Successful complementary therapy with traditional Chinese medicine in a patient with Qi and Jin deficiency symptoms from adult-onset Still's disease: A case report.

BACKGROUND: Adult-onset Still's disease (AOSD) is a rare yet well-documented polygenic and systemic autoinflammatory disease characterized by recurrent spiking fever, transient skin rash, arthralgia, and sore throat. Traditional Chinese medicine (TCM) holds a significant role in complementary and alternative medicine. This study presents a unique case of a female AOSD patient with concurrent Qi and fluid deficiency syndrome who received combined treatment with formulated Zhu Ye Shi Gao Decoction (ZYSGD). CASE PRESENTATION: In this case, a 28-year-old female patient presented with a 15-day history of fever and skin rash accompanied by sore throat, fatigue, myalgia, and arthralgia. Additionally, leucocytosis, aminotransferase abnormalities, and elevated inflammatory factor levels were observed. Infectious diseases, solid tumors, and hematological disorders were all ruled out. Anti-infective treatments proved ineffective, leading to the final diagnosis of AOSD. Glucocorticoid therapy provided only partial relief. Consequently, formulated ZYSGD and hepatoprotective drugs were added to the glucocorticoid treatment. Subsequently, the patient's symptoms and inflammatory biomarkers showed improvement. After discharge, the patient's condition remained stable while continuing the formulated ZYSGD in combination with 4 mg of Medrolol (qd) during a 10-month follow-up period. CONCLUSION: This case report suggests that formulated ZYSGD could be a viable option for complementary and alternative therapy for late-stage AOSD, especially in cases involving both Qi and body fluid imbalances.

Current and emerging biological therapy in adult-onset Still's disease.

Adult-onset Still's disease (AOSD) is a rare, but characteristic non-familial, multi-genic systemic auto-inflammatory disorder, characterized by high spiking fever, salmon-like evanescent skin rash, polyarthritis, sore throat, hyperferritinemia and leucocytosis. The hallmark of AOSD is a cytokine storm triggered by dysregulation of inflammation. Nowadays, with advances in anti-cytokine biologic agents, the treatment of AOSD is no longer limited to NSAIDs, glucocorticoids or conventional synthetic DMARDs. In this review, we focussed on the roles of these cytokines in the pathogenesis of AOSD and summarized the current and emerging biological therapy.

Adult-onset Still's disease successfully treated with Chinese herbal medicine: A case report with 15-month follow-up.

Adult-onset Still's disease (AOSD) is a rare but clinically well-known, polygenic, and systemic autoinflammatory disease, which is characterized by spiking fever, evanescent skin rash, arthralgia, and sore throat. The application of non-steroidal anti-inflammatory drugs and glucocorticoids, which are first-line therapies of AOSD, is limited due to their side effects such as liver injury or disorder of blood glucose. Therefore, patients who suffer from systemic diseases in China prefer to seek help from Chinese herbal medicine (CHM), which is an important part of complementary and alternative medicine. In this case, we report a 28-year-old male badminton coach presenting with a 15-day history of fever and skin rash, accompanied by sore throat, fatigue, myalgia and chills. Additionally, hepatosplenomegaly, multiple lymphadenopathies, aminotransferase abnormality, and elevated inflammatory factor levels were observed during hospitalization. Infectious diseases, solid tumors, hematological diseases, and common autoimmune diseases were excluded. Not benefitting from antibiotic therapy, the patient was finally diagnosed with AOSD, after a careful examination, then showed rapid remission after a six-week treatment with CHM granules based on Xiaochaihu Decoction and Yinqiao Powder. After stopping the treatment, there was no relapse within a 15-month follow-up period. To the best of our knowledge, this is the first well-documented case of this successful treatment. The present case report suggests that CHM is a reliable choice for complementary and alternative therapy for AOSD, but confirming the utility of CHM for AOSD requires further support from prospective studies.

Complex presentation of adult-onset Still's disease.

A 61-year-old woman was admitted with feeling generally unwell with influenza-like symptoms, for almost a month. This was followed by dyspnoea, productive cough and fever of >40°C. She was started on oral antibiotics in community, but due to rising inflammatory markers, she was referred for admission to our hospital. Chest X-ray showed left basal pneumonia and SE was started on intravenous antibiotics according to microbiologist's advice. During admission she developed deranged liver functions with right upper quadrant tenderness, pleural and pericardial effusions. This was followed by multiple joint aches, mouth ulcers and a rash on her chest. Finally, after several days and clinical dilemma, she was diagnosed with adult-onset Still's disease by the rheumatologist and was started on prednisolone, to which she showed marked improvement, and was later maintained on methotrexate and hydrotherapy. She was in remission during her follow-up in the rheumatology clinic.

[ASIA syndrome: breast implant and Still's disease]. / Síndrome ASIA: Prótesis mamarias y enfermedad de Still.

Connective tissue diseases associated with silicone breast implants have been widely discussed. In the last decade, siliconosis has been included in the autoimmune/inflammatory syndrome induced by adjuvants (ASIA) next to Gulf War syndrome, macrophage myofascitis and postvaccination phenomena. The ASIA syndrome may appear as lupus, rheumatoid arthritis, or more rarely, as adult Still's disease. We discuss the case of a patient with prolonged fever and clinical criteria for ASIA and Still's disease. The prostheses were resected and pathology showed absence of breast implant associated anaplastic lymphoma ALK (-). Physicians should be alert to these new entities linked to silicone breast implants.

Síndrome ASIA: prótesis mamarias y enfermedad de Still / ASIA syndrome: breast implant and Still's disease

Las enfermedades del tejido conectivo vinculadas a implantes mamarios de siliconas han sido tema de discusión. En la última década, la siliconosis ha sido incluida dentro del síndrome autoinmune/inflamatorio inducido por adyuvante (ASIA) junto al síndrome de la guerra del Golfo, síndrome de miofascitis macrofágica y fenómenos post vacunales. El ASIA puede manifestarse como lupus, artritis reumatoidea, o más raramente como enfermedad de Still del adulto. Presentamos el caso de una paciente con fiebre prolongada y criterios clínicos compatibles con ASIA y enfermedad de Still. Se resecaron las prótesis y la anatomía patológica descartó linfoma anaplásico ALK (-) vinculado a prótesis. Los médicos debemos estar alertas ante la aparición de estas nuevas entidades asociadas a los implantes mamarios de siliconas.

Connective tissue diseases associated with silicone breast implants have been widely discussed. In the last decade, siliconosis has been included in the autoimmune/inflammatory syndrome induced by adjuvants (ASIA) next to Gulf War syndrome, macrophage myofascitis and postvaccination phenomena. The ASIA syndrome may appear as lupus, rheumatoid arthritis, or more rarely, as adult Still's disease. We discuss the case of a patient with prolonged fever and clinical criteria for ASIA and Still's disease. The prostheses were resected and pathology showed absence of breast implant associated anaplastic lymphoma ALK (-). Physicians should be alert to these new entities linked to silicone breast implants.

Unusual onset of adult still's disease due to a systemic reaction to artificial breast implants.

In this manuscript, we report an exceptional observation study of a young woman suffering from an autoimmune syndrome induced by adjuvants (ASIA). Until today only seven cases of adult-onset Still's disease (AOSD) induced by breast implants have been published. This may be due to the fact that this illness itself is very rare; however, the reason might also be that the community is not sensitized to the case-specific symptoms. Within this article, we show for the first time highly detailed diagnostic test procedures such as PET-CT scans and specific histological staining of the breast tissue, displaying proinflammatory macrophages that are a well-known activator and booster of autoimmune diseases. We hope to give new insights into the clinical picture and pathogenesis of AOSD in order to improve the challenging diagnosis.

About the complexity of adult onset Still's disease and advances still required for its management.

Adult onset Still's disease (AOSD) is a rare inflammatory disorder that remains poorly understood. Its pathophysiology is yet to be completely elucidated, but is known to consist mainly on a cytokine cascade, responsible for the systemic manifestations. AOSD diagnosis is usually difficult and delayed, with physicians having to rule out several other conditions, including cancer or infectious diseases. Prognosis is heterogeneous and difficult to establish, ranging from benign outcome to chronic destructive polyarthritis and/or life-threatening events. In addition, treatment remains to be codified, especially considering the development of new drugs. In this commentary, we attempt to elucidate the complexity of AOSD and to highlight the need of working on prognostic tools for this disorder. We also discuss the numerous advances that would be useful for patients in the daily management of this disease.Please see related article: http://bmcmedicine.biomedcentral.com/articles/ 10.1186/s12916-016-0738-8 .

Usefulness of tacrolimus for refractory adult-onset still's disease: Report of six cases.

Six patients with refractory adult-onset Still's disease (AOSD) were treated with tacrolimus (TAC). Patient 1 was pregnant, for whom high-dose corticosteroid (CS) monotherapy did not achieve clinical remission, whereas TAC concomitant with CS was successful, and her baby had no apparent abnormalities. Patient 2 had hemophagocytic syndrome (HPS), for whom high-dose CS monotherapy did not achieve clinical remission, whereas TAC improved HPS, and a complete clinical remission was achieved with concomitant administration of TAC and methotrexate (MTX) with CS. Cases 3-5 could not have reduced CS doses due to repeated recurrences and other disease-modifying antirheumatic drugs, including MTX, Cyclosporine A, and tumor necrosis factor alpha inhibitors, did not control disease activity. TAC administration allowed for reduced CS doses. Case 6 experienced adverse effects, and TAC was discontinued due to elevated serum creatinine and potassium levels. TAC was useful for five of six patients, which suggests it as an option for refractory AOSD.

When uncommon and common coalesce: adult onset Still's disease associated with breast augmentation as part of autoimmune syndrome induced by adjuvants (ASIA).

Adult onset Still's disease (AOSD) is an uncommon, multisystemic, auto-inflammatory disorder, while breast augmentation is a very common cosmetic procedure. We describe a case in which these two coalesce, AOSD, manifested with pleuritis and pericarditis, developed after breast mammoplasty. The pathogenetic, missing link, behind the development of AOSD following mammoplasty, is thought to be the autoimmune (auto-inflammatory) syndrome induced by adjuvants (ASIA). We reviewed other cases of AOSD associated with breast mammoplasty published to date and the literature regarding AOSD and ASIA syndrome. The review is followed by a short debate of whether silicone implants should be explanted in similar, future cases.

A new alternative therapy in dermatology: tocilizumab.

Tocilizumab (TCZ) is a recombinant-humanized anti-human interleukin 6 receptor monoclonal antibody of the immunoglobulin (Ig) IgG1 subclass with a H2L2 polypeptide structure. Even if it was approved by the US Food and Drug Administration for the treatment of rheumatoid arthritis, systemic juvenile idiopathic arthritis and polyarticular juvenile idiopathic arthritis, satisfying results have also been reported with TCZ in various refractory dermatological diseases such as psoriasis, psoriatic arthritis, Behçet's disease, systemic lupus erythematosus, systemic sclerosis, relapsing polychondritis, vasculitis and atopic dermatitis. TCZ treatment in dermatology and adverse effects of the drug were reviewed here after the pharmacological properties, mechanism of action, dosage and administration of the drug were summarized. We estimate that by the help of newly well-designed studies with wider spectrum of subjects to comprehensively investigate the efficacy and safety will be able to contribute to the clinical management of the diseases especially refractory to the other treatments. Therefore, during the next decade, TCZ will be promising drugs in the treatment of refractory dermatological diseases.

Adult onset Still's disease (AOSD) in the era of biologic therapies: dichotomous view for cytokine and clinical expressions.

Adult onset Still's disease (AOSD) is a rare inflammatory disorder characterized by hectic spiking fever, evanescent rash and joint involvement. Prognosis is highly variable upon disease course and specific involvements, ranging from benign and limited outcome to chronic destructive polyarthritis and/or life-threatening events in case of visceral complications or reactive hemophagocytic lymphohistiocytosis (RHL). AOSD remains a debatable entity at the frontiers of autoimmune diseases and autoinflammatory disorders. The pivotal role of macrophage cell activation leading to a typical Th1 cytokine storm is now well established in AOSD, and confirmed by the benefits using treatments targeting TNF-α, IL-1ß or IL-6 in refractory patients. However, it remains difficult to determine predictive factors of outcome and to draw guidelines for patient management. Herein, reviewing literature and relying on our experience in a series of 8 refractory AOSD patients, we question nosology and postulate that different cytokine patterns could underlie contrasting clinical expressions, as well as responses to targeted therapies. We therefore propose to dichotomize AOSD according to its clinical presentation. On the one hand, 'systemic AOSD' patients, exhibiting the highest inflammation process driven by excessive IL-18, IL-1ß and IL-6 production, would be at risk of life-threatening complications (such as multivisceral involvements and RHL), and would preferentially respond to IL-1ß and IL-6 antagonists. On the other hand, 'rheumatic AOSD' patients, exhibiting pre-eminence of joint involvement driven by IL-8 and IFN-γ production, would be at risk of articular destructions, and would preferentially respond to TNF-α blockers.

Enfermedad de Still del adulto: tratamiento con fármacos biológicos en casos resistentes / Adult-onset Still disease: treatment with biologic agents in refractory cases

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Respuesta: Enfermedad de Still del adulto: tratamiento con fármacos biológicos en casos resistentes / Reply: Adult-onset Still disease: treatment with biologic agents in refractory cases

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Still's disease, lupus-like syndrome, and silicone breast implants. A case of 'ASIA' (Shoenfeld's syndrome).

In recent years, four conditions, siliconosis, Gulf War syndrome (GWS), macrophagic myofasciitis syndrome (MMF) and post-vaccination phenomena, were linked to a previous exposure to an adjuvant, suggesting a common denominator, and it has been proposed to incorporate comparable conditions under a common syndrome entitled Autoimmune/inflammatory Syndrome Induced by Adjuvants (ASIA). We report a case of a female who at the age of 11 years was diagnosed with Still's disease. At the age of 22 she underwent silicone breast implants and presented with a transient lupus-like syndrome. Then, at 25 years old she had a severe activation of Still's disease in association with rupture of silicone breast implants. When the prostheses were removed, the clinical picture improved. This case fulfills the criteria for ASIA and complements seven previous reports of Still's disease in association with silicone breast implants.

Eficacia de tocilizumab en la enfermedad de Still del adulto / Tocilizumab effectiveness in adult Still's disease

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Rheumatoid arthritis.

Rheumatoid arthritis is characterised by persistent synovitis, systemic inflammation, and autoantibodies (particularly to rheumatoid factor and citrullinated peptide). 50% of the risk for development of rheumatoid arthritis is attributable to genetic factors. Smoking is the main environmental risk. In industrialised countries, rheumatoid arthritis affects 0·5-1·0% of adults, with 5-50 per 100 000 new cases annually. The disorder is most typical in women and elderly people. Uncontrolled active rheumatoid arthritis causes joint damage, disability, decreased quality of life, and cardiovascular and other comorbidities. Disease-modifying antirheumatic drugs (DMARDs), the key therapeutic agents, reduce synovitis and systemic inflammation and improve function. The leading DMARD is methotrexate, which can be combined with other drugs of this type. Biological agents are used when arthritis is uncontrolled or toxic effects arise with DMARDs. Tumour necrosis factor inhibitors were the first biological agents, followed by abatacept, rituximab, and tocilizumab. Infections and high costs restrict prescription of biological agents. Long-term remission induced by intensive, short-term treatment selected by biomarker profiles is the ultimate goal.

Terapia biológica con anticuerpos anti factor de necrosis tumoral, ¿ampliando el espectro de sus indicaciones? / Biological therapy with anti-TNF antibodies. Extending the spectrum of its indications?

El factor de necrosis tumoral alfa (TNFα) es un mediador fundamental en la respuesta inflamatoria de muchas enfermedades. Los anticuerpos anti-TNFalfa (infliximab, etanercept, adalimumab) bloquean su acción, impidiendo la respuesta inflamatoria y el daño que produce. En este trabajo revisamos el tratamiento de algunas enfermedades en las que se está experimentando con estos fármacos, como son: colitis ulcerosa, sarcoidosis, enfermedad injerto contra huésped, enfermedad de Still del adulto, algunas vasculitis sistémicas, lupus eritematoso sistémico y enfermedad de Behçet (AU)

Tumor necrosis factor (TNFα) is a main mediator in the inflammatory answer of many diseases. The anti-TNFalpha antibodies (infliximab, etanercept, adalimumab) block their action, preventing the inflammatory answer and the damage it produces. In this paper, we review the treatment of some diseases in which these drugs are experimentally used. These are: ulcerative colitis, sarcoidosis, graft-versus-host disease, adult Still's disease, some systemic vasculitis, systemic lupus erythematosus and Behçet disease (AU)

Enfermedad de Still del adulto / Adult onset Still’s disease

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Adult-onset Still's Disease with Dysphagia: Case report

We experienced a case of adult-onset Still's disease with dysphagia of soft tissue origin. A 38-year-old woman was admitted for intermittent high spiking fever, diffuse pain and swelling on anterior neck with dysphagia. Physical examination revealed a thin woman with trismus, board-like hardness of anterior neck, hepatomegaly, and erythematous evanescent rash on leg. Neck CT displayed bilateral cervical lymphadenopathy with soft tissue swelling, and tonsilitis. Chest and abdomen CT showed the inflammatory changes of multiple organs. Laboratory evaluation revealed neutrophilic leukocytosis, slightly increased alanine transaminase, negative antinuclear antibody and rheumatoid factor, and increased inflammatory markers. Skin, liver, colon, and pleural biopsy demonstrated the chronic inflammation. Videofluoroscopic swallowing study revealed laryngeal penetration with large amount residue in vallecular space and pyriform sinus, and incomplete cricopharyngeal relaxation. Gradual improvement in anterior neck pain and pulmonary edema and the decrease of effortful and multiple swallowing were observed with high dose steroid therapy.