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

Submitted: 13 January 2021
Accepted: 7 April 2021
Published: 3 August 2021
Abstract Views: 2893
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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.

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Citations

Paik JJ. Myopathy in scleroderma and in other connective tissue diseases. Curr Opin Rheumatol. 2016; 28: 631-5. DOI: https://doi.org/10.1097/BOR.0000000000000336
Paik JJ. Muscle disease in scleroderma. Curr Opin Rheumatol. 2018; 30: 576-80. DOI: https://doi.org/10.1097/BOR.0000000000000552
Mimura Y, Ihn H, Jinnin M, et al. Clinical and laboratory features of scleroderma patients developing skeletal myopathy. Clin Rheumatol. 2005; 24: 99-102. DOI: https://doi.org/10.1007/s10067-004-0975-7
Ranque B, Authier FJ, Berezne A, et al. Systemic sclerosis-associated myopathy. Ann N Y Acad Sci. 2007; 1108: 268-82. DOI: https://doi.org/10.1196/annals.1422.029
Pestronk A, Kos K, Lopate G, Al-Lozi MT. Brachio-cervical inflammatory myopathies: Clinical, immune, and myopathologic features. Arthritis Rheum. 2006; 54: 1687-96. DOI: https://doi.org/10.1002/art.21822
Garcin B, Lenglet T, Dubourg O, et al. Dropped head syndrome as a presenting sign of scleromyositis. J Neurol Sci. 2010; 292: 101-3. DOI: https://doi.org/10.1016/j.jns.2010.02.015
Fernández-Serna M, Arboleya L, Alonso S, et al. Dropped head syndrome in a patient with scleromyositis. J Clin Rheumatol. 2013; 19: 32-4. DOI: https://doi.org/10.1097/RHU.0b013e31827d8778
Yoshida T, Yoshida M, Mitsuyo K, et al. Dropped head syndrome and the presence of rimmed vacuoles in a muscle biopsy in scleroderma-polymyositis overlap syndrome associated with anti-ku antibody. Intern Med. 2018; 57: 887-91. DOI: https://doi.org/10.2169/internalmedicine.9363-17
Rojana-Udomsart A, Fabian V, Hollingsworth PN, et al. Paraspinal and scapular myopathy associated with scleroderma. J Clin Neuromuscul Dis. 2010; 11: 213-22. DOI: https://doi.org/10.1097/CND.0b013e3181c139f6
Gao AF, Saleh PA, Kassardjian CD, et al. Brachio-cervical inflammatory myopathy with associated scleroderma phenotype and lupus serology. Neurol Neuroimmunol Neuroinflammation. 2018; 5: 1-3. DOI: https://doi.org/10.1212/NXI.0000000000000410
Suárez-Calvet X, Alonso-Pérez J, Castellví I, et al. Thrombospondin-1 mediates muscle damage in brachio-cervical inflammatory myopathy and systemic sclerosis. Neurol Neuroimmunol Neuroinflammation. 2020; 7: e694. DOI: https://doi.org/10.1212/NXI.0000000000000694
van den Hoogen F, Khanna D, Fransen J, et al. 2013 Classification criteria for systemic sclerosis: An American College of Rheumatology/European League Against Rheumatism Collaborative Initiative. Arthritis Rheum. 2013; 65: 2737-47. DOI: https://doi.org/10.1002/art.38098
Lundberg IE, Tjärnlund A, Bottai M, et al. 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Arthritis Rheumatol. 2017; 69: 2271-82. DOI: https://doi.org/10.1002/art.40320
Audet GN, Fulks D, Stricker JC, Olfert IM. Chronic delivery of a thrombospondin-1 mimetic decreases skeletal muscle capillarity in mice. PLoS One. 2013; 8: e55953. DOI: https://doi.org/10.1371/journal.pone.0055953
Salajegheh M, Raju R, Schmidt J, Dalakas MC. Upregulation of thrombospondin-1(TSP-1) and its binding partners, CD36 and CD47, in sporadic inclusion body myositis. J Neuroimmunol. 2007; 187: 166-74. DOI: https://doi.org/10.1016/j.jneuroim.2007.04.022
Phelan MW, Forman LW, Perrine SR, Faller DV. Hypoxia increases thrombospondin-1 transcript and protein in cultured endothelial cells. J Lab Clin Med. 1998; 132: 519-29. DOI: https://doi.org/10.1016/S0022-2143(98)90131-7
Mansfield PJ, Suchard SJ. Thrombospondin promotes chemotaxis and haptotaxis of human peripheral blood monocytes. J Immunol. 1994; 153: 4219-29.
Le Quintrec JS, Le Quintrec JL. Drug-induced myopathies. Baillieres Clin Rheumatol. 1991; 5: 21-38. DOI: https://doi.org/10.1016/S0950-3579(05)80294-8
Aydintug AO, Cervera R, D’Cruz D, et al. Polymyositis complicating D-penicillamine treatment. Postgrad Med. J 1991; 67: 1018-20. DOI: https://doi.org/10.1136/pgmj.67.793.1018
Preedy VR, Marway JS, Baldwin D, et al. Experimental D-penicillamine-induced myopathy. Biochem Soc Trans. 1996; 24: 2403. DOI: https://doi.org/10.1042/bst024266s
Sato N, Okamoto S, Mori T, et al Recurrent acute myositis after allogeneic bone marrow transplantation for myelodysplasia. Hematology. 2002; 7: 109-12. DOI: https://doi.org/10.1080/10245330290022115
Oda K, Nakaseko C, Ozawa S, et al. Fasciitis and myositis: An analysis of muscle-related complications caused by chronic GVHD after allo-SCT. Bone Marrow Transplant. 2009; 43: 159-67. DOI: https://doi.org/10.1038/bmt.2008.297
Hedermann G, Marquart H, Vissing J. Polymyositis following autologous haematopoietic stem cell transplantation. Scand J Rheumatol 2016; 45: 429-31. DOI: https://doi.org/10.3109/03009742.2015.1125525
Radke J, Pehl D, Aronica E, et al. The lymphoid follicle variant of dermatomyositis. Neurol Neuroimmunol Neuroinflammation. 2014; 1: e19. DOI: https://doi.org/10.1212/NXI.0000000000000019
De Padilla CML, Vallejo AN, Lacomis D, et al. Extranodal lymphoid microstructures in inflamed muscle and disease severity of new-onset Juvenile dermatomyositis. Arthritis Rheum. 2009; 60: 1160-72. DOI: https://doi.org/10.1002/art.24411
Radke J, Koll R, Preuße C, et al Architectural B-cell organization in skeletal muscle identifies subtypes of dermatomyositis. Neurol Neuroimmunol Neuroinflammation. 2018; 5: e451. DOI: https://doi.org/10.1212/NXI.0000000000000451
Jung M, Bonner A, Hudson M, et al.; Canadian Scheroderma Research Group (CSRG). Myopathy is a poor prognostic feature in systemic sclerosis: results from the Canadian Scleroderma Research Group (CSRG) cohort. Scand J Rheumatol. 2014; 43: 217-20. DOI: https://doi.org/10.3109/03009742.2013.868512
Chaigne B, Rodeia S, Benmostefa N, et al Corticosteroid-sparing benefit of intravenous immunoglobulin in systemic sclerosis-associated myopathy: A comparative study in 52 patients. Autoimmun Rev. 2020; 19: 102431. DOI: https://doi.org/10.1016/j.autrev.2019.102431
Ranque B, Authier FJ, Le-Guern V, et al. A descriptive and prognostic study of systemic sclerosis-associated myopathies. Ann Rheum Dis. 2009; 68: 1474-7. DOI: https://doi.org/10.1136/ard.2008.095919

How to Cite

Araujo, C., Miossi, R., De Souza, F., Costa, M., da Silva, A., Campos, E., Zanoteli, E., & Shinjo, S. (2021). Brachio-cervical inflammatory myopathy associated with systemic sclerosis. Case series and review of literature. Reumatismo, 73(2), 122–130. https://doi.org/10.4081/reumatismo.2021.1397