Prognostic analysis of MDA5-associated clinically amyopathic dermatomyositis with interstitial lung disease.

OBJECTIVE: To investigate the prognostic factors of patients with anti-melanoma differentiation-associated gene 5 (MDA5) positive clinically amyopathic dermatomyositis (CADM) and interstitial lung disease (ILD). METHODS: A retrospective analysis was conducted on clinical data of 125 patients with anti-MDA5 + CADM-ILD collected from 10 branches in eastern China between December 2014 and December 2022. Prognostic factors were analyzed using χ2 test, Log-rank test, COX and logistic regression analysis. RESULTS: In this cohort, 125 anti-MDA5 + CADM-ILD patients exhibited a rapidly progressive interstitial lung disease (RPILD) incidence of 37.6%, and an overall mortality rate of 24.8%. One patient was lost to follow-up. After diagnosis of RPILD, a mortality rate of 53.2% occurred in patients died within 3 months, and that of 5.6% appeared in those who survived for more than 3 months. Multiple factor analysis revealed that C-reactive protein (CRP) ≥ 10 mg/L (p = 0.01) and recombinant human tripartite motif containing 21 (Ro52) (+) (p = 0.003) were associated with a higher risk of RPILD in anti-MDA5 + CADM-ILD patients; CRP ≥ 10 mg/L (p = 0.018) and the presence of RPILD (p = 0.003) were identified as the factors influencing survival time in these patients, while arthritis was the protective factor (p = 0.016). CONCLUSION: Patients with anti-MDA5 + CADM-ILD will have a higher mortality rate, and the initial 3 months after diagnosis of RPILD is considered the risk window for the dismal prognosis. Patients with CRP ≥ 10 mg/L, Ro52 (+) and RPILD may be related to a shorter survival time, while patients complicated with arthritis may present with relatively mild conditions.

Autoimmune pulmonary alveolar proteinosis during the treatment of nonspecific interstitial pneumonia complicated by clinically amyopathic dermatomyositis: A case report.

A 46-year-old male was treated with corticosteroids for nonspecific interstitial pneumonia (NSIP). He was referred to our hospital and admitted for worsening dyspnea and diffuse ground-glass opacity on chest computed tomography (CT) during corticosteroid treatment. Gottron's sign was observed, and the patient was diagnosed with clinically amyopathic dermatomyositis on skin biopsy. We increased the corticosteroid dose and added immunosuppressive agents; however, the opacity on the chest CT worsened. Based on periodic-acid-Schiff-positive granular material in the bronchoalveolar lavage fluid and the presence of anti-GM-CSF antibodies, the patient was diagnosed with autoimmune pulmonary alveolar proteinosis (APAP). The concentration of anti-GM-CSF antibodies in preserved serum was also elevated when the patient was diagnosed with NSIP. Thus, we assumed that NSIP and APAP coexisted, and that APAP manifested during immunosuppressive therapy. When exacerbation is observed during the treatment of interstitial pneumonia with immunosuppressive agents, it is necessary to consider APAP.

Anti-nuclear matrix protein 2 antibody-positive amyopathic dermatomyositis presenting in a patient with prostate cancer: A case report.

Nuclear matrix protein (NXP-2) positive amyopathic dermatomyositis (DM) may present without classic symptoms like muscle weakness, dysphagia, and edema, and mimic conditions like cutaneous lupus. Given DM's association with malignancy and interstitial lung disease, prompt and accurate diagnosis is important. Testing for myositis-specific antibodies aids diagnosis in ambiguous cases.

Amyopathic dermatomyositis may be on the spectrum of autoinflammatory disease: A clinical review.

Systemic autoinflammatory diseases (SAIDs) are distinct from autoimmune diseases. The former primarily results from abnormal innate immune response and genetic testing is crucial for disease diagnosis. Similar cutaneous involvement is a main feature for both SAID and dermatomyositis (DM), so they can be confused with each other. A literature search of PubMed and MEDLINE was conducted for relevant articles. The similarities and differences between these two types of diseases were analyzed. We found phenotypic similarities between these two types of disorders. Accumulating data supports a major role of the innate immune system and a similar cytokine profile. Molecular testing using an autoinflammatory disease gene panel may help identify SAID patients from the DM population and may offer therapeutic benefit using interleukin-1 (IL-1) inhibitors. A subset of DM, notably amyopathic dermatomyositis in the absence of autoantibodies may be on the spectrum of autoinflammatory disease.

Clinical characteristics and symptom progression of dermatomyositis subtypes: A retrospective analysis of a prospective database.

BACKGROUND: Disease characteristics of classic dermatomyositis (DM) and clinically amyopathic DM (CADM) are well established, but there exists limited knowledge on the disease progression of these subtypes. OBJECTIVE: The objective of this study was to longitudinally track and characterize classic DM and CADM patients who experience changes in disease presentation. METHODS: We conducted a retrospective review of prospectively collected data on 269 DM patients from a longitudinal database. RESULTS: A total of 51% of the patients had classic DM and 49% had CADM. Forty percent of the classic DM patients became postmyopathic (PmDM). Median Cutaneous Dermatomyositis Disease Area and Severity Index activity (CDASI-A) score was lower in PmDM patients than in classic DM patients (13.0 vs 16.0), but 45% of the PmDM patients had CDASI-A scores > 14. Five percent of the CADM patients developed muscle involvement. Compared with CADM patients, those who developed muscle symptoms had milder skin disease before subtype conversion (median CDASI-A 12.0 vs 16.0) and at subtype conversion (median CDASI-A 9.0 vs 16.0). LIMITATIONS: This was a retrospective study conducted at a single tertiary-care dermatology clinic. CONCLUSIONS: Forty percent of the classic DM patients became PmDM. The majority continue with muscle disease, and many continue to have moderate/severe skin disease. CADM has a low risk of progressing to muscle disease, with the extent of skin disease as a potential predictive factor.

Anti-MDA5 Antibody-positive Clinically Amyopathic Dermatomyositis with Rapidly Progressing Interstitial Lung Disease Successfully Treated by Initiation of Combined Immunosuppressive Therapy Plus Plasma Exchange and Subsequently Switching Tacrolimus to Tofacitinib.

A 36-year-old man with inverse Gottron's sign was admitted for clinically amyopathic dermatomyositis (CADM) with rapidly progressive interstitial lung disease (RP-ILD). Early addition of plasma exchange (PE) to triple therapy improved severe respiratory failure and transiently decreased serum ferritin levels and anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) titers. Furthermore, switching from tacrolimus to tofacitinib resulted in disease remission. Recognition of the inverse Gottron's sign may allow for the earlier diagnosis of anti-MDA5 Ab-positive dermatomyositis, and early addition of PE to triple therapy and administration of tofacitinib in refractory cases may be effective for anti-MDA5 Ab-positive CADM with RP-ILD under life-threatening conditions.

Effectiveness and safety of plasma exchange for anti-MDA5 antibody-positive clinically amyopathic dermatomyositis with rapidly progressive interstitial lung disease refractory to intensive immune suppression therapy: A case series.

INTRODUCTION: Clinically amyopathic dermatomyositis (CADM) with anti-melanoma differentiation-associated gene 5 (MDA5) antibody (Ab) with rapidly progressive interstitial lung disease (RP-ILD) is often refractory for intensive immunosuppression. In this study, we verified the effectiveness and safety of plasma exchange (PEx) for this lethal disease. METHODS: We retrospectively examined the clinical course and adverse effect (AE) of 12 patients with anti-MDA5 Ab-positive CADM between January 2017 and December 2021 in our hospital. RESULTS: Five out of six patients treated with simple PEx using fresh frozen plasma or 5% albumin survived with or without home oxygen therapy. Multiple PEx (15-20 times) were required to achieve satisfactory improvement as well as remission of CADM. The AEs caused by PEx were resolved using conventional methods. CONCLUSION: PEx might be a promising option for controlling the disease activity of anti-MDA5 Ab-positive CADM with severe RP-ILD and may contribute to better survival.

Anti-MDA5 antibody-associated clinically amyopathic dermatomyositis diagnosed after the onset of pulmonary hypertension.

Idiopathic inflammatory myopathies (IIM) are a group of highly heterogeneous systemic autoimmune diseases, of which clinically amyopathic dermatomyositis (CADM) is a distinct sub-type. Pulmonary hypertension (PH) is a life-threatening medical condition that can occur as a complication of connective tissue diseases. Herein, our report first suggests that PH can develop in CADM. A 48-year-old woman came to our hospital due to a 3 months history of facial edema and shortness of breath. Relevant examinations revealed Gottron's sign, normal creatinine kinase levels, elevated levels of mean pulmonary artery pressure, double-positive anti-MDA5 and anti-Ro52 antibodies, and typical pathological changes associated with myositis. The diagnosis of CADM combined with PH was considered. The patient responded well to the immunosuppression therapy and PH-related drug therapy. We provide further insights that patients with IIM need to undergo regular assessment of PH.

Comparison of characteristics and anti-MDA5 antibody distribution and effect between clinically amyopathic dermatomyositis and classic dermatomyositis: a retrospective case-control study.

Background: Clinically amyopathic dermatomyositis (CADM) is a distinct subtype of dermatomyositis (DM) characterized by typical DM cutaneous findings but with minimal or no evidence of myositis. It possesses unique features different from classic DM (CDM). Anti-melanoma differentiation-associated gene 5 (MDA5) antibodies were found in CADM and are thought to increase the risk of rapidly progressive interstitial lung disease (RP-ILD) and are present in both CADM and CDM patients, affecting their condition and prognosis. Nevertheless, no large-sample studies have compared all aspects concerning patients with CADM and those with CDM. This study aimed to investigate differences in clinical characteristics and risk factors for mortality between CADM and CDM and to clarify the distribution and impact of anti-MDA5 antibodies in patients with these conditions. Methods: A retrospective case-control study included 330 patients and collected and analyzed their clinical data from The First Affiliated Hospital of Shandong First Medical University and Shandong Provincial Hospital of Traditional Chinese Medicine between January 2015 and July 2022; all patients were followed up to evaluate changes in their condition and prognosis. Several new cohorts were designed around anti-MDA5 antibodies to explore their distribution and impact in CADM and CDM. Results: We found CADM to be associated with higher rates of mortality, 1-year mortality, interstitial lung disease (ILD), and RP-ILD than CDM. In CADM, RP-ILD, anti-MDA5 antibodies, and high ferritin and lactate dehydrogenase (LDH) levels were identified as independent risk factors for death. In CDM, the neutrophil-to-lymphocyte ratio, anti-MDA5 antibodies, and high ferritin levels were shown to be independent risk factors for death, whereas mechanic's hand was considered a protective factor against it. Anti-MDA5 antibody-positive patients did not exhibit any significant difference based on whether they belonged to the CADM or CDM groups. When no anti-MDA5 antibody-positive patients participated, the ferritin levels and rates of RP-ILD and ILD were still higher in CADM than in CDM; however, such differences decreased, whereas the LDH levels, rates of mortality, and 1-year mortality did not differ. Anti-MDA5 antibody-positive patients consistently showed higher LDH and ferritin levels, lower lymphocyte levels, higher probability of RP-ILD and ILD, and worse prognosis than anti-MDA5 antibody-negative patients, irrespective of whether the patients had DM, CADM, or CDM. Conclusion: Patients with CADM exhibit relatively worse symptoms, serological findings, and prognosis than those with CDM. Furthermore, patients with CADM and those with CDM have commonalities and differences in risk factors for death. Moreover, CADM may necessitate earlier and more aggressive treatment strategies than CDM. Anti-MDA5 antibodies occur at a high level in patients with CADM, not only affecting the symptoms and prognosis of DM but also having a non-negligible impact on the differences between CADM and CDM. Hence, screening for anti-MDA5 antibodies in patients with CADM and CDM is extremely essential.

Extended-Release Tofacitinib Therapy for a MDA5 Antibody-Positive Amyopathic Dermatomyositis Patient with Early-Stage Interstitial Lung Disease.

Introduction: In East Asia, more than half of patients with amyopathic dermatomyositis (ADM) have interstitial lung disease (ILD). There is up to 50% 6-month mortality in MDA5-positive ILD refractory to corticosteroid (CS) combined with immunosuppressant therapy. Patient Details: A 39-year-old local woman had a 1-month history of reddish-purple discoloration around the eyelids (heliotrope rash), and erythematous areas on the upper back and posterior neck (shawl sign) as well as on the front of her chest (V sign), followed by dry cough and mild dyspnea for 1 week. She had normal muscle strength, muscle-enzyme concentrations, and muscular magnetic resonance images. Laboratory tests showed hypoxemia, increased ferritin and CRP levels, and positive MDA5 antibodies. High-resolution chest computed tomography revealed bilateral ground-glass opacity. She received a diagnosis of anti-MDA5-positive ADM with early-stage ILD. Intervention: Pulse methylprednisolone and cyclophosphamide therapies were initiated, followed by high-dose CS treatment. Immediate-release twice-daily 5 mg tofacitinib (Tof) has been demonstrated to be effective induction therapy for early-stage ILD in anti-MDA5-positive ADM. Owing to the patient's preference for once-daily therapy, 11 mg extended-release Tof was prescribed 4 weeks after starting the initial pulse CS treatment for ILD. Outcomes: Respiratory symptoms and cutaneous manifestations were absent and the use of CS spared 5 months after initiating Tof therapy. Laboratory examinations exhibited normalized ferritin/oxygen levels, and chest images displayed completely resolved pulmonary infiltration. ILD remains under adequate control with Tof monotherapy without recurrence at 5 months. Lessons: Owing to a rapid decline in higher mortality in anti-MDA5-positive ADM patients with ILD, early detection with prompt initiation of extended-release Tof induction therapy might achieve a beneficial outcome.

When a Painful Rash Keeps Recurring: A Case of Seronegative Amyopathic Dermatomyositis Without Neurological Sequelae.

We present a case of seronegative amyopathic dermatomyositis (SADM). This clinical entity should be considered in the differential diagnosis of patients with recurring, painful erythematous skin manifestations, and requires close monitoring for the development of neurological manifestations and malignancy. SADM is a rare autoimmune disease that affects the skin and muscles. It is considered a subtype of dermatomyositis (DM), which is a systemic autoimmune disease. The exact cause of SADM is not fully understood but is believed to involve a complex interplay between genetic, environmental, and immunological factors. The diagnosis of SADM is typically made based on clinical evaluation, blood tests, muscle biopsy, and skin biopsy. Treatment options for SADM may include corticosteroids, immunosuppressive drugs, and other supportive measures to manage symptoms and prevent disease progression. A 30-year-old female presented with symptoms of intermittent burning, painful rash primarily on the hands and face. Her medical history was remarkable for a six-year history of multifocal joint pain, chronic low back pain, and intermittent, painful recurring rash in the upper body (face, neck, and chest). Neurological examination revealed scalp tenderness and arthralgia in the upper extremities, with normal motor strength examination. Skin findings included described an erythematous rash on the arms and hands bilaterally. Skin punch biopsy showed compact orthokeratosis, atrophy of the epidermis, interface changes, and increased dermal mucin on the colloidal iron stain, which are suggestive of DM. Electromyography and nerve conduction study were normal. The MRI of the left thigh was normal. C3 and C4 levels were reduced. The extended muscle-specific myositis panel including MDA5 was negative. The patient was placed on a multidrug regimen, including methotrexate, hydroxychloroquine, and prednisone. Within one year of follow-up, she was found to have reductions in skin manifestation and flare-ups. Clinicians should consider amyopathic DM (ADM) in the differential diagnosis of patients with recurring, painful skin manifestations. This condition can be easily overlooked as the development of neurological sequelae may be present much later in the course. We highlight the need for a multi-disciplinary management approach for patients with this unique diagnosis. Close monitoring for the development of neurological manifestations and associated sequelae including malignancy is recommended.

Anti-melanoma differentiation-associated gene 5 and anti-Ro52 antibody-dual positive dermatomyositis accompanied by rapidly lung disease: Three case reports.

BACKGROUND: Clinically amyopathic deramatomyositis was manifested as the various cutaneous dermatomyositis (DM) manifestations without muscle weakness. Anti-melanoma differentiation-associated gene 5 (anti-MDA5) and anti-Ro52 antibody-dual positive clinically amyopathic DM patients are at a high risk of developing rapidly progressive interstitial lung disease, and they exhibit an immensely high half-year mortality. CASE SUMMARY: We presented three patients with anti-MDA5 and anti-Ro52 antibody-dual positive DM patients and we reviewed the previous studies on the link between anti-MDA5 and anti-Ro52 antibody-dual positive DM. Although we aggressively treated these patients similarly, but they all exhibited different prognoses. We reviewed the importance of clinical cutaneous rashes as well as the pathogenesis and treatment in the dual positive anti-MDA5 and anti-Ro52 associated DM. CONCLUSION: Patients with anti-MDA5 anti-Ro52 antibody-dual positive DM should be accurately diagnosed at an early stage and should be treated aggressively, thus, the patient's prognosis can be significantly modified.

IFN-γ: An overlooked cytokine in dermatomyositis with anti-MDA5 antibodies.

Dermatomyositis with anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 DM) is a rare autoimmune disease, often complicated by life-threatening, rapidly progressive interstitial lung disease. Additional manifestations of the disease include skin lesions, vascular abnormalities, joints and muscles pain. Despite its clinical significance, the pathogenesis of anti-MDA5 DM remains largely unknown. Currently, the disease is perceived as driven by type I interferon (IFN) whose expression is increased in most of the patients. Importantly, the regulation of IFN-γ is also altered in anti-MDA5 DM as evidenced by the presence of IFN-γ positive histiocytes in the lungs of patients, and the identification of autoantibodies that directly stimulate the production of IFN-γ by mononuclear cells. This review critically examines the pathogenesis of the disease, shedding light on recent findings that emphasize a potential role of IFN-γ. A novel conceptual framework is proposed, which integrates the molecular mechanisms altering IFN-γ regulation in anti-MDA5 DM with the known functional effects of IFN-γ on key tissues affected during the disease, such as the lungs, skin, and vessels. Understanding the precise role and relevance of IFN-γ in the pathogenesis of the disease will not only enhance the selection of available therapies for anti-MDA5 DM patients but also pave the way for the development of new therapeutic approaches targeting the altered molecular pathways.

Anti-Ro52/Anti-nuclear Matrix Protein 2 Positive Clinically Amyopathic Dermatomyositis Presented Only With Interstitial Lung Disease.

This report contemplates a unique case of clinically amyopathic dermatomyositis (CADM) that presented as interstitial lung disease. The patient was a 55-year-old woman who showed up with progressive exercise intolerance and a dry cough without muscular or dermatological clinical manifestations. Diagnostic workup and imaging revealed the presence of interstitial lung disease, and further evaluation led to a positive autoimmune panel for anti-nuclear matrix protein 2 (anti-NXP2) and anti-Ro52 antibodies, establishing the diagnosis of anti-NXP2 plus anti-Ro52 antibodies-positive amyopathic idiopathic inflammatory dermatomyositis. The patient was started on intravenous corticosteroids. She showed improvement on her chest X-ray (CXR) and was then switched to oral corticosteroids. After six months of steroid treatment, corticosteroids were stopped, and the patient was re-evaluated one month later disease relapse.

Pitfalls in the Diagnosis and Management of an Unusual Presentation of Clinically Amyopathic Dermatomyositis: A Case Report Written With the Assistance of ChatGPT.

Clinically amyopathic dermatomyositis (CADM) is a rare form of dermatomyositis. Patients with this condition present with the typical skin findings of dermatomyositis but lack the characteristic muscle weakness associated with dermatomyositis. This case presentation highlights the unusual clinical manifestation of CADM in a 49-year-old Vietnamese female. The patient initially presented with persistent hyperpigmented plaques on her hands, which did not respond to the standard treatment for atopic dermatitis. The patient later developed respiratory failure and lung fibrosis in Vietnam. This case underscores the challenges in diagnosing and managing CADM, particularly in patients with atypical presentations, and emphasizes the difficulties in managing such cases of CADM in the community setting.

[Clinically amyopathic dermatomyositis : a rare entity associated with acute and severe interstitial lung disease]. / La dermatomyosite amyopathique : une entité rare associée à une pneumopathie interstitielle fulminante.

Dermatomyositis is a rare disease of unknown etiology characterized by a severe inflammatory myopathy associated with a cutaneous syndrome. Dermatomyositis is associated with multisystemic disorders mostly represented by cardiac, pulmonary and articular involvements, which are particularly associated with a bad prognosis. We report a case of a 50-year-old patient suffering from dermatomyositis associated with an interstitial lung disease with a particularly fast and pejorative clinical evolution. The anti-Melanoma Differentiation-Associated gene 5 (anti-MDA5) antibodies are frequently associated with a severe and rapidly progressive lung disease without myositis named «amyopathic dermatomyositis¼. High blood levels of anti-MDA5 were found in our patient. Despite maximal immunosuppressive treatment and supportive care, he died 3 months after the diagnosis. Patients may present different antibodies that correspond to distinct clinical phenotypes of dermatomyositis. The anti-MDA5 is known to be a marker of clinically amyopathic dermatomyositis (CADM) associated with a rapidly progressive interstitial lung disease. Moreover, blood level of anti-MDA5 antibody predicts the response to treatment and survival in CADM.

La dermatomyosite est une maladie rare, d'étiologie inconnue, caractérisée par une myopathie inflammatoire associée à un syndrome cutané typique. Outre l'atteinte musculaire et cutanée, la dermatomyosite peut se manifester par des atteintes organiques, notamment pulmonaires, cardiaques et articulaires qui contribuent à la sévérité de la maladie. Nous rapportons le cas d'un patient âgé de 50 ans atteint d'une dermatomyosite compliquée d'une pneumopathie interstitielle d'évolution clinique particulièrement rapide et péjorative. Le patient présentait des anticorps anti-MDA5 (anti-Melanoma Differentiation-Associated gene 5), anticorps associés assez fréquemment à une atteinte pulmonaire sévère et rapidement progressive, ainsi qu'à une présentation particulière de la maladie appelée «dermatomyosite amyopathique¼. Malgré un traitement immunosuppresseur intensif, l'état pulmonaire du patient s'est rapidement aggravé, entraînant son décès par insuffisance respiratoire trois mois après le diagnostic. Cette histoire clinique illustre le fait que les patients atteints de dermatomyosite peuvent présenter différents anticorps qui correspondent à des phénotypes cliniques distincts. L'association entre anticorps anti-MDA5 et la pathologie pulmonaire interstitielle justifie qu'un screening des anticorps anti-MDA5 soit réalisé chez les patients porteurs d'une dermatomyosite. De plus, le titrage sanguin des anti-MDA5 est un facteur pronostique de la réponse au traitement et de la survie.

An autopsy case of anti-MDA5 antibody-positive amyopathic dermatomyositis with an initial manifestation of panniculitis on the left upper arm.

A 53-year-old man was presented with refractory panniculitis on the left upper arm that had persisted for 10 months. The patient was diagnosed with lupus profundus, wherein oral glucocorticoid therapy was initiated. Four months prior, ulceration was observed in the same area. Dapson was administered instead, scarring the ulcer but enlarging the panniculitis. Five weeks earlier, he developed a fever, productive cough, and dyspnoea. Three weeks earlier, a skin rash was observed on the forehead, left auricle posterior to the neck, and extensor aspect of the left elbow. Chest computed tomography showed pneumonia in the right lung, after which the patient's dyspnoea worsened. The patient was admitted and diagnosed with anti-MDA5 antibody-positive amyopathic dermatomyositis (ADM) based on skin findings, hyperferritinaemia, and rapidly progressive diffuse lung shadows. Glucocorticoid pulse therapy, intravenous cyclophosphamide, and tacrolimus were initiated, and later, plasma exchange therapy was combined. However, his condition worsened and required management with extracorporeal membrane oxygenation. The patient expired on day 28 after hospitalisation. An autopsy revealed hyalinising to fibrotic stages of diffuse alveolar damage. Strong expression of myxovirus resistance protein A was observed in three skin biopsy specimens from the time of initial onset, consistent with ADM. Anti-MDA5 antibody-positive ADM not only manifests typical cutaneous symptoms, but also rarely occurs with localised panniculitis, such as in the present case. In patients with panniculitis of unknown aetiology, the possibility of initial symptoms of ADM should be included in the differential diagnosis.

Soluble CD206 levels correlate with disease deterioration and predict prognosis of anti-MDA5 antibody-positive dermatomyositis related interstitial lung disease.

INTRODUCTION: The prognosis of anti-MDA5 antibody-positive dermatomyositis/clinically amyopathic dermatomyositis-associated interstitial lung disease (MDA5-DM/CADM-ILD) is poor. This study was to evaluate the effect of serum soluble CD206 (sCD206), a biomarker of macrophage activation, on predicting the interstitial lung disease (ILD) deterioration and prognosis for MDA5-DM/CADM-ILD. METHODS: Forty-one patients diagnosed with MDA5-DM/CADM-ILD were retrospectively included. The clinical data were analyzed. Serum sCD206 levels were measured in 41 patients and 30 healthy controls. The relation between sCD206 levels and ILD deterioration was assessed. Receiver operating characteristic (ROC) curve was generated to determine the optimal cut-off value of sCD206 for predicting outcome. The association between sCD206 and survival was examined. RESULTS: The median serum sCD206 level in patients was significantly higher than healthy controls (464.1 ng/mL vs. 349.1 ng/mL, P = 0.002). In DM/CADM patients, the sCD206 level was significantly higher in patients with acute/subacute interstitial lung disease (AILD/SILD) than those with chronic interstitial lung disease (CILD) (539.2 ng/mL vs. 309.4 ng/mL, P = 0.005). The AUC of sCD206 was 0.885 for predicting mortality (95% CI 0.779-0.990). Patients were divided into two groups: sCD206 high level group (≥400 ng/mL) and sCD206 low level group (<400 ng/mL). Patients with sCD206 high level had significantly decreased survival rate than those with low level (25% vs. 88%, P < 0.001). The adjusted hazard ratio of sCD206 for mortality was 1.003 (adjusted for age and gender, P < 0.001), with sCD206 high level associated with higher death risk (HR 4.857, P = 0.006). CONCLUSION: Serum sCD206 might be a potential predictor of ILD deterioration and prognosis for Chinese patients with MDA5-DM/CADM-ILD.