Brachio-cervical inflammatory myopathy associated with systemic sclerosis. Case series and review of literature.

This study was aimed at describing a case series of brachio-cervical inflammatory myopathy (BCIM) associated with systemic sclerosis (SSc), due to its rarity and limited coverage in published data. Another aim was to provide a literature review. We reported four cases of BCIM-SSc from our tertiary center. In addition, we researched the literature and found six articles featuring 17 patients who fit this phenotype. We pooled all cases and reported their features. Most patients were female and had limited SSc, and the median time of BCIM presentation was three years after SSc diagnosis. Asymmetric muscle involvement, scapular winging, dropped head, axial weakness, camptocormia, dysphagia, and dermatomyositis stigmas were common features. All patients had esophageal involvement. Most had positive antinuclear antibody results, a scleroderma pattern in their capillaroscopy images, elevated serum creatine phosphokinase, myopathic electrophysiology, and muscle involvement in magnetic resonance imaging. Muscle histopathological findings varied widely, but in general all showed the presence of lymphoid infiltrates, muscle atrophy, increased MHC-I expression, MAC deposits, vasculopathy, and muscle fiber necrosis. The response to immunosuppressive therapy was highly irregular. BCIM-SSc is a rare disorder that shares many similar phenotypes among the described cases, but has a highly heterogeneous response to treatment. At present, more data on the physiopathology, clinical features, and treatment is still needed.

Is there a reliable association between patient-reported limb claudication and vascular imaging methods in Takayasu arteritis?

Vessel imaging in Takayasu arteritis (TAK) is often performed in clinical practice following laboratory test abnormalities or clinical symptoms, such as limb claudication. Conversely, the association between limb claudication and vessel imaging manifestations has not been assessed. This observational, cross-sectional study analyzed 139 adult TAK patients from 2000 to 2018. Their arterial vessel imaging information (especially significant stenosis and occlusion data) was registered and crosschecked with clinical and laboratory data. When vessel imaging was performed, the median age and disease duration of the patients were 38 (27.3-47.0) and 5.0 (1.0-12.0) years, respectively. There was no association between arterial abnormalities and demographic data, constitutional symptoms or laboratory parameters. Limb claudication was reported in 42 patients (30.2%): 17.3% reported it in the upper left limb (ULL), 12.2% reported it in the upper right limb (URL), 12.9% reported it in the lower left limb (LLL), and 12.2% reported it in the lower right limb (LRL). When crossmatched with imaging, both ULL and URL were associated with left vertebral artery stenosis/occlusion, and URL was associated with right iliac artery stenosis/occlusion, but no other association was found. In contrast, both LLL and LRL claudication were associated with infrarenal aortic, left iliac and right iliac artery stenosis/ occlusion (p<0.05). Moreover, the ULL and URL claudication symptoms were significantly associated with each other (p<0.001). Upper limb claudication was associated only with left vertebral artery stenosis/occlusion, whereas the subclavian arteries were not, suggesting that the symptom might not be fully explained by limb ischemia. In contrast, lower limb claudication was associated especially with infrarenal aortic and/or iliac arteries stenosis/occlusion.