen, Torhild; Molberg, Øyvind

doi:10.1093/rheumatology/kev004

Cited by 182 publications

(124 citation statements)

References 30 publications

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“…Against convention, we started biologic treatment to induce remission in this patient and added a classic disease-modifying antirheumatic agent in the second step for the maintenance therapy. Our therapeutic approach is corroborated by a recent study showing the effectiveness of RTX in antisynthetase syndrome-related lung disease, particularly in patients with acute presentation and short disease duration (28). This treatment was well tolerated and resulted in complete resolution of the musculoskeletal, respiratory, and renal symptoms.…”

Section: Discussionsupporting

confidence: 62%

Respiratory Distress and Nephropathy in a Young Male With Small‐Joint Polyarthritis

Korsten

Konig

Müller

et al. 2016

Arthritis Care & Research

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History of the present illness. A 27-year-old man presented to our department with a 4-week history of worsening fatigue, malaise, and painful swelling of his wrists and finger joints. He further complained of new-onset progressive dyspnea with nonproductive cough, subfebrile temperatures, and proximal muscle weakness in his lower extremities in the last 10 days. Except for the arthritis, he had not experienced these symptoms in the past. Two months prior to admission, he was seen for the first time by a rheumatologist. At that time, he reported severe pain and stiffness in his hands, particularly at night, but no breathing or muscle difficulties. Laboratory tests at the rheumatologist's office showed increased C-reactive protein (CRP) levels and high-titer anti-cyclic citrullinated peptide (CCP) antibodies. Plain radiographs demonstrated an erosion at the fifth metatarsal head of his right foot. He was diagnosed with rheumatoid arthritis (RA) and started on oral methotrexate (MTX) and prednisone. Medical historyThe patient reported having intermittent arthralgias in his hands, elbows, and hips for the past 3 years. He had been treated with nonsteroidal antiinflammatory drugs and oral glucocorticoids as needed by his primary care provider. In the last couple of months, he increasingly experienced painful arthritis in his hands. After being diagnosed with RA, oral treatment with MTX 15 mg weekly and prednisone 50 mg daily (followed by slow tapering) was initiated, along with prophylactic pantoprazole (40 mg daily) and folic acid (5 mg weekly). He was adherent to the therapy and denied taking over-the-counter drugs or supplements. There were no other medical or surgical illnesses.Social and family history. He used to work as a gardener. His social history was negative for smoking and alcohol abuse. He denied recent travel or contact with someone with similar symptoms. His grandmother had a diagnosis of RA, but his family history was otherwise negative for autoimmune or pulmonary disorders. Review of systems.There was no history of photosensitivity, rash, sicca symptoms, Raynaud's phenomenon, back pain, hemoptysis, dysphagia, or diarrhea.Physical examination. On admission, his temperature was 37.08C, blood pressure 130/80 mm Hg, pulse 105 beats per minute, respiratory rate 24 breaths per minute, and oxygen saturation of 95% while breathing ambient air. There was no lymphadenopathy, jaundice, or cyanosis. Auscultation of the lung revealed fine bibasilar crackles. Cardiac (except tachycardia), abdominal, and neurologic examinations were unremarkable. His wrists, metacarpophalangeal, and proximal interphalangeal joints of both hands were swollen and tender with limited range of motion. There was isolated weakness of the thigh muscles (4/5), but no muscle wasting or fasciculations.Laboratory tests and imaging. Initial workup showed a hemoglobin level of 14.5 gm/dl (normal range [NR] 13.5-17.5), white blood cell count of 13.3 3 10 3 /ml (NR 4-11 3 10 3 ), and platelets of 273 3 10 3 /ml (NR 150-350 3 10 3 ). The differe...

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Section: Discussionsupporting

confidence: 62%

Respiratory Distress and Nephropathy in a Young Male With Small‐Joint Polyarthritis

Korsten

Konig

Müller

et al. 2016

Arthritis Care & Research

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“…Many immunosuppressive drugs have been evaluated in ARS-ILD [22-24]. Previous studies have shown that combination therapy improved the findings of PFTs and chest HRCT scans in acute or subacute progressive PM/DM-ILD [22, 25-27].…”

Section: Discussionmentioning

confidence: 99%

Predictive Factors for the Long-Term Deterioration of Pulmonary Function in Interstitial Lung Disease Associated with Anti-Aminoacyl-tRNA Synthetase Antibodies

et al. 2018

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Background: Little has been reported on long-term pulmonary function trends among patients with interstitial lung disease associated with anti-aminoacyl-tRNA synthetase antibodies (ARS-ILD). Objectives: To clarify the factors predictive of progression in ARS-ILD based on patients’ initial clinical and radiological features. Methods: The clinical courses of 88 patients with > 1 year of follow-up data on pulmonary function tests (PFTs) were retrospectively analyzed. Disease behavior was categorized into three groups: (1) improved or (2) worsened (defined as increases or decreases, respectively, of > 10% in forced vital capacity and > 15% in %diffusing capacity of lung carbon monoxide) or (3) stable based on PFT changes compared between 1-year results as the initial data and results at 3 years to assess the long-term course. Results: In the initial course of 75 patients with ARS-ILD who received anti-inflammatory therapy within 6 months after diagnosis, 48 patients (64.0%) improved and 6 patients (8.0%) worsened. The radiological patterns in the patients with ARS-ILD included nonspecific interstitial pneumonia (NSIP) in 46.7% and NSIP with organizing pneumonia overlap in 52.0% of the cases. One-third of the initially improved patients who worsened over the long-term course were assigned to the unstable group. By multivariate logistic analysis, middle lobe traction bronchiectasis was a significant predictive factor for the patients in the unstable group. Conclusions: Most patients with ARS-ILD receiving anti-inflammatory therapy had improved or remained stable in the first year. However, over the long-term course, some patients worsened despite their initial improvement. Even though the extent of the disease is limited, middle lobe traction bronchiectasis in ARS-ILD may be a useful predictor of poor long-term disease behavior.

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“…Moreover, a subsequent subgroup analysis has highlighted that response to rituximab may be influenced by serological phenotype with ASS and antiMi2 patients more likely to gain benefit [122]. A therapeutic role for rituximab within the different clinicoserological phenotypes has been further emphasised in a number of series, including refractory patients with ASS-ILD, anti-MDA5 and anti-SRP disease [117,[123][124][125][126][127][128][129]. Therefore, it seems that stratification based on autoAbs can act as predictive markers of clinical response to treatment, which again highlights their diagnostic utility.…”

Section: Does Clinico-serological Phenotype Influence Outcome and Trementioning

confidence: 99%

The Clinical Features of Myositis-Associated Autoantibodies: a Review

Gunawardena

2015

Clinic Rev Allerg Immunol

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The idiopathic inflammatory myopathies (IIM) are a group of autoimmune diseases traditionally defined by clinical manifestations including skeletal muscle weakness, skin rashes, elevated skeletal muscle enzymes, and neurophysiological and/or histological evidence of muscle inflammation. Patients with myositis overlap can develop other features including parenchymal lung disease, inflammatory arthritis, gastrointestinal manifestations and marked constitutional symptoms. Although patients may be diagnosed as having polymyositis (PM) or dermatomyositis (DM) under the IIM spectrum, it is quite clear that disease course between subgroups of patients is different. For example, interstitial lung disease may predominate in some, whereas cutaneous complications, cancer risk, or severe refractory myopathy may be a significant feature in others. Therefore, tools that facilitate diagnosis and indicate which patients require more detailed investigation for disease complications are invaluable in clinical practice. The expanding field of autoantibodies (autoAbs) associated with connective tissue disease (CTD)-myositis overlap has generated considerable interest over the last few years. Using an immunological diagnostic approach, this group of heterogeneous conditions can be separated into a number of distinct clinical phenotypes. Rather than diagnose a patient as simply having PM, DM or overlap CTD, we can define syndromes to differentiate disease subsets that emphasise clinical outcomes and guide management. There are now over 15 CTD-myositis overlap autoAbs found in patients with a range of clinical manifestations including interstitial pneumonia, cutaneous disease, cancer-associated myositis and autoimmune-mediated necrotising myopathy. This review describes their diagnostic utility, potential role in disease monitoring and response to treatment. In the future, routine use of these autoAb will allow a stratified approach to managing this complex set of conditions.

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Long-term experience with rituximab in anti-synthetase syndrome-related interstitial lung disease

Cited by 182 publications

References 30 publications

Respiratory Distress and Nephropathy in a Young Male With Small‐Joint Polyarthritis

Respiratory Distress and Nephropathy in a Young Male With Small‐Joint Polyarthritis

Predictive Factors for the Long-Term Deterioration of Pulmonary Function in Interstitial Lung Disease Associated with Anti-Aminoacyl-tRNA Synthetase Antibodies

The Clinical Features of Myositis-Associated Autoantibodies: a Review

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