OBJECTIVES: Systemic autoimmune rheumatic diseases can occur as paraneoplastic phenomena in the context of underlying malignancies. We present three illustrative clinical cases and a narrative literature review focusing on systemic sclerosis, dermatomyositis and palmar fasciitis and polyarthritis syndrome. METHODS: Medical data of three patients fr...
Published in Practical neurology
Anti-HMGCR (3-hydroxy-3-methylglutaryl coenzyme A reductase) myopathy is an immune-mediated necrotising myopathy. Atypical presentations hinder its recognition and its prompt treatment. We present two patients with atypical clinical or pathological features. A 45-year-old woman had an asymptomatic serum creatine kinase (CK) of ~10 000 IU/L and musc...
In patients with interstitial lung disease (ILD) complicating classical or amyopathic idiopathic inflammatory myopathy (IIM), lung transplantation outcomes might be affected by the disease and treatments. Here, our objective was to assess survival and prognostic factors in lung transplant recipients with IIM-ILD. We retrospectively reviewed data fo...
Pancreatic cancer is only rarely associated with myopathy. We present a case of a 69-year-old male with recently diagnosed pancreatic cancer, who presented himself with a paraneoplastic myositis of both legs. MRI and EMG contributed to this diagnosis. Treatment was started with high dose corticosteroids followed by urgent laparoscopic pancreaticodu...
BACKGROUND: To address the reactivity and affinity against histidyl-transfer RNA synthetase (HisRS) autoantigen of anti-Jo1 autoantibodies from serum and bronchoalveolar lavage fluid (BALF) in patients with idiopathic inflammatory myopathies/anti-synthetase syndrome (IIM/ASSD). To investigate the associations between the reactivity profile and clin...
Nowadays, the importance of detection of myositis-specific antibodies (MSA) and myositis-associated antibodies (MAA) in diagnosis and in delineating disease subsets of idiopathic inflammatory myopathy (IIM) is highly acknowledged by IIM experts. Consequently, MSA/MAA are increasingly integrated in expert-based myositis (sub)classification criteria ...
Objective: We conducted a systematic review, on behalf of the EULAR Study Group on Microcirculation in Rheumatic Diseases (EULAR SG MC/RD), to investigate the value of nailfold videocapillaroscopy (NVC) in idiopathic inflammatory myopathies (IIM). Methods: Three electronic databases were systematically searched to find all relevant manuscripts repo...
Published in Clinical and experimental rheumatology
Today, the contribution of myositis-specific autoantibodies (MSA) in the diagnostic workup of idiopathic inflammatory myopathies (IIM) is on the rise. The aim of this study was to document MSA frequency as detected by lineblot in a set of consecutive MSA requests and to correlate the results with clinical diagnosis, IIM subtype and indirect immunof...
Introduction/objectives The aim of our study was to investigate possible differences in nailfold videocapillaroscopy (NVC) features between patients with dermatomyositis (DM), overlap myositis (OM), antisynthetase syndrome (ASS), and immune-mediated necrotizing myopathy (IMNM). Methods We performed a cross-sectional monocentric study. All patients ...
Published in The Journal of rheumatology
The optimal treatment strategy for anti-aminoacyl-tRNA synthetase antibody-positive polymyositis/dermatomyositis-associated interstitial lung disease (anti-ARS-PM/DM-ILD) is yet to be established. We aimed to evaluate the efficacy of glucocorticoids and calcineurin inhibitors (CNI) in patients with ARS-PM/DM-ILD. Progression-free survival (PFS) and...
