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1. Full Text A monogenic autoinflammatory disease with fatal vasculitis: deficiency of adenosine deaminase 2
by Sahin, Sezgin and Adrovic, Amra and Kasapcopur, Ozgur
Current Opinion in Rheumatology, ISSN 1040-8711, 01/2020, Volume 32, Issue 1, pp. 3 - 14
PURPOSE OF REVIEWTo recap the expanding clinical spectrum, genotype–phenotype associations and treatment options in the light of recently published articles... 
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2. Full Text Pediatric rheumatology in Turkey
by Adrovic, Amra and Kasapcopur, Ozgur
Rheumatology International, ISSN 0172-8172, 2019, Volume 39, Issue 3, pp. 431 - 440
Pediatric Rheumatology is an emerging specialty in Turkey with increasing number of available centers and specialists all over the country. In this paper, we... 
Spondyloarthropathies | Familial Mediterranean fever | Turkey | Pediatric rheumatology | Juvenile idiopathic arthritis | SYSTEMIC-LUPUS-ERYTHEMATOSUS | BEHCETS-DISEASE | RHEUMATOLOGY | ADENOSINE-DEAMINASE 2 | CHILDHOOD WEGENER GRANULOMATOSIS | HENOCH-SCHONLEIN PURPURA | EULAR/PRINTO/PRES CRITERIA | POLYARTERITIS-NODOSA | ONSET | Pediatrics | Arthritis | Children | Diseases
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3. Full Text Two Randomized Trials of Canakinumab in Systemic Juvenile Idiopathic Arthritis
by Ruperto, Nicolino and Brunner, Hermine I and Quartier, Pierre and Constantin, Tamás and Wulffraat, Nico and Horneff, Gerd and Brik, Riva and McCann, Liza and Kasapcopur, Ozgur and Rutkowska-Sak, Lidia and Schneider, Rayfel and Berkun, Yackov and Calvo, Inmaculada and Erguven, Muferet and Goffin, Laurence and Hofer, Michael and Kallinich, Tilmann and Oliveira, Sheila K and Uziel, Yosef and Viola, Stefania and Nistala, Kiran and Wouters, Carine and Cimaz, Rolando and Ferrandiz, Manuel A and Flato, Berit and Gamir, Maria Luz and Kone-Paut, Isabelle and Grom, Alexei and Magnusson, Bo and Ozen, Seza and Sztajnbok, Flavio and Lheritier, Karine and Abrams, Ken and Kim, Dennis and Martini, Alberto and Lovell, Daniel J and PRCSG and PRINTO
The New England Journal of Medicine, ISSN 0028-4793, 12/2012, Volume 367, Issue 25, pp. 2396 - 2406
In two placebo-controlled trials, canakinumab, an anti-interleukin-1β monoclonal antibody, achieved a response, prevented flares, and allowed glucocorticoid... 
RHEUMATOID-ARTHRITIS | CRITERIA | MEDICINE, GENERAL & INTERNAL | RECEPTOR ANTAGONIST ANAKINRA | EFFICACY | DOUBLE-BLIND | PRELIMINARY DEFINITION | FOLLOW-UP | CLASSIFICATION | SELECT CATEGORIES | CHILDREN | Glucocorticoids - therapeutic use | Double-Blind Method | Humans | Antibodies, Monoclonal - adverse effects | Kaplan-Meier Estimate | Child, Preschool | Antibodies, Monoclonal - therapeutic use | Male | Thrombocytopenia - chemically induced | Methotrexate - therapeutic use | Infection - chemically induced | Arthritis, Juvenile - drug therapy | Macrophage Activation Syndrome - etiology | Anti-Inflammatory Agents, Non-Steroidal - therapeutic use | Adolescent | Arthritis, Juvenile - complications | Female | Drug Therapy, Combination | Neutropenia - chemically induced | Child | Interleukin-1beta - antagonists & inhibitors | Drugs | Dose-response relationship (Biochemistry) | Product/Service Evaluations | Drug therapy | Juvenile arthritis | Pediatrics | Cytokines | Fever | Rheumatology | Index Medicus | Abridged Index Medicus
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4. Full Text Mutant Adenosine Deaminase 2 in a Polyarteritis Nodosa Vasculopathy
by Navon Elkan, Paulina and Pierce, Sarah B and Segel, Reeval and Walsh, Tom and Barash, Judith and Padeh, Shai and Zlotogorski, Abraham and Berkun, Yackov and Press, Joseph J and Mukamel, Masha and Voth, Isabel and Hashkes, Philip J and Harel, Liora and Hoffer, Vered and Ling, Eduard and Yalcinkaya, Fatos and Kasapcopur, Ozgur and Lee, Ming K and Klevit, Rachel E and Renbaum, Paul and Weinberg-Shukron, Ariella and Sener, Elif F and Schormair, Barbara and Zeligson, Sharon and Marek-Yagel, Dina and Strom, Tim M and Shohat, Mordechai and Singer, Amihood and Rubinow, Alan and Pras, Elon and Winkelmann, Juliane and Tekin, Mustafa and Anikster, Yair and King, Mary-Claire and Levy-Lahad, Ephrat
The New England Journal of Medicine, ISSN 0028-4793, 03/2014, Volume 370, Issue 10, pp. 921 - 931
Adenosine deaminase 2 (ADA2) is a protein with at least two functions. It is a growth factor affecting leukocytes and endothelial cells and an enzyme that... 
CRITERIA | MEDICINE, GENERAL & INTERNAL | CLASSIFICATION | GRANULOMATOSIS | MICE | Adenosine Deaminase - chemistry | Georgia (Republic) | Adenosine Deaminase - genetics | Adenosine Deaminase - metabolism | Humans | Middle Aged | Intercellular Signaling Peptides and Proteins - genetics | Child, Preschool | Intercellular Signaling Peptides and Proteins - chemistry | Infant | Male | Polyarteritis Nodosa - genetics | Genes, Recessive | Jews - genetics | Polyarteritis Nodosa - pathology | Exome | Intercellular Signaling Peptides and Proteins - metabolism | Turkey | Pedigree | Adolescent | Age of Onset | Female | Mutation | Child | Blood circulation disorders | Genetic aspects | Research | Gene mutations | Risk factors | Pediatrics | Adenosine | Genealogy | Rheumatology | Heredity | Adenosine deaminase | Mammalian cells | Proteins | Vasculitis | Vascular diseases | Enzymatic activity | Children | Protein structure | Deoxyribonucleic acid--DNA
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5. Biological agents in familial mediterranean fever focusing on colchicine resistance and amyloidosis
by Sozeri, Betul and Kasapcopur, Ozgur
Current Medicinal Chemistry, ISSN 0929-8673, 2015, Volume 22, Issue 16, pp. 1986 - 1991
Familial Mediterranean fever (FMF) is the most common hereditary autoinflammatory syndrome. FMF is caused by mutations in the MEFV gene which encodes the pyrin... 
Colchicine resistance | Therapy | Familial Mediterranean fever | CHEMISTRY, MEDICINAL | PROTEIN | EFFICACY | PERIODIC SYNDROME | therapy | CANAKINUMAB | BIOCHEMISTRY & MOLECULAR BIOLOGY | SUBCLINICAL INFLAMMATION | ARTHRITIS | TRIAL | INTERFERON-ALPHA | DOUBLE-BLIND | PHARMACOLOGY & PHARMACY | TURKISH PATIENTS | Biological Factors - therapeutic use | Colchicine - pharmacology | Interleukin-1 - metabolism | Colchicine - therapeutic use | Humans | Amyloidosis - drug therapy | Drug Resistance - drug effects | Familial Mediterranean Fever - drug therapy | Biological Factors - pharmacology
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6. Full Text Efficacy and safety of canakinumab in adolescents and adults with colchicine-resistant familial Mediterranean fever
by Gül, Ahmet and Ozdogan, Huri and Erer, Burak and Ugurlu, Serdal and Kasapcopur, Ozgur and Davis, Nicole and Sevgi, Serhan
Arthritis Research and Therapy, ISSN 1478-6354, 09/2015, Volume 17, Issue 1, p. 243
Introduction: This open-label pilot study aimed to investigate the efficacy of canakinumab in colchicine-resistant familial Mediterranean fever (FMF) patients.... 
TRIAL | PERIODIC SYNDROME | RHEUMATOLOGY | Colchicine - pharmacology | Drug Administration Schedule | Tubulin Modulators - pharmacology | Colchicine - therapeutic use | Humans | Antibodies, Monoclonal - adverse effects | Antibodies, Monoclonal - therapeutic use | Male | Treatment Outcome | Familial Mediterranean Fever - drug therapy | Tubulin Modulators - therapeutic use | Young Adult | Vomiting - chemically induced | Pilot Projects | C-Reactive Protein - metabolism | Adolescent | Adult | Female | Surveys and Questionnaires | Child | Headache - chemically induced | Drug Resistance | Youth | Familial Mediterranean fever | Genetic aspects | Teenagers | Colchicine
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7. Full Text Familial Mediterranean fever and periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: shared features and main differences
by Adrovic, Amra and Adrovic, Amra and Sahin, Sezgin and Sahin, Sezgin and Barut, Kenan and Barut, Kenan and Kasapcopur, Ozgur and Kasapcopur, Ozgur
Rheumatology International, ISSN 0172-8172, 1/2019, Volume 39, Issue 1, pp. 29 - 36
Autoinflammatory diseases are characterized by fever attacks of varying durations, associated with variety of symptoms including abdominal pain,... 
Medicine & Public Health | Recurrent fever | Rheumatology | Familial Mediterranean fever | PFAPA syndrome | Colchicine | Anti-interleukin-1 agents | CRITERIA | ADULT PATIENTS | ACTIVATION | RHEUMATOLOGY | CHILDHOOD | MEFV GENE-MUTATIONS | CHILDREN
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8. Full Text Clinical Features, Treatment, and Outcome of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A Multinational, Multicenter Study of 362 Patients
by Minoia, Francesca and Davì, Sergio and Horne, AnnaCarin and Demirkaya, Erkan and Bovis, Francesca and Li, Caifeng and Lehmberg, Kai and Weitzman, Sheila and Insalaco, Antonella and Wouters, Carine and Shenoi, Susan and Espada, Graciela and Ozen, Seza and Anton, Jordi and Khubchandani, Raju and Russo, Ricardo and Pal, Priyankar and Kasapcopur, Ozgur and Miettunen, Paivi and Maritsi, Despoina and Merino, Rosa and Shakoory, Bita and Alessio, Maria and Chasnyk, Vyacheslav and Sanner, Helga and Gao, Yi‐Jin and Huasong, Zeng and Kitoh, Toshiyuki and Avcin, Tadej and Fischbach, Michel and Frosch, Michael and Grom, Alexei and Huber, Adam and Jelusic, Marija and Sawhney, Sujata and Uziel, Yosef and Ruperto, Nicolino and Martini, Alberto and Cron, Randy Q and Ravelli, Angelo and Pediat Rheumatology Collaborative and Histiocyte Soc and Childhood Arthrit Rheumatology Res and Pediat Rheumatology Int Trials Org and Pediatric Rheumatology Collaborative Study Group and Pediatric Rheumatology International Trials Organization and Childhood Arthritis and Rheumatology Research Alliance and Histiocyte Society
Arthritis & Rheumatology, ISSN 2326-5191, 11/2014, Volume 66, Issue 11, pp. 3160 - 3169
Objective To describe the clinical, laboratory, and histopathologic features, current treatment, and outcome of patients with macrophage activation syndrome... 
RHEUMATOID-ARTHRITIS | DIAGNOSIS | DISEASE | GUIDELINES | LUPUS-ERYTHEMATOSUS | DISORDERS | ANAKINRA | RHEUMATOLOGY | HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS | CHILDREN | CYCLOSPORINE | Macrophage Activation Syndrome - mortality | Prevalence | Macrophage Activation Syndrome - drug therapy | Humans | Child, Preschool | Etoposide - therapeutic use | Intensive Care Units - statistics & numerical data | Male | Survival Rate | Treatment Outcome | International Cooperation | Adrenal Cortex Hormones - therapeutic use | Fever - epidemiology | Macrophage Activation Syndrome - etiology | Cyclosporine - therapeutic use | Hepatomegaly - epidemiology | Splenomegaly - epidemiology | Arthritis, Juvenile - complications | Female | Retrospective Studies | Child | Biological Products - therapeutic use | Cohort Studies | Laboratories | Patients | Mortality
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9. Full Text Anaphylactic reaction to anakinra in a child with steroid-dependent idiopathic recurrent pericarditis and successful management with canakinumab
by Epçaçan, Serdar and Sahin, Sezgin and Kasapcopur, Ozgur
Cardiology in the Young, ISSN 1047-9511, 04/2019, Volume 29, Issue 4, pp. 549 - 551
Conventional algorithm for treatment of pericarditis and prevention of recurrences consists of non-steroid anti-inflammatory drugs and/or colchicine, followed... 
Idiopathic recurrent pericarditis | treatment | anti-IL 1 agents | PEDIATRICS | CARDIAC & CARDIOVASCULAR SYSTEMS | RECEPTOR ANTAGONIST ANAKINRA | Corticoids | Pericarditis | Antiinflammatory agents | Corticosteroids | Disease | Cytokines | Interleukin 1 receptor antagonist | Interleukin | Rheumatology | Inflammation | Family medical history | Patients | Anaphylaxis | Algorithms | Interleukin 1 | Nonsteroidal anti-inflammatory drugs | Colchicine | Cardiology | Drug dosages | Steroids
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10. Full Text Canakinumab for the Treatment of Autoinflammatory Recurrent Fever Syndromes
by De Benedetti, Fabrizio and Gattorno, Marco and Anton, Jordi and Ben-Chetrit, Eldad and Frenkel, Joost and Hoffman, Hal M and Koné-Paut, Isabelle and Lachmann, Helen J and Ozen, Seza and Simon, Anna and Zeft, Andrew and Calvo Penades, Inmaculada and Moutschen, Michel and Quartier, Pierre and Kasapcopur, Ozgur and Shcherbina, Anna and Hofer, Michael and Hashkes, Philip J and Van der Hilst, Jeroen and Hara, Ryoki and Bujan-Rivas, Segundo and Constantin, Tamas and Gul, Ahmet and Livneh, Avi and Brogan, Paul and Cattalini, Marco and Obici, Laura and Lheritier, Karine and Speziale, Antonio and Junge, Guido
The New England Journal of Medicine, ISSN 0028-4793, 05/2018, Volume 378, Issue 20, pp. 1908 - 1919
The anti–interleukin-1 antibody canakinumab was effective at controlling and preventing recurrence of flares in autoimmune inflammatory diseases: familial... 
MEDICINE, GENERAL & INTERNAL | FAMILIAL MEDITERRANEAN FEVER | INFLAMMATION | HYPERIMMUNOGLOBULINEMIA-D | INTERLEUKIN-1-BETA SECRETION | PHASE-II | OPEN-LABEL | ANAKINRA | COLCHICINE-RESISTANT | PERIODIC SYNDROME TRAPS | AA AMYLOIDOSIS | Double-Blind Method | Humans | Antibodies, Monoclonal - adverse effects | Child, Preschool | Antibodies, Monoclonal - therapeutic use | Male | Familial Mediterranean Fever - drug therapy | Mevalonate Kinase Deficiency - drug therapy | Hereditary Autoinflammatory Diseases - drug therapy | Dose-Response Relationship, Drug | Young Adult | Antibodies, Monoclonal - administration & dosage | Injections, Subcutaneous | Adolescent | Adult | Female | Child | Fever - drug therapy | Interleukin-1beta - antagonists & inhibitors | Prevention | Treatment outcome | Fever in children | Diagnosis | Analysis | Pediatrics | Writers | Cytokines | Internal medicine | Laboratories | Rheumatology | Disease control | Patients | Fever | Medicine | Mevalonate kinase | Infectious diseases | Mevalonic acid | Immunology | Hospitals | Familial Mediterranean fever | Mutation | Colchicine | Drug dosages | University colleges
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11. Full Text Effect of strengthening versus balance-proprioceptive exercises on lower extremity function in patients with juvenile idiopathic arthritis
by Baydogan, Saime Nilay and Tarakci, Ela and Kasapcopur, Ozgur
American Journal of Physical Medicine and Rehabilitation, ISSN 0894-9115, 06/2015, Volume 94, Issue 6, pp. 417 - 428
exercise | balance | proprioception | Juvenile idiopathic arthritis
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12. Juvenile Scleroderma-What has Changed in the Meantime?
by Adrovic, Amra and Sahin, Sezgin and Barut, Kenan and Kasapcopur, Ozgur and Kasapcopur, Ozgur
Current rheumatology reviews, ISSN 1573-3971, 2018, Volume 14, Issue 3, pp. 219 - 225
Background: Juvenile scleroderma is a rarely seen chronic connective tissue disorder characterized by stiffening of the skin. The frequency of the disease was... 
morphea | skin stiffening | linear scleroderma | juvenile localized scleroderma | organ involvement | Juvenile systemic sclerosis | Cyclosporins | Histocompatibility antigen HLA | Genetic factors | Children | Localization | Scleroderma | Methotrexate
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13. Full Text Panniculitis in juvenile dermatomyositis: Report of a case and review of the published work
by Salman, Andac and Kasapcopur, Ozgur and Ergun, Tulin and Durmus Ucar, Ayse Nigar and Demirkesen, Cuyan
The Journal of Dermatology, ISSN 0385-2407, 08/2016, Volume 43, Issue 8, pp. 951 - 953
We report a 15‐year‐old girl who presented with indurated, subcutaneous nodules in addition to classical findings of juvenile dermatomyositis.... 
subcutaneous tissue | panniculitis | dermatomyositis | juvenile dermatomyositis | pediatric | Juvenile dermatomyositis | Subcutaneous tissue | Pediatric | Dermatomyositis | Panniculitis | CHILDHOOD DERMATOMYOSITIS | SKIN | MANIFESTATIONS | DERMATOLOGY | Panniculitis - diagnosis | Diagnosis, Differential | Panniculitis - etiology | Humans | Adolescent | Female | Panniculitis - pathology | Dermatomyositis - complications | Dermatomyositis - pathology
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14. Full Text Clinical, imaging and genotypical features of three deceased and five surviving cases with ADA2 deficiency
by Sahin, Sezgin and Sahin, Sezgin and Adrovic, Amra and Adrovic, Amra and Barut, Kenan and Barut, Kenan and Ugurlu, Serdal and Ugurlu, Serdal and Turanli, Eda Tahir and Turanli, Eda Tahir and Ozdogan, Huri and Ozdogan, Huri and Kasapcopur, Ozgur and Kasapcopur, Ozgur
Rheumatology International, ISSN 0172-8172, 1/2018, Volume 38, Issue 1, pp. 129 - 136
Deficiency of adenosine deaminase type 2 (DADA2) is a rare form of autoinflammatory disorder with limited reported cases. In this paper, we have presented the... 
CECR1 | Deficiency of ADA2 | Stroke | Vasculitis | Medicine & Public Health | Autoinflammatory disease | Rheumatology | Livedo reticularis | PHENOTYPE | RHEUMATOLOGY | ADENOSINE-DEAMINASE 2 | POLYARTERITIS-NODOSA | MUTATIONS | VASCULOPATHY | TYPE-2 | Agammaglobulinemia - genetics | Adenosine Deaminase - genetics | Severe Combined Immunodeficiency - diagnosis | Humans | Severe Combined Immunodeficiency - drug therapy | Child, Preschool | Magnetic Resonance Angiography | Agammaglobulinemia - diagnosis | Genotype | Infant | Young Adult | Severe Combined Immunodeficiency - genetics | Agammaglobulinemia - drug therapy | Adolescent | Age of Onset | Agammaglobulinemia - diagnostic imaging | Adult | Adenosine Deaminase - deficiency | Child | Severe Combined Immunodeficiency - diagnostic imaging | Tumor Necrosis Factor-alpha - antagonists & inhibitors | Biological Products - therapeutic use | Stroke (Disease) | Medical records | Gene mutations | Therapeutics | Homeopathy | Materia medica and therapeutics | Patients | Mutation
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15. Full Text Leap Motion Controller-based training for upper extremity rehabilitation in children and adolescents with physical disabilities: A randomized controlled trial
by Tarakci, Ela and Arman, Nilay and Tarakci, Devrim and Kasapcopur, Ozgur
Journal of hand therapy : official journal of the American Society of Hand Therapists, ISSN 0894-1130, 04/2019
Randomized controlled trial. Juvenile idiopathic arthritis (JIA), cerebral palsy (CP), and brachial plexus birth injury (BPBI) are the most common disorders... 
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16. Full Text Evaluation of co-existing diseases in children with familial Mediterranean fever
by Yildiz, Mehmet and Adrovic, Amra and Tasdemir, Emre and Baba-Zada, Khanim and Aydin, Muhammed and Koker, Oya and Sahin, Sezgin and Barut, Kenan and Kasapcopur, Ozgur
Rheumatology international, ISSN 0172-8172, 07/2019
Familial Mediterranean fever (FMF) is A common periodic fever syndrome. The causative gene of the FMF is named Mediterranean Fever gene (MEFV). Increased... 
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17. Full Text Performance of recently proposed periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome criteria in a region endemic for familial Mediterranean fever
by Adrovic, Amra and Yıldız, Mehmet and Kanber, Melisa and Ulkersoy, Ipek and Gucuyener, Neslihan and Koker, Oya and Sahin, Sezgin and Barut, Kenan and Kasapcopur, Ozgur
Rheumatology international, ISSN 0172-8172, 07/2019
The periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is an auto-inflammatory condition characterized by recurrent... 
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18. Full Text FMF50: a score for assessing outcome in familial Mediterranean fever
by Ozen, Seza and Demirkaya, Erkan and Duzova, Ali and Erdogan, Ozlem and Erken, Eren and Gul, Ahmet and Kasapcopur, Ozgur and Kasifoglu, Timucin and Kisacik, Bunyamin and Ozdogan, Huri and Tunca, Mehmet and Acikel, Cengizhan and FMF Arthrit Vasculitis Orphan Dis and Turkish FMF Study Grp and FMF Arthritis Vasculitis and Orphan disease Research in pediatric rheumatology (FAVOR) and Turkish FMF study group and for the FMF Arthritis Vasculitis and Orphan disease Research in pediatric rheumatology (FAVOR) and Turkish FMF study group
Annals of the Rheumatic Diseases, ISSN 0003-4967, 05/2014, Volume 73, Issue 5, pp. 897 - 901
Background Colchicine is the main treatment for familial Mediterranean fever (FMF). However, biological agents and other treatments are available for patients... 
CRITERIA | DIAGNOSIS |