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Rheumatology AND Dermatomyositis [keywords]
- Adult dermatomyositis with bleeding ulcer in the pharynx. [Journal Article]
- Case Rep Otolaryngol 2014.:854841.
Dermatomyositis (DM) is one of the idiopathic inflammatory myopathies caused by complement-mediated vasculopathy or vasculitis in the muscle. Although the gastrointestinal (GI) mucosa has been reported to be involved as a result of vasculitis or vasculopathy, ulceration in the pharynx is a rare manifestation of DM. A 54-year-old woman complaining of muscle weakness in the extremities, low-grade fever, and dysphagia was diagnosed as having DM. Despite medical treatment with corticosteroids and immunosuppressive agents, her DM progressed rapidly, leading to exacerbation of the dysphagia. About 3 weeks after undergoing tracheostomy as a preventive measure against aspiration, the patient developed intractable respiratory tract hemorrhage. Repeated laryngoendoscopy revealed a bleeding ulceration in the pharynx that required hemostasis with electric cautery under general anesthesia. No bleeding recurred thereafter. Histopathologically, the pharynx exhibited nonspecific inflammatory cell infiltration in the muscle tissue. This rare manifestation may be considered in cases of DM with unexplainable airway bleeding.
- Drug-Associated Dermatomyositis Following Ipilimumab Therapy: A Novel Immune-Mediated Adverse Event Associated With Cytotoxic T-Lymphocyte Antigen 4 Blockade. [JOURNAL ARTICLE]
- JAMA Dermatol 2014 Oct 15.
Ipilimumab, a human monoclonal antibody targeted against cytotoxic T-lymphocyte antigen 4, has shown promise in the treatment of metastatic melanoma. However, given its mechanism of action, immune-related adverse effects have been reported with this therapy. Despite increasing reports of immune-related adverse effects related to ipilimumab therapy, dermatomyositis associated with this agent has not previously been reported.We describe a woman undergoing treatment with ipilimumab for metastatic melanoma who developed classic cutaneous findings of dermatomyositis along with proximal muscle weakness and elevated muscle enzymes.This case adds to the expanding literature regarding immune-related adverse events associated with ipilimumab. To our knowledge, drug-induced dermatomyositis from ipilimumab has not previously been reported. Physicians should be aware of these potential immune-related adverse events and consider drug-associated dermatomyositis in the differential diagnosis in patients receiving ipilimumab who present with a cutaneous eruption or muscle weakness.
- Nailfold capillaroscopic changes in dermatomyositis and polymyositis. [JOURNAL ARTICLE]
- Clin Rheumatol 2014 Oct 17.
Inflammatory myopathies (IM) are a group of muscle diseases occurring both in children and adults. Nailfold videocapillaroscopy (NVC) alterations are described in IM, but available data are discordant, including differences between polymyositis (PM) and dermatomyositis (DM). The aim of this study was to describe the capillaroscopic differences between PM and DM patients and possible correlation with clinical and serological features. We analyzed 52 unselected patients with IM in a cross-sectional study in a 6-month period. NVC findings of 29 DM and 23 PM patients were compared with those of 52 patients with primary Raynaud's phenomenon. Tortuosities, capillary loss, enlarged and giant capillaries, microhemorrhages, and ramified capillaries were scored by a semiquantitative rating; disorganization of the vascular array, avascular areas, and scleroderma pattern were scored as presence/absence. Sex, mean age, and mean disease duration were similar in both groups. Disorganization of the vascular array, enlarged and giant capillaries, capillary loss, and scleroderma-like pattern were observed almost only in IM patients. Significant differences were observed between PM and DM with higher frequency and mean score of NVC changes in DM. In DM patients with disease duration ≤6 months (14/29 patients), capillary density was significantly reduced (P = 0.039) and giant capillaries more frequent (P = 0.027), compared with patients with longer disease duration, while a scleroderma pattern tended to be more frequent in patients with a disease duration of less than 6 months. On the contrary, no differences were observed for ramified capillaries with regard to disease duration. Capillaroscopic alterations are identified only in DM patients as expression of diffuse microangiopathy; surprisingly, more severe changes were associated with shorter disease duration, while persistence of ramified capillaries with long-standing disease.
- A crucial role of anti-RPLP0 and anti-galectin-3 antibodies in the development of skin lesions in systemic lupus erythematosus. [JOURNAL ARTICLE]
- Arthritis Rheumatol 2014 Oct 10.
Objective: The specific autoantibodies and antigens which mediate systemic lupus erythematosus (SLE)-related organ injuries remain largely unknown. This study aimed to investigate the antibody-mediated immune response that leads to SLE skin lesions. Methods: The study involved 85 SLE patients with specific lupus skin lesions and 31 without skin lesions. The reactivity of serum antibody to skin antigens was determined by immunoblot using human foreskin as the substrate. Skin antigens were identified using mass spectrometry. Serum antibody was isolated by affinity purification and was injected intracutaneously into mouse skin to determine pathogenicity. The antibody serum levels were monitored by enzyme-linked immunoabsorbent assay. Results: We determined that 78% of patients with skin lesions presented serum antibodies reactive to 35kD and/or 25kD skin antigens, which was significantly higher than patients without skin lesions (p < 0.0001), suggesting a correlation between immune response and skin lesions. Acidic ribosomal P protein 0 (RPLP0) and galectin-3 were two target antigens identified from 35kD and 25kD proteins, respectively. Purified serum anti-RPLP0 and anti-galectin-3 antibodies induced lupus-like histological changes after intracutaneous injection. Anti-RPLP0 and anti-galectin-3 antibodies were significantly higher in SLE patients than in healthy controls and decreased with skin recovery. Anti-galectin-3 antibody was also significantly higher in SLE patients than in patients with dermatomyositis and systemic scleroderma, and strongly related to lupus cutaneous vasculitis. Additionally, the two antibodies were positively correlated with leucopenia and C3 deficiency, and anti-RPLP0 antibody was also positively correlated with arthritis and SLE disease activity. Conclusion: The immune response mediated by serum anti-RPLP0 and anti-galectin-3 antibodies play a key role in the pathogenesis of SLE skin lesions. These findings provide new insights into the mechanism of SLE-related organ disorders. © 2014 American College of Rheumatology.
- The efficacy of calcineurin inhibitors for the treatment of interstitial lung disease associated with polymyositis/dermatomyositis. [REVIEW]
- Lupus 2014 Oct 8.
Interstitial lung disease (ILD) in patients with polymyositis (PM) and dermatomyositis (DM) is often resistant to treatment and life threatening, being recognized as one of the severest complication in these autoimmune disorders. Patients with clinically amyopathic dermatomyositis (CADM) or those with anti-CADM140/MDA5 antibody are especially prone to develop rapidly progressive interstitial pneumonia. We retrospectively analyzed 46 patients with PM/DM admitted to our hospital and identified DM, rapidly progressive disease, honeycomb lung, CADM and extensive ILD as risk factors for recurrence or death. In the presence of two or more risk factors, the sensitivity and specificity for the prediction of death or relapse were 81.3% and 76.7%, respectively. Calcineurin inhibitors have been widely used as induction and maintenance therapy for PM/DM-associated ILD. Recently we reported the benefit of tacrolimus on the disease-free survival and event-free survival of the patients with PM/DM-associated ILD. Among those patients treated with tacrolimus, poor prognostic factors for death, recurrence or severe adverse event were identified as acute progression of the disease, honeycomb lung, forced vital capacity (FVC) less than 80% and having DM. The potential effectiveness of an intensive therapy protocol with triple therapy that comprises high-dose corticosteroids, calcineurin inhibitors and cyclophosphamide has been reported.
- Clinical and laboratory features of fatal rapidly progressive interstitial lung disease associated with juvenile dermatomyositis. [JOURNAL ARTICLE]
- Rheumatology (Oxford) 2014 Oct 6.
Rapidly progressive interstitial lung disease (RP-ILD) is a rare but potentially fatal complication of JDM. The aim of this study was to establish markers for the prediction and early diagnosis of RP-ILD associated with JDM.The clinical records of 54 patients with JDM were retrospectively reviewed: 10 had RP-ILD (7 died, 3 survived), 19 had chronic ILD and 24 were without ILD. Routine tests included a high-resolution CT (HRCT) scan of the chest and measurement of serum levels of creatine phosphokinase, ferritin and Krebs von den Lungen-6 (KL-6). Anti-melanoma differentiation-associated gene 5 (MDA5) antibodies and IL-18 levels were measured by ELISA.No differences were found in the ratio of juvenile clinically amyopathic DM between the three groups. Initial chest HRCT scan findings were variable and could not distinguish between RP-ILD and chronic ILD. Anti-MDA5 antibodies were positive in all 8 patients with RP-ILD and 10 of 14 with chronic ILD, but none of the patients without ILD. Serum levels of anti-MDA5 antibody, ferritin, KL-6 and IL-18 were significantly higher in the RP-ILD group than in the chronic ILD and non-ILD groups. Serum levels of IL-18 positively correlated with serum KL-6 (R = 0.66, P < 0.001).High serum levels of IL-18, KL-6, ferritin and anti-MDA5 antibodies (e.g. >200 units by ELISA) are associated with RP-ILD. These can be used as an indication for early intensive treatment. Both alveolar macrophages and autoimmunity to MDA5 are possibly involved in the development of RP-ILD associated with JDM.
- Clinical and pathologic differences in interstitial lung disease based on antisynthetase antibody type. [JOURNAL ARTICLE]
- Respir Med 2014 Sep 18.
Interstitial lung disease (ILD) is a common extramuscular manifestation of the idiopathic inflammatory myopathies (IIMs), dermatomyositis (DM) and polymyositis (PM). Patients with antisynthetase antibodies (ASA) demonstrate some or all of the features of the antisynthetase syndrome including IIM and ILD. It has been hypothesized that the clinical expression of antisynthetase syndrome varies between specific ASAs.We sought to determine whether the myositis-associated ILD (MA-ILD) phenotype differs based on the presence of ASAs and by ASA subtype.A cross-sectional and longitudinal analysis of consecutive patients enrolled at the Johns Hopkins Myositis Center with ILD in the setting of clinically diagnosed autoimmune myositis was conducted.Seventy-seven subjects were included; 36 were ASA negative, 28 were anti-Jo1 positive, and 13 were non-Jo1 ASA positive (5 anti-PL-12, 4 anti-PL-7, 2 anti-EJ, and 2 anti-OJ). Non-Jo1 ASA positive participants were more likely to be African-American than Caucasian as compared to both the anti-Jo1 positive (p = 0.01) and ASA negative groups (p < 0.01). ASA negative participants had better mean forced vital capacity percent predicted (FVC%) and total computed tomography scores over time compared to those with anti-Jo1 after controlling for potential confounders.ASA status was significantly different by race. Those with anti-Jo1 antibodies had worse lung function and CT scores over time compared to those without detectable antisynthetase antibodies. Further prospective study in a larger cohort is needed to determine whether these apparent antibody-specific differences in demographics and manifestations of disease translate into meaningful disparities in clinical outcomes.
- Leishmaniasis and autoimmune diseases in pediatric age. [REVIEW]
- Cell Immunol 2014 Aug 27; 292(1-2):9-13.
Leishmaniasis is a group of diseases caused by the protozoa Leishmania, endemic in the Mediterranean countries. Clinical manifestations can be divided into three different forms: cutaneous leishmaniasis, mucosal leishmaniasis and the visceral leishmaniasis, the most severe form which is potentially lethal if untreated. Immunology and pathogenesis are complex: many different aspects of immune response, resistance and susceptibility to Leishmania have been studied but many others remain to be clarified. The gold standard in diagnosis of visceral Leishmaniasis is the presence of amastigotes in bone marrow or tissue sections. Patients can be initially misdiagnosed as having an autoimmune disease because it may mimic diseases like systemic lupus erythematosus, autoimmune hepatitis, dermatomyositis or others disorders. As in pediatric age the risk of life-threatening complications is very high, leishmaniasis, must be kept in mind to the clinician, in order to avoid wrong diagnosis and an inappropriate immunosuppressive therapy.
- Acute acalculous cholecystitis in a patient with juvenile dermatomyositis. [Journal Article]
- BMJ Case Rep 2014.
Juvenile dermatomyositis (JDM) is a rare autoimmune disease, characterised by a systemic capillary vasculopathy that typically affects skin and muscle. Gastrointestinal involvement is relatively rare. We report the case of an 11-year-old girl admitted for investigation of skin rash, progressive symmetric proximal muscle weakness, dysphagia and weight loss. The diagnosis of JDM was confirmed and during hospitalisation the patient developed abrupt and intense right hypocondrium pain associated with nausea and vomiting. Abdominal ultrasound revealed a thick gallbladder wall (8 mm) with pericholecystic fluid and no evidence of gallstones. An acute acalculous cholecystitis was assumed and the patient was started on intravenous fluids, prednisolone and analgaesic therapy. Clinical resolution was verified after 48 h. We hypothesised that the vasculitic process of JDM could have been the basis for this complication as described in other autoimmune diseases.