{Reference Type}: Journal Article
{Title}: In inflammatory myopathies, dropped head/bent spine syndrome is associated with scleromyositis: an international case-control study.
{Author}: Pijnenburg L;Giannini M;Bouchard-Marmen M;Arnaud L;Barsotti S;Bellando-Randone S;Bernardi L;Bini P;Blagojevic J;Codullo V;Couderc M;De Moreuil C;Dernis E;Diamanti L;Dubost JJ;Duval F;Emmi G;Galempoix JM;Geny B;Gottenberg JE;Groza M;Guffroy A;Guichard I;Guilpain P;Hervier B;Hudson M;Iaccarino L;Iannone F;Lebrun D;Marchioni E;Mariampillai K;Maurier F;Mosca M;Nadaj-Pakleza A;Nannini C;Piot JM;Prieto-González S;Poursac N;Rouanet E;Sellam J;Selva-O'Callaghan A;Séverac F;Sibilia J;Sole G;Soulages A;Terrier B;Tournadre A;Troyanov Y;Vernier N;Vesperini V;Viallard JF;Ziane R;Cavagna L;Meyer A;
{Journal}: RMD Open
{Volume}: 9
{Issue}: 3
{Year}: 2023 09
{Factor}: 5.806
{DOI}: 10.1136/rmdopen-2023-003081
{Abstract}: Some myopathies can lead to dropped head or bent spine syndrome (DH/BS). The significance of this symptom has not been studied in inflammatory myopathies (IM).<BR>To assess the significance of DH/BS in patients with IM.<BR>Practitioners from five IM networks were invited to report patients with IM suffering from DH/BS (without other known cause than IM). IM patients without DH/BS, randomly selected in each participating centre, were included as controls at a ratio of 2 to 1.<BR>49 DH/BS-IM patients (DH: 57.1%, BS: 42.9%) were compared with 98 control-IM patients. DH/BS-IM patients were older (65 years vs 53 years, p<0.0001) and the diagnosis of IM was delayed (6 months vs 3 months, p=0.009). Weakness prevailing in the upper limbs (42.9% vs 15.3%), dysphagia (57.1% vs 25.5%), muscle atrophy (65.3% vs 34.7%), weight loss (61.2% vs 23.5%) and loss of the ability to walk (24.5% vs 5.1%) were hallmarks of DH/BS-IM (p≤0.0005), for which the patients more frequently received intravenous immunoglobulins (65.3% vs 34.7%, p=0.0004). Moreover, DH/BS-IM patients frequently featured signs and/or complications of systemic sclerosis (SSc), fulfilling the American College of Rheumatology/European Alliance of Associations for Rheumatology criteria for this disease in 40.8% of the cases (vs 5.1%, p<0.0001). Distribution of the myopathy, its severity and its association with SSc were independently associated with DH/BS (p<0.05). Mortality was higher in the DH/BS-IM patients and loss of walking ability was independently associated with survival (p<0.05).<BR>In IM patients, DH/BS is a marker of severity and is associated with SSc (scleromyositis).