Mechanical Hand Research Articles

Clinical profiles and treatment outcomes of outpatients with interstitial lung disease and mechanic's hands: A retrospective and observational cohort.

Idiopathic inflammatory myopathies, especially antisynthetase syndrome, often appear outside of the muscles as interstitial lung disease (ILD). Another typical finding is the presence of mechanic's hands. The aim of the present study was to describe the clinical, functional, tomographic, and serological data of patients with ILD and mechanic's hands and their response to treatment and survival rates. This is a retrospective study of ILD with concurrent myopathy. Among the 119 patients initially selected, 51 had mechanic's hands. All the patients were screened for anti-Jo-1 antibodies. An expanded panel of myopathy autoantibodies was also performed in 27 individuals. Of the 51 patients, 35 had 1 or more antibodies. The most common were anti-Jo-1, anti-PL-7, and anti-PL-12, while of the associated antibodies, anti-Ro52 was present in 70% of the 27 tested individuals. A significant response to treatment was characterized by an increase in predicted forced vital capacity (FVC) of at least 5% in the last evaluation done after 6 to 24 months of treatment. A decrease in predicted FVC of at least 5%, the need for oxygen therapy, or death were all considered treatment failures. All patients were treated with corticosteroids, and 71% with mycophenolate. After 24 months, 18 patients had an increase in FVC, 11 had a decrease, and 22 remained stable. After a median follow-up of 58 months, 48 patients remained alive and three died. Patients with honeycombing on high-resolution chest tomography (log-rank = 34.65; P < .001) and a decrease in FVC ≥5% (log-rank = 18.28, P < .001) had a poorer survival rate. Patients with ILD and mechanic's hands respond well to immunosuppressive treatment.

Cutaneous manifestations of anti‐synthetase syndrome: Case series and literature review

AbstractBackgroundAnti‐synthetase syndrome (ASyS) is a rare inflammatory myopathy associated with anti‐aminoacyl transfer RNA synthetase antibodies, and is characterized by a triad of interstitial lung disease, arthritis, and myopathy. ASyS has been considered to represent a subtype of dermatomyositis (DM), however it is not clear whether cutaneous findings overlap in both diseases.ObjectivesWe aimed to characterize mucocutaneous features which should raise suspicion of ASyS and prompt early diagnostic testing.MethodsWe reviewed 28 cases of ASyS in our institution, and retrieved data from 43 articles reporting clinical features of ASyS in the literature.ResultsDermatologic manifestations of ASyS can be categorized as: specific cutaneous signs strongly associated with ASyS (‘Mechanic's hands’, ‘Hiker's feet’); mucocutaneous signs overlapping with other collagenoses; nailfold changes (resembling those of DM and systemic sclerosis); and mucocutaneous signs of undetermined significance. Only 20% had pathognomonic DM signs.ConclusionsDermatologic manifestations of ASyS are heterogeneous and do not always reproduce the pattern seen in DM. Presence of palmar hyperkeratosis (‘Mechanic's hands’), cuticular overgrowth, skin ulcerations and nailfold capillary changes should prompt further diagnostic workup of ASyS. Limitations of our study include inconsistent reporting of dermatologic findings within our series between virtual and in‐person visits. Not all patients were seen in dermatology consultation.

Clinical characteristics and prognostic analysis of idiopathic inflammatory myopathy with positive anti-aminoacyl-tRNA synthetase antibodies: A single center experience.

To identify the differences of clinical characteristics, laboratory findings, and the long-term outcomes in patients with anti-synthetase syndrome (ASS) of different anti-aminoacyl-transfer RNA synthetase antibodies. We retrospectively enrolled 119 patients with ASS, and the clinical characteristics and laboratory findings were collected. Additionally, multivariate COX regression analysis was performed to estimate the risk factors of prognosis in patients with ASS. The frequency of interstitial lung disease (ILD) reached 93.3% in our cohort, of 28 (23.5%) was classified as rapidly progressive (RP)-ILD. The highest incidence of RP-ILD was 36.4% in the PL12 group of ASS patients. The ILD group was characterized by an older age, a lower prevalence of V sign, and a higher prevalence of pulmonary symptoms when contrasted with the non-ILD group. There were statistical differences of clinical significance in arthritis, myositis, mechanic's hands, triad, shawl sign, V sign, and Raynaud's phenomenon among the four subgroups (all p < .05). Additionally, the prevalence rates of arthritis, myositis, mechanic's hands, triad, and V sign in the anti-Jo1 antibody-positive group were significantly higher than anti-Jo1 antibody-negative patients with ASS (all p < .05). Multivariate Cox regression analysis showed mechanic's hands (odds ratio [OR] = 6.47, p < .001), anti-nuclear antibodies (ANA) (OR = 2.13, p = .026), ILD (OR = 10.50, p < .001), and V sign (OR = 0.30, p = .007) were independent factors affecting the prognosis of patients with ASS. The incidences of RP-ILD, arthritis, myositis, triad, mechanic's hands, and shawl sign were more frequent in the anti-Ro52 antibody-positive group than the anti-Ro52 antibody-negative patients with ASS (all p < .05). Patients with ASS accompanied with ILD are highly prevalent. Mechanic's hands, ANA, and ILD may be a potential biomarker for predicting a poor prognosis in patients with ASS. Additionally, the detection of the anti-Ro52 antibody provides valuable insights for managing and predicting disease progression and long-term outcomes.

Comparison of clinical features between patients with anti-synthetase syndrome and dermatomyositis: results from the MYONET registry.

To compare clinical characteristics, including the frequency of cutaneous, extramuscular manifestations and malignancy, between adults with anti-synthetase syndrome (ASyS) and DM. Using data regarding adults from the MYONET registry, a cohort of DM patients with anti-Mi2/-TIF1γ/-NXP2/-SAE/-MDA5 autoantibodies, and a cohort of ASyS patients with anti-tRNA synthetase autoantibodies (anti-Jo1/-PL7/-PL12/-OJ/-EJ/-Zo/-KS) were identified. Patients with DM sine dermatitis or with discordant dual autoantibody specificities were excluded. Sub-cohorts of patients with ASyS with or without skin involvement were defined based on presence of DM-type rashes (heliotrope rash, Gottron's papules/sign, violaceous rash, shawl sign, V-sign, erythroderma, and/or periorbital rash). In total 1054 patients were included (DM, n = 405; ASyS, n = 649). In the ASyS cohort, 31% (n = 203) had DM-type skin involvement (ASyS-DMskin). A higher frequency of extramuscular manifestations, including Mechanic's hands, Raynaud's phenomenon, arthritis, interstitial lung disease and cardiac involvement differentiated ASyS-DMskin from DM (all P < 0.001), whereas higher frequency of any of four DM-type rashes-heliotrope rash (n = 248, 61% vs n = 90, 44%), violaceous rash (n = 166, 41% vs n = 57, 9%), V-sign (n = 124, 31% vs n = 28, 4%), and shawl sign (n = 133, 33% vs n = 18, 3%)-differentiated DM from ASyS-DMskin (all P < 0.005). Cancer-associated myositis (CAM) was more frequent in DM (n = 67, 17%) compared with ASyS (n = 21, 3%) and ASyS-DMskin (n = 7, 3%) cohorts (both P < 0.001). DM-type rashes are frequent in patients with ASyS; however, distinct clinical manifestations differentiate these patients from classical DM. Skin involvement in ASyS does not necessitate increased malignancy surveillance. These findings will inform future ASyS classification criteria and patient management.

Fever in the initial stage of IIM patients: an early clinical warning sign for AE-ILD

BackgroundFever is a common symptom of Idiopathic inflammatory myopathies (IIM). However, the exact correlation between fever and the prognosis of IIM is still unclear. This study aims to clarify if the IIM patients initiated with fever are associated with poorer outcomes.MethodsThis was a single-center retrospective cohort study. Data were collected from 79 newly diagnosed, treatment-naive IIM patients in the Affiliated Wuxi People’s Hospital of Nanjing Medical University (Wuxi, Jiangsu, China) from November 2016 to June 2020. According to the presence or absence of fever at the onset, the IIM patients were divided into two groups(fever group n = 28, without fever group n = 51) Clinical characteristics, laboratory data, treatment, and outcomes were recorded. The Kaplan-Meier and log-rank tests were used to compare the all-cause mortality, relapse rate, and acute exacerbation of interstitial lung disease (AE-ILD) incidence. The association of fever with the outcomes was assessed in the unadjusted and adjusted forward logistic regression model.ResultsCompared with the non-fever group, the age at onset of the fever group was higher, and mechanic’s hands (MH) and interstitial lung disease (ILD) were more common. Systemic inflammation (CRP and ESR) was significantly higher in the fever group, while the level of albumin(ALB) and muscle enzymes were lower. The fever group seemed to be received more aggressive treatment, with higher dose glucocorticoids and higher rates of intravenous immunoglobulins(IVIG) use. The all-cause mortality rate and the incidence rate of AE-ILD were higher in the fever group. Even adjusted for the age at onset and treatments, fever was significantly associated with AE-ILD and all-cause mortality.ConclusionOur study has demonstrated that fever at initial diagnosis is associated with AE-ILD and mortality. Fever should serve as an early clinical warning sign for poor outcomes in IIM patients.

Phenotypic Profiles Among 72 Caucasian and Afro-Caribbean Patients with Antisynthetase Syndrome Involving Anti-PL7 or Anti-PL12 Autoantibodies

ObjectivesAntisynthetase syndrome (ASyS) is a rare autoimmune disease. We aimed to determine clinical, biological, radiological, and evolutive profiles of ASyS patients with anti-PL7 or anti-PL12 autoantibodies. MethodsWe performed a retrospective study that included adults with overt positivity for anti-PL7/anti-PL12 autoantibodies and at least one Connors’ criterion. ResultsAmong 72 patients, 69% were women, 29 had anti-PL7 and 43 anti-PL12 autoantibodies, median age was 60.3 years, and median follow-up period was 52.2 months. At diagnosis, 76% of patients had interstitial lung disease, 61% had arthritis, 39% myositis, 25% Raynaud's phenomenon, 18% mechanic's hands, and 17% had fever. The most frequent pattern on initial chest computed tomography was non-specific interstitial pneumonia and 67% had fibrosis at last follow-up. During follow-up, 12 patients had pericardial effusion (18%), 19 had pulmonary hypertension (29%), 9 (12.5%) had neoplasms, and 14 (19%) died. Sixty-seven patients (93%) received at least one steroid or immunosuppressive drug. Patients with anti-PL12 autoantibodies were younger (p=0.01) and more frequently exhibited anti-SSA autoantibodies (p=0.01); patients with anti-PL7 autoantibodies had more severe weakness and higher maximum creatine kinase levels (p=0.03 and 0.04, respectively). Initial severe dyspnoea was more common in patients from the West Indies (p=0.009), with lower predicted values of forced vital capacity, forced expiratory volume in 1s, and total lung capacity (p=0.01, p=0.02, p=0.01, respectively) contributing to a more severe ‘respiratory’ initial presentation. ConclusionsThe high mortality and significant numbers of cardiovascular events, neoplasms and lung fibrosis in anti-PL7/12 patients justify close monitoring and question addition of antifibrotic drugs.

Mechanic's hands are associated with interstitial lung disease in myositis patients regardless of the presence of antisynthetase antibodies.

Mechanic's hands are associated with interstitial lung disease in myositis patients regardless of the presence of antisynthetase antibodies.

Risk of Early Infection in Idiopathic Inflammatory Myopathies: Cluster Analysis Based on Clinical Features and Biomarkers.

Patients with idiopathic inflammatory myopathies (IIMs), referred to as myositis, are prone to infectious complications, which hinder the treatment of the disease and worsen the outcome of patients. The purpose of this study was to explore the different types of infectious complications in patients with myositis and to determine the predisposing factors for clinical reference. A retrospective study was conducted on 66 patients with IIM who were divided into different subpopulations by an unsupervised analysis of their clinical manifestations, laboratory features, and autoantibody characteristics. Combined with the incidence of infectious complications, the types of infectious pathogens and the sites of infection, the characteristics of infection, and susceptibility factors were explored. Three clusters with significantly different clinical characteristics and coinfection rates were identified (76.2% vs. 41.6% vs. 36.4%, p = 0.0139). Cluster 1 (n = 12) had a moderate risk of infection, with an infection rate of 41.6%. The patients in cluster 1 had a high probability of positive mechanic's hands, periungual erythema, anti-Ro52 antibody, and anti-Jo1 antibody. CD3 and CD4 were the highest among the three groups. Cluster 2 (n = 21) had a high risk of infection, and the incidence of infection was 76.2%. Almost all patients in this cluster had a rash, prominent clinical symptoms, and decreased WBC, PMN, LYM, CD3, and CD4 counts. Cluster 3 (n = 33) had a low risk of infection, with an infection rate of 36.4%. Compared with the other two clusters, cluster 3 (n = 33) lacked a typical rash but had a high ANA-positive rate. The patients in cluster 1 and cluster 3 were mainly infected by viruses, followed by bacterial infections. In cluster 2 patients, bacterial infections were the most prevalent. Fungal and Pneumocystis carinii were common causes of cluster 2 and 3 infections. In addition, the patients within a cluster often have a single infection, and pulmonary infections are the most common. We clustered the patients with IIM complicated with infection into three different types by their clinical symptoms and found that there were differences in the infection risk and infection types among the different cluster groups.

Autoantigenic properties of the aminoacyl tRNA synthetase family in idiopathic inflammatory myopathies

ObjectivesAutoantibodies are thought to play a key role in the pathogenesis of idiopathic inflammatory myopathies (IIM). However, up to 40% of IIM patients, even those with clinical manifestations of anti-synthetase syndrome (ASSD), test seronegative to known myositis-specific autoantibodies. We hypothesized the existence of new potential autoantigens among human cytoplasmic aminoacyl tRNA synthetases (aaRS) in patients with IIM. MethodsPlasma samples from 217 patients with IIM according to 2017 EULAR/ACR criteria, including 50 patients with ASSD, 165 without, and two with unknown ASSD status were identified retrospectively, as well as age and gender-matched sera from 156 population controls, and 219 disease controls. Patients with previously documented ASSD had to test positive for at least one of the five most common anti-aaRS autoantibodies (anti-Jo1, -PL7, -PL12, -EJ, and -OJ) and present with one or more of the following clinical manifestations: interstitial lung disease, myositis, arthritis, Raynaud's phenomenon, fever, or mechanic's hands. Demographics, laboratory, and clinical data of the IIM cohort (ASSD and non-ASSD) were compared. Samples were screened using a multiplex bead array assay for presence of autoantibodies against a panel of 117 recombinant protein variants, representing 33 myositis-related proteins, including all nineteen cytoplasmic aaRS. Prospectively collected clinical data for the IIM cohort were retrieved and compared between groups within the IIM cohort and correlated with the results of the autoantibody screening. Principal component analysis was used to analyze clinical manifestations between ASSD, non-ASSD groups, and individuals with novel anti-aaRS autoantibodies. ResultsWe identified reactivity towards 16 aaRS in 72 of the 217 IIM patients. Twelve patients displayed reactivity against nine novel aaRS. The novel autoantibody specificities were detected in four previously seronegative patients for myositis-specific autoantibodies and eight with previously detected myositis-specific autoantibodies. IIM individuals with novel anti-aaRS autoantibodies (n = 12) all had signs of myositis, and they had either muscle weakness and/or muscle enzyme elevation, 2/12 had mechanic's hands, 3/12 had interstitial lung disease, and 2/12 had arthritis. The individuals with novel anti-aaRS and a pathological muscle biopsy all presented widespread up-regulation of major histocompatibility complex class I. The reactivities against novel aaRS could be confirmed in ELISA and western blot. Using the multiplex bead array assay, we could confirm previously known reactivities to four of the most common aaRS (Jo1, PL12, PL7, and EJ (n = 45)) and identified patients positive for anti-Zo, -KS, and -HA (n = 10) that were not previously tested. A low frequency of anti-aaRS autoantibodies was also detected in controls. ConclusionOur results suggest that most, if not all, cytoplasmic aaRS may become autoantigenic. Autoantibodies against new aaRS may be found in plasma of patients previously classified as seronegative with potential high clinical relevance.

Widespread Mechanic's Hands in Antisynthetase Syndrome With Anti-OJ Antibody.

Mechanic's hand is a representative manifestation of antisynthetase syndrome (AS) but is observed in other forms of dermatomyositis1 This is a nonpruritic, hyperkeratotic, and scaly eruption on the ulnar side of the thumb and radial side of other fingers; occasionally the palms, fingertips, and feet are involved. However, a widespread type affecting the whole palm and fingers is extremely rare in anti-OJ antibody.

Clinical characteristics of Vietnamese patients with idiopathic inflammatory myopathies and autoantibodies to aminoacyl-transfer RNA synthetases.

To assess clinical phenotypes of anti-aminoacyl-transfer RNA synthetases (aaRS) autoantibodies in Vietnamese patients of Kinh ethnicity with idiopathic inflammatory myopathies (IIM). In a cross-sectional study 23 patients with anti-aaRS autoantibodies were compared to 36 patients with other myositis-specific antibodies and to 69 seronegative patients with IIM. Assessments included muscle performance, extra-muscular involvement, and disease activity according to the International Myositis Assessment and Clinical Studies (IMACS). Sera were tested by a line immunoassay (Euroline Myositis Profile 4). The frequency of anti-Jo-1 antibodies was 56.5%, anti-EJ antibodies 26.1%, and anti-PL-7 antibodies 17.4%, while anti-PL-12 and anti-OJ antibodies were not present in any case. All patients with anti-aaRS autoantibodies had signs of myositis. At time of investigation 22/23 patients had muscle weakness, 52.2% arthritis, 34.8% Raynaud's phenomenon, 73.9% fever, 14.3% mechanic's hands and 56.5% dysphagia. Interstitial lung disease was present in 52.2%, and pulmonary hypertension in 56.5%. The anti-aaRS autoantibody positive group had higher disease activity in the domains of skin and pulmonary disease compared to the seronegative group and had lower disease activity in skeletal disease compared to the anti-melanoma differentiation-associated protein 5-positive patients. The clinical presentation of antisynthetase syndrome was similar between the aaRS autoantibody specificities with the exception of more frequent pulmonary hypertension in anti-Jo-1 positive patients. Different aaRS autoantibody specificities may vary between different ethnic populations for reasons that still need to be clarified. Furthermore, the high frequency of pulmonary hypertension is noteworthy but otherwise clinical manifestations associated with aaRS autoantibodies did not differ from other ethnic populations.

Mechanic's Hands and Drug Eruption to Hydroxychloroquine: Precious Diagnostic Tools

The clinical diagnosis of dermatomyositis can be difficult and challenging. In the right clinical setting and with the use of the recently described myositis specific antibodies, an earlier diagnosis can be made and therapeutic approaches promptly started, minimizing the risk of disease progression. We herein present a case of a patient with mechanic’s hands and a previously documented drug eruption to hydroxychloroquine. Although these clinical findings are not pathognomonic of dermatomyositis, in the right clinical scenario, they are precious diagnostic tools.

Mechanic hands: clinical and capillaroscopy manifestations of patients with connective tissue diseases presented with and without mechanic hands.

The condition known as 'Mechanic's Hands' is a thickened, hyperkeratotic eruption, which is bilaterally symmetric along the fingers, and often occurs in patients with some connective tissue diseases. Nail fold capillaroscopy is a non-invasive technique for evaluation of connective tissue diseases. We evaluated the prevalence of mechanic hands in patients with connective tissue diseases and compared the clinical manifestations and capillaroscopic changes in the patients with and without mechanic hands. The clinical manifestations and capillaroscopy of 576 patients with scleroderma, dermatomyositis, systemic lupus erythematosus, Sjogren's syndrome, undifferentiated and mixed connective tissue diseases were evaluated and compared in patients with and without mechanic hands. A total of 576 patients were enrolled. Mechanic hands were observed in 17.2% of patients: 50% of mixed connective tissue disease, 35% of dermatomyositis, 15.4% of scleroderma, 14.9% of undifferentiated connective tissue disease, 14.3% of Sjogren's syndrome, and no patient with SLE. Among them, 80.8% had abnormal capillaroscopic findings. In dermatomyositis patients, Raynaud's phenomenon, anti-Jo-1 positivity, and some capillaroscopy findings were detected more frequently in patients with mechanic hand. In scleroderma, positive Scl70 and capillary loss were observed more frequently in patients without mechanic hands. Mechanic hands can be a presenting sign of some systemic connective tissue diseases. Probably, finding this sign on examination, especially together with Raynaud's phenomenon or abnormal capillaroscopy, can be helpful in the early diagnosis of the connective tissue diseases and can be used as a predictive and prognostic tool in future studies.

Dermatomyositis: A dermatological perspective

Dermatomyositis (DM) is an autoimmune idiopathic inflammatory myopathy which has distinctive skin lesions, specific autoantibodies, and most importantly, symmetrical proximal muscle weakness and muscle inflammation of subacute onset. Some cases of DM present with only characteristic skin lesions without muscle disease and referred to as clinically amyopathic DM. Characteristic skin lesions include violaceous discoloration around the eyelids (heliotrope rash), erythematous rash over the face, erythematous scaly papules over the interphalangeal and metacarpophalangeal joints (Gottron's papules), erythema over the same joints (Gottron's sign), photosensitive pruritic erythema over the anterior chest and neck (V-sign), erythema extending to the shoulders and back (Shawl sign), distorted cuticles with dilated capillary loops over the proximal nail folds, and hyperkeratotic fissured skin over the palms and lateral aspect of the fingers (Mechanic's hands). Causes for mortality and morbidity in DM include malignancy, respiratory disease, and cardiac disease. After ruling out internal associations, Mangement involves photoprotection and medications including systemic corticosteroids, antimalarials, mycofenolate mofetil, biologicals and IV immunoglobulins, depending upon patient parameters.

Mechanics hands in patients with antisynthetase syndrome: 25 cases

Antisynthetase antibodies (ASA) are directed against aminoacyl-tRNA-synthetases, ubiquitous enzymes of which eight types have hitherto been described. They are seen primarily in antisynthetase syndrome (ASS), in which diffuse interstitial lung disease is associated with inflammatory myopathy, joint involvement and cutaneous signs, in particular mechanic's hands. The aim of this study was to determine the prevalence and semiological characteristics of cutaneous involvement in patients presenting ASA. We carried out a retrospective study of the medical files of patients with ASA diagnosed at the Strasbourg University Hospital between 1994 and 2009. We identified 22 women and 3 men presenting ASS (n=21), dermatomyositis (n=3) or sclerodermatomyositis (n=1). Mean age at the time of diagnosis was 56 years (12-79). The most commonly seen antibodies were anti-Jo1 (n=19), with the other cases of ASA involving anti-PL12 (n=3), anti-PL7 (n=2) and anti-EJ (n=1) antibodies. Five patients died from pulmonary complications. Mechanic's hands (characteristic plaques and papules along the edge of the first fingers on both hands) were found in 10 patients with ASS (7 cases) or dermatomyositis (3 cases), at the time of diagnosis in 7 cases and during a systemic episode in 3 cases. Muscular involvement was seen in all patients: 9 had diffuse interstitial lung disease and 8 had joint involvement. Cutaneous signs regressed totally or partially in all patients under treatment; in 6 patients, worsening was seen during systemic episodes of the disease. One of the 10 patients died through pulmonary complications. Mechanic's hands are a key indicator in cases of ASA and its outcome is intimately linked with underlying systemic involvement, particularly pulmonary. The characteristic semiology enables this disorder to be recognised and allows differentiation from psoriasis or irritant contact dermatitis of the hands, and it does not vary according to antibody. Whether or not the disease is life-threatening is unaffected by the presence of this sign.

Muscular and extramuscular clinical features of patients with anti-PM/Scl autoantibodies.

To define the clinical features of myositis patients with anti-PM/Scl-75 and/or anti-PM/Scl-100 autoantibodies at disease onset and during the course of disease and compare them to patients with other forms of myositis. In this longitudinal cohort study, the prevalence and severity of clinical features at disease onset and during follow-up were compared between anti-PM/Scl-positive patients and those with the antisynthetase syndrome (AS), dermatomyositis (DM), and immune-mediated necrotizing myopathy (IMNM). Forty-one anti-PM/Scl-positive, 132 AS, 178 DM, and 135 IMNM patients were included. Although muscle weakness was a presenting feature in just 37% of anti-PM/Scl-positive patients, 93% eventually developed weakness. Unlike the other groups, anti-PM-Scl-positive patients had more severe weakness in arm abductors than hip flexors. Interstitial lung disease was a presenting feature in just 10% of anti-PM/Scl-positive patients, but occurred in 61% during follow-up; fewer patients with DM (13%, p < 0.001) and IMNM (6%, p < 0.001) and more patients with AS (80%, p < 0.05) developed interstitial lung disease during the course of disease. Mechanic's hands (80%), Raynaud syndrome (78%), sclerodactyly (66%), telangiectasias (66%), esophageal reflux disease (61%), subcutaneous edema (46%), puffy hands (39%), and calcinosis (39%) occurred more frequently in anti-PM/Scl-positive patients than in the other groups. Although 30% of anti-PM/Scl-positive patients met criteria for systemic sclerosis, less than 5% had renal crisis or finger ulcerations. No differences were found between patients with only anti-PM/Scl-100 or only anti-PM/Scl-75 autoantibodies. Unlike patients with DM, AS, or IMNM, anti-PM/Scl-positive patients have weaker arm abductors than hip flexors. Anti-PM/Scl-positive patients also have the most extensive extramuscular features.

"Hiker's feet": a novel cutaneous finding in the inflammatory myopathies.

Mechanic's hands is a well-characterized manifestation of select idiopathic inflammatory myopathy (IIM) syndromes. Less well characterized is the hyperkeratosis of the toes and plantar surface of the feet that can also accompany these disorders. We aim to describe common pedal signs in the context of IIM, and suggest that it may be another key feature in the presentation of these syndromes. A cohort of 2145 myositis patient charts gathered since 2003 were retrospectively reviewed using the key search terms "mechanic's feet" and/or "mechanic's foot." Charts that included either phrase were further reviewed for clinical characteristics. Nine patients were identified with documentation describing "mechanic's feet" or "mechanic's foot." All nine affected individuals carried a diagnosis of DM, seven of whom also met criteria for antisynthetase syndrome. In eight patients (89%), it presented in conjunction with mechanic's hands. Six (67%) presented with anti-Jo-1 antibodies, and three (33%) were seronegative. Although the term "mechanic's feet" has been used to describe this clinical finding in patients in our myositis cohort, we propose the term "hiker's feet," given that the presentation resembles a callousing pattern more typical of avid hikers or long-distance walkers. Prevalence data are not yet known but should be considered for further study. If the presenting signs of IIM are expanded to include hiker's feet, it could aid in not only diagnosis and management but also provide insights into the pathophysiology of these diseases.

Mechanic's hands lesions in antisynthetase syndrome.

Mechanic's hands lesions in antisynthetase syndrome.

Idiopathic Inflammatory Myopathies: A Case of a Woman with Antisynthetase Syndrome

Antisynthetase Syndrome (ASS) is a rare disorder characterized by myositis, Raynaud's phenomenon, fever, interstitial lung disease, mechanic's and arthropathy associated with the presence of antibodies against aminoacyl transfer RNA synthetases (anti-ARS), especially anti-Jo-1. Patients with progressive interstitial lung disease have a poorer prognosis and lung involvement is the leading cause of morbidity and mortality. Presence of Mechanic's hands with hyperkeratosis on the palmar side of the fingers and fissuring at their tips and radial margins, is an important diagnostic manifestation of this syndrome. Early diagnosis followed by immunosuppressive therapy can significantly improve the health status of these patients and increase their quality of life. This article reviews some of the most important pathophysiologic, clinical and diagnostic features of Inflammatory Idiopathic Miopathies and presents the case of a woman with myalgia, malaise, cough, chest pain and skin involvement who was diagnosed with Antisynthetase Syndrome and received systemic treatment.

Anti-synthetase syndrome. Mechanic's hands

Numerosas variedades de amiloidosis afectan al sistema osteoarticular. Las manifestaciones más variadas se dan en la amiloidosis inmunoglobulínica (amiloidosis AL). Es muy importante conocerlas, ya que, aunque no comprometen el pronóstico vital, pueden revelar esta enfermedad, cuyo diagnóstico suele ser tardío. Los depósitos afectan a estructuras articulares y periarticulares. La afección articular es polimorfa, pero la forma más frecuente es una poliartritis bilateral simétrica, de instauración progresiva, que afecta sobre todo a hombros, muñecas, articulaciones metacarpofalángicas, interfalángicas proximales y rodillas, semejante a la artritis reumatoide. El síndrome del túnel carpiano es frecuente y a menudo revela la enfermedad. En casos más inusuales, la amiloidosis AL puede presentarse como una seudopoliartritis rizomélica o una enfermedad de Horton. La afectación muscular se manifiesta por un déficit, más asociado a una atrofia que a una seudohipertrofia. La infiltración de la médula es frecuente en la amiloidosis AL, aunque sin un síntoma definido. En casos más excepcionales, la amiloidosis se expresa en forma de tumor óseo (amiloidoma), aislado o integrado en una forma multisistémica de amiloidosis AL. La amiloidosis por beta-2-microglobulina sólo aparece en pacientes sometidos a diálisis durante largo tiempo. En Francia, se ha vuelto excepcional gracias a los avances en las técnicas de depuración y, sobre todo, al desarrollo del trasplante renal. Esta forma de amiloidosis se manifiesta también por un síndrome del túnel carpiano, artropatías crónicas y, sobre todo, por una espondiloartropatía destructiva. La amiloidosis de la transtiretina, tanto en su forma genética como en su forma senil, también causa un síndrome del túnel carpiano. Con el envejecimiento, se observan depósitos amiloides en las estructuras articulares (meniscos, cartílagos, discos intervertebrales, articulaciones), de los que no se conoce con precisión su naturaleza y su significado clínico.