Corticosteroid-sparing benefit of intravenous immunoglobulin in systemic sclerosis-associated myopathy: A comparative study in 52 patients

Chaigne, Benjamin; Rodeia, Simão; Benmostefa, Nouria; Bérezné, Alice; Authier, J.; Cohen, Pascal; Régent, Alexis; Terrier, B.; Costédoat-Chalumeau, Nathalie; Guillevin, Loı̈c; Jeunne, Claire Le; Mouthon, Luc

doi:10.1016/j.autrev.2019.102431

Cited by 28 publications

(17 citation statements)

References 26 publications

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“…Histopathological studies of muscle biopsies in SSc have suggested that myositis should be considered as a distinct feature of SSc 23 24 38 39 because of unique myopathological findings. 25 The presence of microangiopathy was demonstrated in scleromyositis associated with anti-centromere and anti-topoisomerase I aAbs.…”

Section: Discussionmentioning

confidence: 99%

“… For patients not meeting the SSc classification criteria, the presenting SSc features could either be Raynaud phenomenon, as implied by the concept of very early diagnosis of SSc 21 ; Interstitial lung disease (ILD), as epitomised by ILD with anti-Th/To SSc aAbs 22 ; Isolated muscle involvement with neither Raynaud phenomenon nor ILD, as suggested by SSc-specific muscle histopathology. 23–29 …”

Section: Methodsmentioning

confidence: 99%

“…Isolated muscle involvement with neither Raynaud phenomenon nor ILD, as suggested by SSc-specific muscle histopathology. 23–29 …”

Section: Methodsmentioning

confidence: 99%

“…For patients not meeting the SSc classification criteria, the presenting SSc features could either be a. Raynaud phenomenon, as implied by the concept of very early diagnosis of SSc 21 ; b. Interstitial lung disease (ILD), as epitomised by ILD with anti-Th/To SSc aAbs 22 ; c. Isolated muscle involvement with neither Raynaud phenomenon nor ILD, as suggested by SSc-specific muscle histopathology. [23][24][25][26][27][28][29] Serology Baseline immunological studies were performed at CHUM and HSCM as previously described. 17 18 ANAs were determined by IIF on HEp-2 cells and the threshold value for positivity was >1:160.…”

Section: Patientsmentioning

confidence: 99%

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Recognising the spectrum of scleromyositis: HEp-2 ANA patterns allow identification of a novel clinical subset with anti-SMN autoantibodies

et al. 2020

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ObjectiveTo describe systemic sclerosis (SSc) with myopathy in patients without classic SSc-specific and SSc-overlap autoantibodies (aAbs), referred to as seronegative scleromyositis.MethodsTwenty patients with seronegative scleromyositis diagnosed by expert opinion were analysed retrospectively for SSc features at myositis diagnosis and follow-up, and stratified based on HEp-2 nuclear patterns by indirect immunofluorescence (IIF) according to International Consensus of Autoantibody Patterns. Specificities were analysed by protein A−assisted immunoprecipitation. Myopathy was considered an organ involvement of SSc.ResultsSSc sine scleroderma was a frequent presentation (45%) at myositis diagnosis. Myositis was the most common first non-Raynaud manifestation of SSc (55%). Lower oesophagal dysmotility was present in 10 of 11 (91%) investigated patients. At follow-up, 80% of the patients met the American College of Rheumatology/EULAR SSc classification criteria. Two-thirds of patients had a positive HEp-2 IIF nuclear pattern (all with titers ≥1/320), defining three novel scleromyositis subsets. First, antinuclear antibody (ANA)-negative scleromyositis was associated with interstitial lung disease (ILD) and renal crisis. Second, a speckled pattern uncovered multiple rare SSc-specific aAbs. Third, the nuclear dots pattern was associated with aAbs to survival of motor neuron (SMN) complex and a novel scleromyositis subset characteriszed by calcinosis but infrequent ILD and renal crisis.ConclusionsSSc skin involvement is often absent in early seronegative scleromyositis. ANA positivity, Raynaud phenomenon, SSc-type capillaroscopy and/or lower oesophagal dysmotility may be clues for scleromyositis. Using HEp-2 IIF patterns, three novel clinicoserological subsets of scleromyositis emerged, notably (1) ANA-negative, (2) ANA-positive with a speckled pattern and (3) ANA-positive with nuclear dots and anti-SMN aAbs.

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Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

“…Isolated muscle involvement with neither Raynaud phenomenon nor ILD, as suggested by SSc-specific muscle histopathology. 23–29 …”

Section: Methodsmentioning

confidence: 99%

Section: Patientsmentioning

confidence: 99%

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Recognising the spectrum of scleromyositis: HEp-2 ANA patterns allow identification of a novel clinical subset with anti-SMN autoantibodies

et al. 2020

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“…A randomized control trial assessing the change in skin fibrosis as primary endpoint failed to demonstrate a significant beneficial reduction of modified Rodnan skin score in Intravenous immunoglobulin (IVIg) administration versus placebo [71]. Different authors have shown the beneficial effect of the use of IVIg in SSc patients with arthritis [72] and inflammatory myopathy [73], and they have demonstrated some potential benefit on earlystage ILD, with the authors reporting a regression in ground glass opacity, septal thickening, and a full recovery of lung function [74]. A randomized phase II trial (NCT04137224) is currently testing a possible similar effect and the safety of subcutaneous immunoglobulins and IVIg [75].…”

Section: Immunoglobulinsmentioning

confidence: 99%

The Treatment of Lung Involvement in Systemic Sclerosis

et al. 2021

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Systemic sclerosis (SSc) patients are often affected by interstitial lung disease (ILD) and, although there have been recent treatment advances, it remains the leading cause of death among SSc, with a 10-year mortality up to 40%. African Americans and subjects with diffuse cutaneous SSc or anti-topoisomerase 1 antibodies are most commonly affected. Currently, early ILD diagnosis can be made, and it is pivotal to improve the prognosis. The diagnostic mainstay test for SSc-ILD is high-resolution computed tomography for the morphology and pulmonary function tests for the functional aspects. Treatment planning and intensity are guided by the disease severity and risk of progression. Traditionally, therapy has depended on combinations of immunosuppressants, particularly cyclophosphamide and mycophenolate mofetil, which can be supplemented by targeted biological and antifibrotic therapies. Benefits have been observed in trials on hematopoietic autologous stem cell transplantation for patients with progressive SSc, whilst lung transplantation is reserved for refractory SSc-ILD cases. Herein, recent advances in SSc-ILD treatment will be explored.

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