DOCSLIB.ORG

Tofacitinib Treatment of Refractory Systemic Juvenile Idiopathic Arthritis Zhixiang Huang, MD,A Pui Y

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Tofacitinib Treatment of Refractory Systemic Juvenile Idiopathic Arthritis Zhixiang Huang, MD,A Pui Y Tofacitinib Treatment of Refractory Systemic Juvenile Idiopathic Arthritis Zhixiang Huang, MD,a Pui Y. Lee, MD, PhD,b Xiaoyan Yao, MD,a Shaoling Zheng, MD,a Tianwang Li, MD, PhDa Systemic juvenile idiopathic arthritis (sJIA) is an aggressive form of childhood abstract arthritis accompanied by persistent systemic inflammation. Patients with sJIA often exhibit poor response to conventional disease-modifying antirheumatic drugs, and chronic glucocorticoid use is associated with significant adverse effects. Although biologics used to target interleukin 1 and interleukin 6 are efficacious, the long-term commitment to frequent injections or infusions remains a challenge in young children. Janus-activated kinase (JAK) inhibitors block the signaling of numerous proinflammatory cytokines and are now used clinically for the treatment of rheumatoid arthritis in adults. Whether this new class of medication is effective for sJIA has not been reported. Here, we describe the case of a 13-year-old girl with recalcitrant sJIA characterized aDepartment of Rheumatology and Immunology, Guangdong by polyarticular arthritis, fever, lymphadenopathy, and serological features Second Provincial General Hospital, Guangzhou, China; and bDivision of Allergy, Immunology and Rheumatology, Boston of inflammation. She showed minimal response to nonsteroidal Children’s Hospital, Boston, Massachusetts antiinflammatory drugs, glucocorticoids, conventional disease-modifying Dr Huang supervised data collection and drafted the antirheumatic drugs, and etanercept. She also developed osteoporosis and manuscript; Dr Lee designed the study and revised vertebral compression fracture as the result of chronic glucocorticoid therapy. the manuscript; Dr Yao analyzed the data and Oral therapy with the JAK inhibitor tofacitinib was initiated, and the patient drafted part of the manuscript; Dr Zheng collected the patient’s data and reviewed the manuscript; Dr Li experienced steady improvement of both arthritis and systemic features. designed the study, assigned the treatment protocol, Complete remission was achieved after 3 months, and no evidence of disease and revised the manuscript; and all authors activity or adverse effects was seen through 6 months of follow-up. Our approved the final manuscript as submitted and agree to be accountable for all aspects of the work. experience reveals the effectiveness of JAK inhibition in a case of refractory DOI: sJIA. Tofacitinib is an intriguing oral alternative to the available biologics for https://doi.org/10.1542/peds.2018-2845 children with sJIA, and its efficacy and safety should be further assessed by Accepted for publication Dec 5, 2018 clinical trial. Address correspondence to Tianwang Li, MD, PhD, Department of Rheumatology and Immunology, Guangdong Second Provincial General Hospital, 466 Xingangzhong Rd, Guangzhou 510317, China. E-mail: [email protected] 4 Juvenile idiopathic arthritis (JIA) is 50% in Asian countries. Compared PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, a chronic rheumatic disease in children with other childhood arthritides, sJIA 1098-4275). and adolescents with a prevalence of has the highest mortality because of Copyright © 2019 by the American Academy of ∼70 per 100 000 children.1 The numerous complications of persistent Pediatrics International League of Associations for inflammation and chronic FINANCIAL DISCLOSURE: The authors have indicated Rheumatology classification criteria immunosuppression. These include they have no financial relationships relevant to this divide JIA into 7 categories.2 Systemic macrophage activation syndrome article to disclose. juvenile idiopathic arthritis (sJIA) is (MAS), infections, pulmonary FUNDING: No external funding. a unique category characterized by hypertension, and interstitial lung POTENTIAL CONFLICT OF INTEREST: The authors have variable arthritis and prominent disease. Therefore, finding effective indicated they have no potential conflicts of interest systemic features that include treatment for children with sJIA and to disclose. quotidian fever, evanescent rash, controlling the disease early is generalized lymphadenopathy, a priority in pediatric rheumatology. To cite: Huang Z, Lee PY, Yao X, et al. Tofacitinib hepatomegaly and/or splenomegaly, or Treatment of Refractory Systemic Juvenile serositis.3 sJIA accounts for 10% of JIA sJIA is traditionally treated with Idiopathic Arthritis. Pediatrics. 2019;143(5): e20182845 cases in North America and as high as nonsteroidal antiinflammatory drugs, Downloaded from www.aappublications.org/news by guest on September 26, 2021 PEDIATRICS Volume 143, number 5, May 2019:e20182845 CASE REPORT glucocorticoids, and/or conventional disease-modifying antirheumatic drugs in the prebiologics era. Unfortunately, approximately half of patients still suffer from active disease, whereas many experience the cumulative adverse effects of chronic glucocorticoid treatment.5 In recent years, biologics used to target interleukin 1 (IL-1) (anakinra, canakinumab, and rilonacept) and interleukin 6 (IL-6) (tocilizumab) have significantly improved outcomes in sJIA.6 These biologic agents, however, are not uniformly available, and frequent subcutaneous injections or intravenous infusion are also challenging for young children. Janus-activated kinase (JAK) inhibitors, such as tofacitinib and baricitinib, have revealed favorable efficacy and safety data in adults with 7 rheumatoid arthritis (RA). Because FIGURE 1 JAK signaling is critical for the signal Compression fracture of the T6 vertebral body likely secondary to osteoporosis. A, Radiograph transduction of multiple demonstration of the affected vertebral body. B, MRI (T2-weighted image with hyperintense signal in inflammatory cytokines, including IL- short-t inversion recovery) demonstration of the affected vertebral body. 6, orally available JAK inhibitors may be an effective treatment of sJIA. In With recurrence of fever and arthritis, anti–cyclic-citrullinated peptide this report, we describe the treatment she was admitted to our antibodies, and HLA antigen B27 tests of refractory sJIA by using tofacitinib. rheumatology service for initial were negative. An ultrasound evaluation in February 2017. The examination revealed axillary lymph patient exhibited poor growth, with node enlargement. A bone marrow CASE REPORT height and weight both under the first biopsy and MRI of the sacroiliac joint We present a patient who developed percentile for age (height: 130 cm; were unremarkable. The diagnostic daily fever of 40°C and arthritis at weight: 24.5 kg). On physical workup supported the diagnosis of the age of 11. The affected joints were examination, her left knee, left ankle, sJIA, and treatment was resumed by the left knee, the left ankle, and and wrists were warm, swollen, and using methylprednisolone (8 mg po both wrists. Abdominal computed tender to palpation. She reported daily), diclofenac (25 mg po twice tomography revealed multiple right hip pain with movement, and daily), and methotrexate (12.5 mg po enlarged lymph nodes in the the result of a Patrick’s test was weekly), with the addition of retroperitoneum, and an MRI positive. Laboratory investigations etanercept (25 mg subcutaneously confirmed synovitis of her left knee. revealed leukocytosis (20.2 3 109/L) weekly) to better capture her arthritis With persistence of symptoms for with neutrophil predominance and inflammation. weeks, the patient was diagnosed (75.0%), a normal platelet count with sJIA and was started on (320 3 109/L), an erythrocyte Although her fever resolved, minimal a treatment regimen that comprised sedimentation rate (ESR) of 102 improvement of arthritis was seen. In methylprednisolone (12 mg po daily), mm/hour (normal: ,15 mm/hour), addition, the patient was readmitted naproxen (250 mg po twice daily), and a C-reactive protein (CRP) level 1 month later with epigastric pain. An and methotrexate (12.5 mg po of 86.0 mg/L (normal: ,8.0 mg/L). endoscopy revealed mild chronic weekly). Fever and arthritis partially Serum creatinine levels, transaminase superficial gastritis. Surprisingly, improved on this regimen, but her levels, lipid profile, and procalcitonin a chest radiograph revealed suspicion disease promptly flared with levels were within normal limits, of a T6 vertebral compression attempts to wean her medications whereas results for rheumatoid fracture (Fig 1A). A thoracic MRI over the next 8 months. factor, antinuclear antibody, confirmed the fracture and revealed Downloaded from www.aappublications.org/news by guest on September 26, 2021 2 HUANG et al bone edema in the T6 vertebral body clinical examination and disease inflammation. The systemic features (Fig 1B). Dual radiograph activity score. are thought to be driven by absorptiometry of the L1 to L4 proinflammatory cytokines IL-1 and After 6 months of tofacitinib therapy, vertebral body and left femoral neck IL-6.12,13 A “window of opportunity” the patient was able to discontinue revealed z scores of 22.8 and 23.2, may exist in the early systemic phase corticosteroid treatment for the first respectively, suggesting osteoporosis of disease before the inflammatory time since her initial diagnosis and as the likely cause of her vertebral milieu primes the development of remained well without arthritis or compression fracture. A salcatonin autoreactive T cells, leading to fever. She did not experience any injection was given, and the dose of chronic arthritis.14 Consistent with infection or other adverse effects methylprednisolone was the importance of IL-1 and IL-6,
Load more

Recommended publications
  • 2013 Update of the 2011 American College of Rheumatology Recommendations for the Treatment of Juvenile Idiopathic Arthritis
    ARTHRITIS & RHEUMATISM Vol. 65, No. 10, October 2013, pp 2499–2512 DOI 10.1002/art.38092 © 2013, American College of Rheumatology Arthritis & Rheumatism An Official Journal of the American College of Rheumatology www.arthritisrheum.org and wileyonlinelibrary.com SPECIAL ARTICLE 2013 Update of the 2011 American College of Rheumatology Recommendations for the Treatment of Juvenile Idiopathic Arthritis Recommendations for the Medical Therapy of Children With Systemic Juvenile Idiopathic Arthritis and Tuberculosis Screening Among Children Receiving Biologic Medications Sarah Ringold,1 Pamela F. Weiss,2 Timothy Beukelman,3 Esi Morgan DeWitt,4 Norman T. Ilowite,5 Yukiko Kimura,6 Ronald M. Laxer,7 Daniel J. Lovell,4 Peter A. Nigrovic,8 Angela Byun Robinson,9 and Richard K. Vehe10 Guidelines and recommendations developed and/or endorsed by the American College of Rheumatology (ACR) are intended to provide guidance for particular patterns of practice and not to dictate the care of a particular patient. The ACR considers adherence to these guidelines and recommendations to be voluntary, with the ultimate determina- tion regarding their application to be made by the physician in light of each patient’s individual circumstances. Guidelines and recommendations are intended to promote beneficial or desirable outcomes but cannot guarantee any specific outcome. Guidelines and recommendations developed or endorsed by the ACR are subject to periodic revi- sion as warranted by the evolution of medical knowledge, technology, and practice. The American College of Rheumatology is an independent, professional, medical and scientific society which does not guarantee, warrant, or endorse any commercial product or service. INTRODUCTION tions represented the first such effort by the ACR that focused entirely on the treatment of a pediatric rheu- The American College of Rheumatology (ACR) matic disease, and included recommendations for the published treatment recommendations for juvenile idio- initial and subsequent treatment of patients with syno- pathic arthritis (JIA) in 2011 (1).
    [Show full text]
  • Differential Diagnosis of Juvenile Idiopathic Arthritis
    pISSN: 2093-940X, eISSN: 2233-4718 Journal of Rheumatic Diseases Vol. 24, No. 3, June, 2017 https://doi.org/10.4078/jrd.2017.24.3.131 Review Article Differential Diagnosis of Juvenile Idiopathic Arthritis Young Dae Kim1, Alan V Job2, Woojin Cho2,3 1Department of Pediatrics, Inje University Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Korea, 2Department of Orthopaedic Surgery, Albert Einstein College of Medicine, 3Department of Orthopaedic Surgery, Montefiore Medical Center, New York, USA Juvenile idiopathic arthritis (JIA) is a broad spectrum of disease defined by the presence of arthritis of unknown etiology, lasting more than six weeks duration, and occurring in children less than 16 years of age. JIA encompasses several disease categories, each with distinct clinical manifestations, laboratory findings, genetic backgrounds, and pathogenesis. JIA is classified into sev- en subtypes by the International League of Associations for Rheumatology: systemic, oligoarticular, polyarticular with and with- out rheumatoid factor, enthesitis-related arthritis, psoriatic arthritis, and undifferentiated arthritis. Diagnosis of the precise sub- type is an important requirement for management and research. JIA is a common chronic rheumatic disease in children and is an important cause of acute and chronic disability. Arthritis or arthritis-like symptoms may be present in many other conditions. Therefore, it is important to consider differential diagnoses for JIA that include infections, other connective tissue diseases, and malignancies. Leukemia and septic arthritis are the most important diseases that can be mistaken for JIA. The aim of this review is to provide a summary of the subtypes and differential diagnoses of JIA. (J Rheum Dis 2017;24:131-137) Key Words.
    [Show full text]
  • 200212.Full.Pdf
    Manisha Ramphul 1, Kathy Gallagher1, Kishore Warrier1, Sumit Jagani2, Jayesh Mahendra Bhatt 1 [email protected] Review Why is a paediatric respiratory specialist integral to the paediatric rheumatology clinic? Cite as: Ramphul M, Systemic connective tissue diseases (CTDs) are characterised by the presence of autoantibodies and Gallagher K, Warrier K, et al. multiorgan involvement. Although CTDs are rare in children, they are associated with pulmonary Why is a paediatric respiratory complications, which have a high morbidity and mortality rate. The exact pathophysiology remains specialist integral to the paediatric rheumatology unclear. The pleuropulmonary complications in CTD are diverse in their manifestations and are clinic? Breathe 2020; 16: often complex to diagnose and manage. 200212. The most common CTDs are discussed. These include juvenile systemic lupus erythematosus, juvenile dermatomyositis, juvenile systemic sclerosis, Sjögren’s syndrome and mixed connective tissue disease. We describe the clinical features of the pleuropulmonary complications, focusing on their screening, diagnosis and monitoring. Treatment strategies are also discussed, highlighting the factors and interventions that influence the outcome of lung disease in CTD and pulmonary complications of treatment. Early detection and prompt treatment in a multidisciplinary team setting, including respiratory and rheumatology paediatricians and radiologists, is paramount in achieving the best possible outcomes for these patients. Educational aims ●● To discuss the pulmonary manifestations in children with CTD. ●● To outline the diagnostic modalities used to diagnose and monitor pulmonary manifestations in CTD. ●● To review the various treatment strategies in CTD-related lung disease and the importance of a multidisciplinary approach to management. @ERSpublications Pleuropulmonary complications of CTD, though rare in paediatrics, can be associated with high morbidity and mortality.
    [Show full text]
  • Drug-Induced Autoimmunity Fatma Dedeoglu
    Drug-induced autoimmunity Fatma Dedeoglu Children’s Hospital Boston, Harvard Medical School, Purpose of review Boston, Massachusetts, USA This review aims to draw attention to the increased spectrum of the features of drug- Correspondence to Fatma Dedeoglu, MD, Children’s induced autoimmunity (DIA), including both clinical and autoantibody profiles in addition Hospital Boston, Harvard Medical School, 300 Longwood Avenue Fegan 6, Boston, MA 02115, USA to the potential chronicity of the syndrome. Tel: +1 617 355 6117; fax: +1 617 730 0249; Recent findings e-mail: [email protected] In recent years, not only has the number of medications causing DIA increased but the Current Opinion in Rheumatology 2009, spectrum of the features has broadened as well. With the use of newer medications, 21:547–551 especially biologics, mostly directed towards immune system manipulation, the range of signs and symptoms of DIA as well as the patterns of autoantibody profiles have widened. Rashes and visceral involvement have started to be reported more often, especially with tumor necrosis factor antagonists. In addition, autoantibodies such as antidouble-stranded DNA, which are usually seen with idiopathic systemic lupus erythematosus, are appearing in place of the antihistone antibodies, typically found in drug-induced lupus. Finally, some medications have been implicated in causing the very same entity, which they may be used to treat. It is clear that progress in the field of pharmacogenetics and pharmacogenomics will help further our understanding of these and other adverse effects of medications. Summary Even though DIA has been known for many years, the underlying mechanisms remain unclear.
    [Show full text]
  • Rheumatoid Arthritis: Early Diagnosis, Early Treatment
    Rheumatoid Arthritis: Early Diagnosis, Early Treatment Pascale Schwab, MD Arthritis and Rheumatic Diseases OHSUOregon Health and Science University 09/05/2019 Disclosures OHSU• None At the end of this talk, you should be able to: • Recognize the clinical features and differential diagnosis of early rheumatoid arthritis (RA) • List some key factors in the pathogenesis of RA • Describe the laboratory evaluation helpful in early diagnosis • Have an understanding of treatment strategies in RA • Know what you can do to help the rheumatologist in the co- OHSUmanagement of RA Case • 37 year-old woman • 7-week history of progressive polyarthralgias • Pain in wrists, hands and feet • Swelling in some joints, decreased hand function • Morning stiffness for 3 hours • Excessive fatigue OHSU• ROS otherwise non-contributory • Some relief from ibuprofen Case • Exam with swelling and tenderness at the bilateral wrists, 2nd MCPs, and 2nd PIPs, and bilateral MTP joints; no nodules • Rest of exam including skin is normal • Labs with mild normocytic anemia, mildly elevated ESR 28, normal renal and liver function tests • Radiographs of hands with some soft tissue swelling around the OHSUwrists and PIPs, otherwise normal Case • Does she have inflammatory arthritis? • Yes • History of joint swelling, early morning stiffness lasting ≥30 minutes, systemic symptoms such as fatigue, improvement of symptoms with anti-inflammatory medication • Objective evidence of joint swelling and tenderness on examination • Raised ESR or CRP, normocytic normochromic anemia; could
    [Show full text]
  • Case Report Gastrointestinal Symptom Due to Lupus Peritonitis: a Rare Form of Onset of SLE
    Int J Clin Exp Med 2014;7(12):5917-5920 www.ijcem.com /ISSN:1940-5901/IJCEM0002859 Case Report Gastrointestinal symptom due to lupus peritonitis: a rare form of onset of SLE Rongquan Liu*, Li Zhang*, Sujun Gao, Lei Chen, Lu Wang, Zhen Zhu, Wei Lu, Haihang Zhu Department of Gastroenterology, Clinical Medical College of Yangzhou University, Yangzhou 225001, Jiangsu, P.R. China. *Equal contributors. Received September 28, 2014; Accepted December 8, 2014; Epub December 15, 2014; Published December 30, 2014 Abstract: Serositis is commonly seen in systemic lupus erythematosus (SLE). Approximately 16% of patients with SLE have pleural or pericardial involvement. However, peritoneal involvement is extremely rare, and SLE with ascites as the first manifestation is an even rarer condition. This is the case report of a 19-year old male with discoid lupus who evolved with gastrointestinal symptoms as the first manifestation of the disease, characterized by significant abdominal distension and pain, asthenia, vomiting, and signs of ascites. An abdominal CT scan demonstrated asci- tes and marked edematous thickening of the bowel wall, which appeared as “target sign”, and “double-track sign”. Laboratory tests showed that his serum complement levels decreased and that he was positive for anti-nRNP/Sm antibodies, anti-Sm antibodies, anti-SS-A antibody, and anti-nuclear antibodies. The patient was treated with pred- nisone and chloroquine, with substantial improvement of his condition. Keywords: Systemic lupus erythematosus, serositis, ascites, lupus peritonitis, CT Introduction nal pain associated with nausea and vomiting for three days. Overall, he had been well until Systemic lupus erythematosus (SLE) is a chron- three days before his presentation.
    [Show full text]
  • Scleroderma Lung Disease – Other Lung Complications in Systemic
    Cur gy: ren lo t o R t e a s Almeida, Rheumatology 2012, S:1 e m a u r c e h h DOI:10.4172/2161-1149.S1-009 R Rheumatology : Current Research ISSN: 2161-1149 Review Article Open Access Scleroderma Lung Disease – Other Lung Complications in Systemic Sclerosis Maria do Socorro Teixeira Moreira Almeida Department of General Practice, Federal University of Piaui, Brazil Abstract Scleroderma or systemic sclerosis (SSc) is a clinically heterogeneous, multi-system autoimmune disorder characterized by endothelial dysfunction, dysregulation of fibroblasts resulting in excessive production of collagen and profound abnormalities of the immune system. Pulmonary involvement is common and occurs in all SSc subsets, including limited cutaneous systemic sclerosis, diffuse cutaneous systemic sclerosis and SSC sine scleroderma. Aside from the common complications of pulmonary vasculopathy and interstitial lung disease (ILD], other less frequent pulmonary complications have been reported in SSc. The emphasis of this review will be on other lung complications in systemic sclerosis. Keywords: Systemic sclerosis; Scleroderma lung disease _ Diffuse alveolar damage (DAD) Introduction _ Cryptogenic organizing pneumonia (COP) Scleroderma or systemic sclerosis (SSc) is a heterogeneous 2. Pulmonary hypertension disorder characterized by endothelial dysfunction, dysregulation of 3. Pleural involvement fibroblasts resulting in excessive production of collagen and profound abnormalities of the immune system. These changes cause progressive 4. Aspiration pneumonia fibrosis of the skin and internal organs, system failure and death [1]. 5. Alveolar hemorrhage Patients with SSc may exhibit proliferative small artery and obliterative microvascular disease, plus inflammation and fibrosis affecting the skin, 6. Small airways disease oesophagus, respiratory tract and other target organs [2].
    [Show full text]
  • Advances in the Pathogenesis and Treatment of Systemic Juvenile Idiopathic Arthritis
    Review nature publishing group Advances in the pathogenesis and treatment of systemic juvenile idiopathic arthritis Colleen K. Correll1 and Bryce A. Binstadt1 Systemic juvenile idiopathic arthritis (s-JIA) is clinically distinct s-JIA are features that are more typical of other subtypes of JIA, from other types of JIA. It is typified by extraarticular features such including psoriasis in the patient or a first-degree relative (sug- as quotidian fevers, rash, splenomegaly, lymphadenopathy, lab- gesting psoriatic JIA); human leukocyte antigen B27 positivity oratory abnormalities (including leukocytosis, thrombocytosis,­ in a male with arthritis starting after the age of 6 y (suggest- anemia, hyperferritinemia, and elevated inflammatory markers), ing enthesitis-related JIA); ankylosing spondylitis, enthesitis- and a close association with the macrophage activation syn- related arthritis, sacroiliitis with inflammatory bowel disease, drome. Recent investigations have highlighted ­dysregulation Reiter’s syndrome, or acute anterior uveitis, or a history of one of the innate immune system as the critical pathogenic driver of these disorders in a first-degree relative (suggesting enthesi- of s-JIA. Key innate immune mediators of s-JIA are the macro- tis-related JIA); or a positive immunoglobulin M (IgM) rheu- phage-derived cytokines interleukin-1 (IL-1) and IL-6. Increased matoid factor on two occasions at least 3 mo apart (suggest- understanding of the roles of IL-1 and IL-6 in the pathogenesis ing rheumatoid factor–positive polyarticular JIA; see online of s-JIA has led to major changes in therapeutic options. Until Supplementary Appendix online) (2). recently, the most commonly used medications included cor- According to studies from European nations, s-JIA accounts ticosteroids, methotrexate, and tumor necrosis factor (TNF) for ~4–9% of all cases of JIA; however, the subjects in these inhibitors, which are incompletely effective in most cases.
    [Show full text]
  • Cutaneous Manifestations of Systemic Disease
    Cutaneous Manifestations of Systemic Disease Dr. Lloyd J. Cleaver D.O. FAOCD Northeast Regional Medical Center A.T.Still University/KCOM Assistant Vice President/Professor ABOIM Board Review Disclosure I have no financial relationships to disclose I will not discuss off label use and/or investigational use in my presentation I do not have direct knowledge of AOBIM questions I have been granted approvial by the AOA to do this board review Dermatology on the AOBIM ”1-4%” of exam is Dermatology Table of Test Specifications is unavailable Review Syllabus for Internal Medicine Large amount of information Cutaneous Multisystem Cutaneous Connective Tissue Conditions Connective Tissue Diease Discoid Lupus Erythematosus Subacute Cutaneous LE Systemic Lupus Erythematosus Scleroderma CREST Syndrome Dermatomyositis Lupus Erythematosus Spectrum from cutaneous to severe systemic involvement Discoid LE (DLE) / Chronic Cutaneous Subacute Cutaneous LE (SCLE) Systemic LE (SLE) Cutaneous findings common in all forms Related to autoimmunity Discoid LE (Chronic Cutaneous LE) Primarily cutaneous Scaly, erythematous, atrophic plaques with sharp margins, telangiectasias and follicular plugging Possible elevated ESR, anemia or leukopenia Progression to SLE only 1-2% Heals with scarring, atrophy and dyspigmentation 5% ANA positive Discoid LE (Chronic Cutaneous LE) Scaly, atrophic plaques with defined margins Discoid LE (Chronic Cutaneous LE) Scaly, erythematous plaques with scarring, atrophy, dyspigmentation DISCOID LUPUS Subacute Cutaneous
    [Show full text]
  • Chapter 23 of Systemic Sclerosis
    Systemic Sclerosis 2nd Edition by Philip J. Clements and Daniel E. Furst Chapter 23: Introduction Facing an illness like scleroderma is often seen as a crisis, yet the overall impact of the disease can be positively influenced by the input, education and compassion provided by the patient’s medical team. Knowledge about their illness empowers patients to become advocates for their health and informed partners in treatment. The manner in which the patient is approached and the style of subsequent interactions are critical components of patient care. Although scleroderma is often mild in its expression, the patient must deal with the complex concept of living with a rare, chronic disease that is incompletely understood by the medical profession. Newly diagnosed patients frequently state: “My physician says I have a serious, life-threatening disease that he does not see very often. He also says that there is no treatment and does not know what else to do for me.” Obviously, these statements will leave the patient confused, anxious and frightened. Although scleroderma can cause pain and disability, fear and misunderstanding may create more distress than the disease itself. In fact, studies find that mood and quality of life in scleroderma are affected more by personality traits and social support than the severity of the disease (1,2). In this chapter, we present a conceptual framework that we use when attempting to explain the complexities of scleroderma to patients. During the actual encounter, we individualize the degree and amount of detail based on the current manifestations of the disease, the patient’s educational level, and the complexity of diagnostic and treatment 1 program.
    [Show full text]
  • Systemic Lupus Erythematosus: Pathogenesis 20 and Clinical Features
    Systemic Lupus Erythematosus: Pathogenesis 20 and Clinical Features George Bertsias, Ricard Cervera, Dimitrios T Boumpas A previous version was coauthored by Ricard Cervera, Gerard Espinosa and David D’Cruz Learning objectives: • Use the epidemiology and natural history of nervous, gastrointestinal, and haematological systemic lupus erythematosus (SLE) to inform systems diagnostic and therapeutic decisions • Evaluate the challenges in the diagnosis and • Describe and explain the key events in the differential diagnosis of lupus and the pitfalls pathogenesis of SLE and critically analyse the in the tests used to diagnose and monitor contribution of genetics, epigenetics, hormonal, lupus activity and environmental factors to the immune • Identify important aspects of the disease such aberrancies found in the disease as women’s health issues (ie, contraception and • Explain the key symptoms and signs of the pregnancy) and critical illness diseases and the tissue damage associated • Outline the patterns of SLE expression in with SLE specific subsets of patients depending on age, • State the classification criteria of lupus and their gender, ethnicity, and social class limitations when used for diagnostic purposes • Classify and assess patients according to • Describe and explain the clinical manifestations the severity of system involvement and use of SLE in the musculoskeletal, dermatological, appropriate clinical criteria to stratify patients in renal, respiratory, cardiovascular, central terms of the risk of morbidity and mortality 1 Introduction ‘wolf’s bite’. In 1846 the Viennese physician Ferdinand von Hebra (1816–1880) introduced the butterfl y metaphor to Systemic lupus erythematosus (SLE) is the prototypic describe the malar rash. He also used the term ‘lupus multisystem autoimmune disorder with a broad spectrum erythematosus’ and published the fi rst illustrations in his of clinical presentations encompassing almost all organs Atlas of Skin Diseases in 1856.
    [Show full text]
  • Systemic Sclerosis: Is There a Treatment Yet?
    Analls ofthe Rheumatic Diseases 1990; 49: 735-737 735 REVIEW ARTICLE Ann Rheum Dis: first published as 10.1136/ard.49.9.735 on 1 September 1990. Downloaded from Systemic sclerosis: Is there a treatment yet? Carol M Black Systemic sclerosis is, as yet, an untreatable An important international step in classifi- disase and the choice and evaluation of any cation has been the agreement by a number of modifying treatment are extremely difficult. doctors, as outlined in a recent editorial,' that This is because: (a) the disorder is heterogeneous there are two major variants ofsystemic sclerosis: and its severity and rate of progression highly the diffuse cutaneous and the limited cutaneous variable; (b) there is a tendency towards spon- subtypes (table). Choice of any treatment and taneous improvement during the later stages of design ofany therapeutic trial should be planned the disease, particularly within the more benign with these distinct variants taken into account; and numerically larger subset; (c) the cause is if not, the results may well be meaningless. For unknown, its pathogenesis uncertain, and the example, patients with late stage diffuse disease relation between the vascular damage, immune are usually not suitable for clinical trials as dysfunction, and fibrosis speculative; (d) there measurable improvement can hardly be expected is a paucity of objective criteria for ascertain- when advanced fibrosis, vascular damage, and ing improvement (or deterioration) in the tissue atrophy are present; equally, aggressive disease, particularly with respect to visceral disease modifying drugs are hardly justified in change. patients who have indolent disease with limited This review will not seek to describe the extensions and in whom measurable change will numerous ways in which systemic sclerosis has probably be absent.
    [Show full text]