[Eosinophilic fasciitis: From pathophysiology to therapeutics]. / Fasciite à éosinophiles : actualités physiopathologiques et nouvelles voies thérapeutiques.

Eosinophilic fasciitis (EF) is a rare connective tissue disorder characterized by painful edema and induration of the limbs and trunk, likely associated with hypereosinophilia and hypergammaglobulinemia. EF causes arthralgia and range of motion limitation, leading to significant functional impairment and poor quality of life. Since its description by Shulman in 1974, over 300 cases have been reported. We present here a review of the latest diagnostic, pathophysiological and therapeutic developments in this disease. Magnetic resonance imaging appears useful to guide diagnosis and biopsy. Diagnosis is based on a deep skin biopsy involving the fascia, which will reveal edema, sclerofibrosis of the muscular fascia and subcutaneous tissue, and an inflammatory infiltrate sometimes composed of eosinophilic polynuclear cells. EF may occur in patients treated with immune checkpoint inhibitors and the diagnosis should be raised in case of cutaneous sclerosis in these patients. The pathophysiology of the disease remains poorly understood, and its management lacks randomized, controlled, blinded trials. First-line treatment consists in oral corticosteroid therapy, sometimes combined with an immunosuppressant, mainly methotrexate. A better understanding of the pathophysiology has opened new therapeutic perspectives and clarified the role of targeted therapies in the management of EF, such as interleukin-6 inhibitors, whose efficacy has been reported in several cases.

[Enteroviral infections in adults treated with rituximab: A new case of chronic meningitis and myofasciitis and literature review]. / Infections à entérovirus de l'adulte traité par rituximab : un nouveau cas de méningite et myofasciite chronique et revue de la littérature.

INTRODUCTION: Chronic enterovirus infections can occur in primary immunodeficiency with hypogammaglobulinemia. They usually associate meningitis and myofasciitis. Such infections have also been described in adults with rituximab-induced hypogammaglobulinemia. CASE REPORT: We report the case of a 33-year-old woman who was given rituximab for immune thrombocytopenia and developed rituximab-induced hypogammaglobulinemia (IgG 4.4g/L). One year after the last rituximab infusion, she developed lower limbs myofasciitis, followed two months later by a chronic lymphocytic meningitis. PCR in the serum and the cerebrospinal fluid at the time of the meningitis and the myofasciitis were positive to the same enterovirus (echovirus 11) while it was negative in the fascia biopsy. Under treatment with intravenous immunoglobulins, all symptoms and laboratory abnormalities improved and enterovirus PCR became negative. CONCLUSION: We report a case of chronic enterovirus infection associating meningitis and myofasciitis in an adult with rituximab-induced hypogammaglobulinemia. Outcome was favorable under treatment with intravenous immunoglobulins.

[Clinical observation of mild to moderate lumbodorsal fascitis treated with distal acupoints along meridian plus exercising combined with penetration needling on yang meridians of back].

OBJECTIVE: To compare the therapeutic effect between distal acupoints along meridian plus exercising combined with penetration needling on yang meridians of back and simple penetration needling on yang meridians of back for mild to moderate lumbodorsal fascitis. METHODS: A total of 60 patients with mild to moderate lumbodorsal fascitis were randomized into an observation group and a control group, 30 cases in each one. In the control group, penetration needling on yang meridians of back was applied at acupoints of the Governor vessel (T2-L5) and the first line of bladder meridian, penetration needling was performed from the top down along the governor vessel and the first line of bladder meridian of the lumbar back pain (from one acupoint down to another acupoint), until there was no pain. In the observation group, distal acupoints along meridian plus exercising were adopted on the base of treatment in the control group. The distal acupoints along meridian plus exercising was applied at Cuanzhu (BL 2) for 30 min, at the same time, lumbar back anteflexion, hypsokinesis and turning sides were used in combination for 10 min. And then penetration needling on yang meridians of back was performed. The treatments were given once a day, 5 consecutive treatments a week, 1 week as a course and 2 courses were required. The visual analogue scale (VAS) score and Oswestry disability index (ODI) before treatment, after treatment and 1-month in follow-up were observed in the two groups, and the clinical effects were compared. RESULTS: Compared before treatment, the VAS score and ODI were reduced after treatment in the two groups (P<0.01). The changes of the VAS score and ODI in the observation group were larger than those in the control group (P<0.01, P<0.05). In follow-up, the VAS score and ODI in the observation group were lower than those in the control group (P<0.05). The total effective rate in the observation group was 90.0% (27/30), which was superior to 83.3% (25/30) in the control group (P<0.05). CONCLUSION: Distal acupoints along meridian plus exercising combined with penetration needling on yang meridians of back have a better therapeutic effect than simple penetration needling on yang meridians of back in the treatment of mild to moderate lumbodorsal fascitis.

Negative pressure therapy with the ULTRAVAC instillation in the therapy of infected laparotomies with fascitis temporary results of a prospective randomized study.

INTRODUCTION: Negative pressure wound therapy is a healing modality utilizing continuous or intermittently applied vacuum to the wound bed. Nowadays is available a vacuum system supported by an automated instillation with volumetric control. It`s main therapeutic benefit is the dilution of the exudate, which reduces the viscosity and secures the «delicate¼ necrectomy. METHOD: Presentation of the temporary results of a prospective randomized study comparing the effectiveness of both therapeutic techniques. Entirely 41 patients were enrolled in the study from November 2016 to September 2018. The primary goal of the project is to compare the length of therapy, the number of surgical debridements and evaluation the financial costs. Secondary targets are observed changes in biological load and bacterial spectrum. RESULTS: The duration of the therapy was 2 days shorter in the experimental group compared to the control sample. However, the average number of applications was higher. Defects with the instillation system were characterized by a shorter cleaning phase (p=0.057). The secondary suture was reached in the experimental group at 84.2% and in the control group at 72.7%. The differences in these parameters were not statistically significant. Fascial disruption was observed in the trial group in 2 patients. The financial costs of the material used was significantly higher in patients with irrigation system (p.

Steroid-resistant eosinophilic fasciitis successfully treated with addition of extracorporeal photopheresis.

Eosinophilic fasciitis (EF) is an uncommon fibrosing disease of the fascia with characteristic cutaneous and hematologic manifestations. Although EF is most commonly treated with corticosteroids at the beginning, a considerable number of patients show an inadequate response and hence various therapeutic strategies have been tried, including extracorporeal photopheresis (ECP). We describe the case of a 66-year-old woman with steroid-resistant EF that improved significantly after ECP was added to her treatment regimen. To date, only six cases of this therapeutic strategy have been reported in English literature.

Eosinophilic fasciitis: From pathophysiology to treatment.

Eosinophilic fasciitis is a disease originally proposed as "diffuse fasciitis with eosinophilia" by Shulman in 1974. The patients with this disease often have history of strenuous exercise or labor a few days to 1-2 weeks before the onset. The chief symptoms are symmetrical, full-circumference swelling and plate-like hardness of the distal limbs. This is accompanied by redness and pain in the early stages, with many cases exhibiting systemic symptoms such as fever or generalized fatigue. The lesions have been observed extending to the proximal limbs, though never on the face or fingers. En bloc biopsies from the skin to the fascia show marked fascial thickening and inflammatory cell infiltration by the lymphocytes and plasma cells. Eosinophilic infiltration is useful for the diagnosis but is only seen in the early stages of the disease. Recently, "Diagnostic criteria, severity classification, and clinical guidelines for eosinophilic fasciitis" were published. This review article discusses about eosinophilic faciitis in detail, from its pathophysiology to the treatment.

[Paraneoplastic syndromes in rheumatology]. / Paraneoplastische Syndrome in der Rheumatologie.

Malignancies can present as inflammatory rheumatic diseases. These rheumatic paraneoplastic syndromes are rare, but characteristic in their pattern. This article focuses on epidemiology, clinical and diagnostic features as well as treatment of paraneoplasic rheumatic diseases such as paraneoplastic arthritides, vasculitides, myositis and hypertrophic osteoarthropathy. The knowledge of their clinical patterns is of utmost importance for early diagnosis and prognosis of yet undiagnosed malignancies.

Diagnostic criteria, severity classification and guidelines of eosinophilic fasciitis.

We established diagnostic criteria and severity classification of eosinophilic fasciitis because there is no established diagnostic criteria or widely accepted severity classification of the disease. Also, there has been no clinical guideline for eosinophilic fasciitis, so we established its clinical guideline ahead of all over the world. In particular, the clinical guideline was established by clinical questions based on evidence-based medicine according to the New Minds Clinical Practice Guideline Creation Manual (version 1.0). We aimed to make the guideline easy to use and reliable based on the newest evidence, and to present guidance as specific as possible for various clinical problems in treatment of eosinophilic fasciitis.

Long-term outcome of eosinophilic fasciitis: A cross-sectional evaluation of 35 patients.

BACKGROUND: Eosinophilic fasciitis (EF) is a connective tissue disease with an unknown long-term course. OBJECTIVE: To evaluate presence and determinants of residual disease damage in patients with EF after long-term follow-up. METHODS: Patients with biopsy-proven EF were included for this cross-sectional study. Outcome measures included the Physician's Global Assessment of Disease Activity, Physician's Global Assessment of Damage (PhysGA-D), skin pliability scores, passive range of motion, and health-related quality of Life (HRQoL) questionnaires. RESULTS: In total, 35 patients (24 of whom were female [68.6%]) with a median age of 60 years participated. All patients had detectable residual damage. Impairment of HRQoL, assessed by the Dermatology Quality of Life Index and the 36-Item Short-Form Survey, correlated to the extent of residual damage. The PhysGA-D score at participation correlated to signs of severe disease at presentation, such as increased C-reactive protein level (Spearman's rho [rs ] = 0.486, P = .006), involvement of the neck (rs = 0.528, P = .001) and trunk (rs = 0.483, P = .003), prolonged time to disease remission (rs = 0.575, P = .003), and presence of concomitant morphea (rs = 0.349, P = .040). Lastly, maximum methotrexate dose correlated negatively to PhysGA-D score at study participation (rs = -0.393, P = .022). LIMITATIONS: Sample size. CONCLUSION: All patients with EF had detectable residual damage. Impairment of HRQoL correlated to the extent of residual damage. Advanced age and signs of severe disease at presentation were associated with the severity of residual damage.

Primary retroperitoneal fasciitis; A rare cause of acute abdominal pain.

The retroperitoneal fascial planes can be affected by various clinical disorders. In most of the cases retroperitoneal involvement occurs secondary to spread of a distinct underlying etiology. Herein we report two cases of primary retroperitoneal fasciitis diagnosed with imaging findings. The diagnosis of retroperitoneal fasciitis should be made by exclusion since various and more frequently encountered disorders including acute pancreatitis, duodenitis, pyelonephritis, and appendicitis may present with similar imaging findings.

Juvenile eosinophilic fasciitis: three case reports with review of the literature.

OBJECTIVES: Eosinophilic fasciitis is an uncommon scleroderma-like disorder characterised by induration and thickening of skin and soft tissue, usually associated with peripheral eosinophilia, poorly characterised in childhood. METHODS: We report 3 paediatric cases of eosinophilic fasciitis showing unusual clinical and histopathological features with a review of the literature. RESULTS: All cases presented progressive motility impairment started from upper limbs with no skin abnormalities. All cases showed systemic inflammatory involvement and 2 patients had acute complications. Two patients developed disabling outcomes despite appropriate treatments. CONCLUSIONS: Eosinophilic fasciitis may present unusual clinical and histopathological features during childhood and requires early recognition in order to prevent acute complications and disabling outcomes.

Consenso sobre utilización de las infiltraciones en el deporte. Documento de Consenso de la Sociedad Española de Medicina del Deporte / Consensus on use of infiltration in sport. Document of Consensus of the Spanish Society of Sports Medicine

La infiltración es una opción terapéutica, utilizada para el tratamiento de diversas patologías, que consiste en la administración inyectada en localizaciones precisas de diferentes sustancias. Pueden tener un efecto analgésico y/o antiinflamatorio y curativo. Su uso es frecuente en el tratamiento de muchas lesiones de tejidos blandos como bursitis, sinovitis, fascitis plantar, esguinces, lesiones musculares, tendinopatías y lesiones condrales y deben de ir precedido del correspondiente diagnóstico. Casi todas las infiltraciones tienen efectos secundarios locales, leves y, en algunas ocasiones, sistémicos y pueden presentar algunas contraindicaciones específicas que dependen de la sustancia administrada. La mayor parte de los efectos adversos son debidos a uso inapropiado del medicamento. Los principios activos más utilizados son: Anestésicos locales que producen un alivio inmediato del dolor, como lidocaína y bupivacaína. Se pueden usar solos o en combinación con corticosteroides ejerciendo un efecto combinado analgésico inmediato del dolor local y un efecto terapéutico de mayor duración. Corticoides cuya propiedad fundamental es una acción antiinflamatoria muy potente. Los más utilizados son betametasona, metilprednisolona y triamcinolona. Ácido hialurónico, utilizado en el tratamiento de patologías articulares, especialmente la artrosis de rodilla y las condromalacias. Lubrifica las articulaciones y parece tener efectos directos sobre la función de las células sinoviales y el líquido sinovial. Escleroterapia, que es la introducción de una sustancia química en la luz de los vasos sanguíneos, provocando una obliteración y fibrosis secundaria. Está indicada fundamentalmente en las tendinopatías con proliferación vascular. Biorreguladores: Estimulan la curación al modular o activar diversas sustancias implicadas. Plasma rico en plaquetas: plasma autólogo que contiene más concentración de plaquetas que la sangre normal que segregan una gran cantidad de factores de crecimiento. Proloterapia, que consiste en la infiltración de sustancias que estimulan la regeneración y reparación de los tejidos. Otros: Antiinflamatorios no esteroideos, factores de crecimiento, células madre y terapias relacionadas

Infiltration is a therapeutic option used for the treatment of various diseases, which comprises injected administration into precise locations of different substances. They may have an analgesic and / or anti-inflammatory and healing effect. Its use is common in the treatment of many soft tissue injuries such as bursitis, synovitis, plantar fasciitis, sprains, muscle injuries, tendinopathies and chondral injuries and must be preceded by the appropriate diagnosis. Almost all local infiltrations have mild side effects and, sometimes, these may be systemic and may have some specific contraindications depending on the administered substance. Most of the adverse effects are caused by improper use of the drug. The most used active substances are: local anesthetics that produce immediate pain relief, such as lidocaine and bupivacaine. They can be used alone or in combination with corticosteroids producing an immediate analgesic combined effect on local pain and a therapeutic effect of longer duration. Corticosteroids, whose main property is a very powerful anti-inflammatory action. The most used are betamethasone, methylprednisolone and triamcinolone. Hyaluronic acid, used in the treatment of joint diseases, particularly knee osteoarthritis and chondromalacias/chondropathias . It lubricates joints and appears to have direct effects on the function of synovial cells and synovial fluid. Sclerotherapy, which is the introduction of a chemical substance in the light of the blood vessels, causing obliteration and secondary fibrosis. It is indicated mainly in tendinopathies with vascular proliferation. Biorregulators: They stimulate healing when modulate or activate various involved substances. Platelet-rich plasma: autologous plasma containing more platelet concentration that normal blood, secreting a large amount of growth factors. Prolotherapy, it consists in substances infiltration that stimulate regeneration and tissue repair. Other: Nonsteroidal anti-inflammatory drugs, growth factors, stem cells and related therapies

Eosinophilia in Rheumatologic/Vascular Disorders.

Peripheral and tissue eosinophilia can be a prominent feature of several unique rheumatologic and vascular diseases. These diseases span a wide range of clinical features, histologic findings, therapeutic approaches, and outcomes. Despite the rare nature of these entities--which makes large-scale studies challenging--knowledge has continued to grow regarding their epidemiology, pathophysiology, and management. This review compares and contrasts 5 rheumatologic and vascular conditions in which eosinophilia can be seen: eosinophilic granulomatosis with polyangiitis (Churg-Strauss), immunoglobulin G4-related disease, diffuse fasciitis with eosinophilia, eosinophilia-myalgia syndrome, and eosinophilic myositis.

Clinical, neuropathological and radiological evidence for a rare complication of rituximab therapy.

We report a rare case of myofasciitis and meningitis with deafness caused by systemic enterovirus infection in the setting of hypogammaglobulinaemia induced by rituximab. Whilst effective and generally safe, anti- CD 20 antibody therapy is increasingly recognised to result in unusual infectious complications to be considered in a treated patient presenting with neurological symptoms. These cases may pose diagnostic difficulties and can have atypical presentations. We present this rare complication of rituximab therapy, with histopathological confirmation of myofasciitis. In the older literature, enterovirus associated myofasciitis may have erroneously been termed dermatomyositis and we review the literature to demonstrate this important nosological point.

Extracorporeal photopheresis for the treatment of autoimmune diseases.

The immune system is tasked with the unique challenge of recognizing foreign pathogens and damaged cells while at the same time preserving and protecting the integrity of "self". When this process fails, severe consequences including cancer and autoimmunity are the end result. Current therapies aimed at treating autoimmune disorders result in generalized immunosuppression and place the patient at increased risk for infection and malignancy. ECP is a potential therapeutic intervention that recapitulates natural physiologic processes of tolerance induction to restore immune homeostasis. Several clinical trials suggest that ECP may be used to treat a broad spectrum of autoimmune diseases.

Imatinib mesylate for the treatment of steroid-refractory sclerotic-type cutaneous chronic graft-versus-host disease.

Sclerotic skin manifestations of chronic graft-versus-host disease (ScGVHD) lead to significant morbidity, including functional disability from joint range of motion (ROM) restriction. No superior second-line therapy has been established for steroid-refractory disease. Imatinib mesylate is a multikinase inhibitor of several signaling pathways implicated in skin fibrosis with in vitro antifibrotic activity. We performed an open-label pilot phase II trial of imatinib in children and adults with corticosteroid-refractory ScGVHD. Twenty patients were enrolled in a 6-month trial. Eight received a standard dose (adult, 400 mg daily; children, 260 mg/m(2) daily). Because of poor tolerability, 12 additional patients underwent a dose escalation regimen (adult, 100 mg daily initial dose up to 200 mg daily maximum; children, initial dose 65 mg/m(2) daily up to 130 mg/m(2) daily). Fourteen patients were assessable for primary response, improvement in joint ROM deficit, at 6 months. Primary outcome criteria for partial response was met in 5 of 14 (36%), stable disease in 7 of 14 (50%), and progressive disease in 2 of 14 (14%) patients. Eleven patients (79%), including 5 with partial response and 6 with stable disease, demonstrated a positive gain in ROM (range of 3% to 94% improvement in deficit). Of 13 patients with measurable changes at 6 months, the average improvement in ROM deficit was 24.2% (interquartile range, 15.5% to 30.5%; P = .011). This trial is registered at http://clinicaltrials.gov as NCT007020689.