scholarly journals Systemic Onset Juvenile Idiopathic Arthritis (sJIA) or Still's Disease: A Diagnostic Challenge; while Presented as Fever of Unknown Origin. 2 Cases

2019 ◽  
Vol 10 (1) ◽  
pp. 84-88
Author(s):  
Gule Tajkia ◽  
Syed Khairul Amin ◽  
Shamim Rima ◽  
Soma Halder ◽  
Fabia Hannan
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Fever Of Unknown Origin ◽  
Unknown Origin ◽  
Chronic Inflammatory Disease ◽  
Joint Disease ◽  
Unknown Etiology ◽  
Still’S Disease ◽  
Diagnostic Challenge ◽  
Still's Disease ◽  
Systemic Onset

Systemic-onset juvenile idiopathic arthritis (sJIA) or Still's disease is a chronic inflammatory disease of unknown etiology belongs to the group of Juvenile Idiopathic Arthritis. In contrast to other JIA patients in whom the joint disease usually overshadows the more general symptomatology, in Systemic-onset juvenile idiopathic arthritis (sJIA) extra-articular features such as spiking fever, hepatosplenomegaly, lymphadenopathy, rash, pleurisy, or pericarditis, and vasculopathy are most prominent. Thus the onset of disease can be vary nonspecific and may suggest bacterial or viral infection, malignancy or other rheumatic disease. As it is highly characterized by its extra-articular systemic illness features, in some ways, it resembles a fever of unknown origin. We present 2 cases of fever of unknown origin, initially presented with fever and other extra-articular features, without any arthritis, but after several months develops arthritis and finally diagnosed as Systemic-onset juvenile idiopathic arthritis (sJIA) or Still's disease . Anwer Khan Modern Medical College Journal Vol. 10, No. 1: Jan 2019, P 84-88

Fever of unknown origin in a patient of systemic onset juvenile idiopathic arthritis

2010 ◽  
Vol 64 (7) ◽  
pp. 333
Author(s):  
VinodKolar Vishwanath ◽  
Arun Karyampudi ◽  
Aishwarya Krishnamurthy ◽  
TarunKumar Dutta
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Fever Of Unknown Origin ◽  
Unknown Origin ◽  
Systemic Onset

scholarly journals S100A12 is a novel molecular marker differentiating systemic-onset juvenile idiopathic arthritis from other causes of fever of unknown origin

2008 ◽  
Vol 58 (12) ◽  
pp. 3924-3931 ◽  
Author(s):  
Helmut Wittkowski ◽  
Michael Frosch ◽  
Nico Wulffraat ◽  
Raphaela Goldbach-Mansky ◽  
Tilmann Kallinich ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Molecular Marker ◽  
Fever Of Unknown Origin ◽  
Unknown Origin ◽  
Systemic Onset

scholarly journals Adult onset Still’s disease presenting as fever of unknown origin: a case report with review of literature

2017 ◽  
Vol 11 ◽  
Author(s):  
Harpreet Singh ◽  
Deepak Jain ◽  
Saroj Dhankhar ◽  
Rekha Mathur
Keyword(s):  
Joint Pain ◽  
Fever Of Unknown Origin ◽  
Unknown Origin ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Rheumatologic Diseases ◽  
Systemic Inflammatory Disease ◽  
Adult Onset Still's Disease

Adult onset Still’s disease (AOSD) is a systemic inflammatory disease with exact etiology and pathogenesis yet to be discovered. AOSD, being an important cause of fever of unknown origin, is diagnosed after ruling out infections, malignancy and other rheumatologic diseases. It may present with fever without typical rash although typical triad is of fever, joint pain and rash. A 35-year old previously healthy man was referred to our hospital with 6 months of fever, joint pain and weight loss. Examination and investigations revealed anaemia, leukocytosis (predominant neutrophilia), lymphadenopathy, hepatomegaly, arthritis and evidence of interstitial lung disease with raised serum ferritin levels. The hematological disorders, infections and other rheumatologic diseases were excluded. The diagnosis of adult onset Still’s disease can be very difficult as there are no specific tests and diagnosis is based on symptom complex. AOSD presenting as fever of unknown origin could be a challenge for the physician to diagnose and manage timely.

scholarly journals Adult-Onset Still’s Disease: Clinical Aspects and Therapeutic Approach

2021 ◽  
Vol 10 (4) ◽  
pp. 733
Author(s):  
Stylianos Tomaras ◽  
Carl Christoph Goetzke ◽  
Tilmann Kallinich ◽  
Eugen Feist
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Large Body ◽  
Adult Onset Still’S Disease ◽  
Clinical Aspects ◽  
Disease Course ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Systemic Onset ◽  
Adult Onset Still's Disease

Adult-onset Still’s disease (AoSD) is a rare systemic autoinflammatory disease characterized by arthritis, spiking fever, skin rash and elevated ferritin levels. The reason behind the nomenclature of this condition is that AoSD shares certain symptoms with Still’s disease in children, currently named systemic-onset juvenile idiopathic arthritis. Immune dysregulation plays a central role in AoSD and is characterized by pathogenic involvement of both arms of the immune system. Furthermore, the past two decades have seen a large body of immunological research on cytokines, which has attributed to both a better understanding of AoSD and revolutionary advances in treatment. Additionally, recent studies have introduced a new approach by grouping patients with AoSD into only two phenotypes: one with predominantly systemic features and one with a chronic articular disease course. Diagnosis presupposes an extensive diagnostic workup to rule out infections and malignancies. The severe end of the spectrum of this disease is secondary haemophagocytic lymphohistiocytosis, better known as macrophage activation syndrome. In this review, we discuss current research conducted on the pathogenesis, diagnosis, classification, biomarkers and complications of AoSD, as well as the treatment strategy at each stage of the disease course. We also highlight the similarities and differences between AoSD and systemic-onset juvenile idiopathic arthritis. There is a considerable need for large multicentric prospective trials.

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