Objective Anti-melanoma differentiation-associated protein 5 (MDA5) positive dermatomyositis (DM) is associated with rapidly progressive interstitial lung disease (RP-ILD) and high mortality. This multicentre retrospective study aimed to identify predictors for mortality and RP-ILD. Methods Anti-MDA5 positive DM patients were identified from the Hong Kong Myositis Registry and the Clinical Data Analysis and Reporting System. Clinical characteristics were reviewed. Risk factors for mortality and RP-ILD were identified. Results Among the 116 recruited patients, 100 (86.2%) had ILD, 47 (40.5%) had RP-ILD and 44 (37.9%) patients died. Cox regression analysis revealed that RP-ILD (HR 9.735, 95%CI 3.905–24.272), age >52 (HR 4.750, 95%CI 1.692–13.333), ferritin level >2800pmol/l (HR 3.042, 95%CI 1.323–6.997) and lactate dehydrogenase (LDH) >400 IU/l (HR 2.290, 95% CI 1.009–5.198) were independent predictors of mortality. With regard to RP-ILD, analyses showed that potential predictors at baseline included age >50 years old (HR 2.640, 95%CI 1.277–5.455), LDH >300IU/l (HR3.189, 95%CI 1.469–6.918), fever (HR 1.903, 95% CI: 0.956–3.790) and neutrophil to lymphocyte ratio (NLR) >7.0 (HR 1.967, 95%CI 0.942–4.107). We proposed a prediction model, based on Fever, LDH, Age and White cell count (“FLAW”), to stratify risk of development of RP-ILD. The probability of RP-ILD in a patient with a score of 4 was 100%. A small internal validation cohort showed the odds of RP-ILD with FLAW scores of 0, 1, 2 and 3 were 0%, 0%, 42.9% and 75% respectively. Conclusions Anti-MDA5-associated RP-ILD is significantly associated with poor survival rates. The “FLAW” model maybe useful to predict the development of RP-ILD.
With limited access to biologic disease-modifying antirheumatic drugs, treatment efforts toward DAS28 and SDAI remission had similar effects in preventing the progression of arterial stiffness at 1 year. However, achieving sustained DAS28 remission was associated with a significantly greater improvement in PWV. [Clinical Trial registration: Clinicaltrial.gov NCT01768923.].
Urinary IL-22 mRNA level is decreased in patients with SLE with proliferative nephritis, while urinary IL-10 mRNA levels correlates with its intrarenal mRNA level and disease activity. Urinary IL-10 mRNA levels may also predict treatment response. These results suggest that urinary mRNA levels of IL-10 and IL-22 might be used as biomarkers for assessing disease activity and risk stratification in lupus nephritis.
Adult-onset Still's disease (AOSD) is a multi-systemic inXammatory disorder aVecting all ethnic groups. Even though there are several published diagnostic criteria, AOSD remains diYcult to diagnose clinically due to the overlapping features with infective, neoplastic and other rheumatological conditions [1,2]. The problem is further complicated by the fact that treatments for AOSD, including steroid and immunosuppressive agents, can worsen the clinical course of the above conditions.Regarding laboratory markers, a markedly increased serum ferritin concentration is a common diagnostic marker among AOSD patients in active disease [3][4][5]. Generally speaking, serum ferritin concentration above 2,000 g/L is considered to be consistent with AOSD. Fautrel et al. [6] reported that a Wvefold increase in serum ferritin over upper limit of normal had 80% sensitivity and 41% speciWcity for AOSD. As marked elevation of serum ferritin concentration can also be seen in other disorders, the low speciWcity limits its diagnostic use. The percentage of glycosylated ferritin (GF), an isoform of ferritin, over total ferritin oVers additional diagnostic value. In healthy participants, 50-80% of ferritin is glycosylated. In inXammatory diseases, the glycosylated portion drops to 20-50%, while in AOSD, it is often ·20%. Using ·20% as the cut-oV, the sensitivity and speciWcity of percentage GF for AOSD were 80 and 66%, respectively. The combination of a percentage GF · 20% with ferritin over Wve times of normal produced a sensitivity of 43% and speciWcity of 93% [6].We decided to develop an assay for the measurement of serum GF. Based on method described by Vignes et al. [7], GF could be easily separated from non-glycosylated ferritin by dividing the target serum sample into two aliquots, adding concanavalin A (Con-A) Sepharose 4B to one aliquot and only Sepharose 4B to the other aliquot and incubating them in a water bath for 2 h at room temperature. GF bound to Con-A, whereas non-glycosylated ferritin did not. The two aliquots were then centrifuged at 3,000 rpm for 15 min. The non-glycosylated ferritin unbound to Con-A was recovered in the supernatant and measured by immunometric assay. Accordingly, the non-glycosylated ferritin and total ferritin concentrations could be determined in the aliquots with Con-A Sepharose 4B and Sepharose 4B, respectively. The percentage GF was calculated from the diVerence between total ferritin and non-glycosylated ferritin divided by total ferritin.We had retrospectively analysed archived serum samples taken on admission from 5 newly diagnosed AOSD patients referred to our laboratory, in whom diagnosis was made clinically according to the Yamaguchi's criteria. These 5 patients had serum ferritin concentrations of 8,047-41,479 g/L and percentage GF of 1.9-16.2%. The combined results were compatible with the diagnosis of AOSD. We had also randomly selected 15 patient samples with ferritin level over 2,000 g/L. These were patients with chronic anaemia requiring regular transfusion and patients with fulm...
Anti‐melanoma differentiation‐associated gene 5 (MDA5) dermatomyositis is characterized by serological detection of anti‐MDA5 antibody and rapidly progressive interstitial lung disease. In this study, the largest cohort of skin biopsies to date of anti‐MDA5 dermatomyositis was reviewed and compared with cases of dermatomyositis with negative serology. Findings contribute to the histological diagnosis and evaluation of the severity of cutaneous inflammation in anti‐MDA5 dermatomyositis. Skin biopsies collected over a 7‐year period from individuals with clinically and histologically confirmed dermatomyositis with anti‐MDA5 serology were reviewed. A total of 46 cases with 17 anti‐MDA5 positive cases were retrieved. Patients with positive antibody were younger (53.7 vs. 60.6 years, p = .013). No differences in epidermal changes (p > .05) were observed. Pertaining to interface changes, anti‐MDA5 dermatomyositis showed a higher degree of pigmentary incontinence (p = .014), suggesting increased and sustained cutaneous inflammation. Periodic acid–Schiff (PAS) stain demonstrated a greater degree of basement membrane thickening (p = .045). Other parameters, including dermal inflammation, dermal mucin deposition and vasculitic/vasculopathic features did not show statistical difference between anti‐MDA5 positive and negative dermatomyositis (p > .05). Findings suggest increased cutaneous inflammation for anti‐MDA5 dermatomyositis. In skin biopsies, marked pigmentary incontinence or basement membrane thickening should raise suspicion of anti‐MDA5 dermatomyositis.
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