Related Experiment Video
Updated: Jan 22, 2026
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
20.8K
Immunopathogenesis of Juvenile Systemic Sclerosis
Anne M Stevens1, Kathryn S Torok2, Suzanne C Li3
1Division of Rheumatology, Department of Pediatrics, University of Washington, Seattle, WA, United States.
Frontiers in Immunology
|July 12, 2019
Summary
Juvenile-onset systemic sclerosis (jSSc) is a rare autoimmune disease. Research suggests targeting dermal white adipose tissue dendritic cells may reverse fibrosis in jSSc.
Area of Science:
- Rheumatology
- Immunology
- Pediatrics
Background:
- Juvenile-onset systemic sclerosis (jSSc) is a rare, severe autoimmune condition.
- jSSc involves life-threatening organ inflammation and fibrosis, yet shows better outcomes than adult SSc.
- jSSc shares etiological similarities with adult SSc, including inflammatory mediators and autoantibodies, but has unique genetic factors.
Purpose of the Study:
- To summarize the current understanding of juvenile-onset systemic sclerosis.
- To highlight the differences and similarities between jSSc and adult-onset SSc.
- To explore potential new therapeutic targets for the fibrotic stage of jSSc.
Main Methods:
- Review of existing literature on jSSc.
- Comparison of jSSc characteristics with adult-onset SSc.
- Analysis of genetic and immunological data.
Main Results:
- jSSc organ manifestations resemble adult SSc but with improved survival.
- jSSc genetics differ from adult female SSc, aligning more with adult male SSc, with unique childhood-specific HLA genes.
- A significant subset of jSSc patients exhibit uncharacterized anti-nuclear antibodies.
Conclusions:
- jSSc presents unique genetic associations compared to adult SSc.
- Current treatments focus on inflammation, but fibrotic mechanisms offer novel therapeutic avenues.
- Dermal white adipose tissue dendritic cells represent a potential target for reversing fibrosis in jSSc.
Related Concept Videos
01:18Second Order systems II
392
In an underdamped second-order system, where the damping ratio ζ is between 0 and 1, a unit-step input results in a transfer function that, when transformed using the inverse Laplace method, reveals the output response. The output exhibits a damped sinusoidal oscillation, and the difference between the input and output is termed the error signal. This error signal also demonstrates damped oscillatory behavior. Eventually, as the system reaches a steady state, the error diminishes to zero.
392
01:21First Order Systems
409
First-order systems, such as RC circuits, are foundational in understanding dynamic systems due to their straightforward input-output relationship. Analyzing their responses to different input functions under zero initial conditions reveals significant insights into system behavior.
When a first-order system is subjected to a unit-step input, its response is characterized by its transfer function. By applying the Laplace transform of the unit-step input to the transfer function, expanding the...
When a first-order system is subjected to a unit-step input, its response is characterized by its transfer function. By applying the Laplace transform of the unit-step input to the transfer function, expanding the...
409
01:20Second Order systems I
579
A servo system exemplifies a second-order system, featuring a proportional controller and load elements that ensure the output position aligns with the input position. The relationship between these components is described by a second-order differential equation. Applying the Laplace transform under zero initial conditions yields the transfer function, showing how inputs are converted to outputs in the system.
By reinterpreting the system, one can derive the closed-loop transfer function, which...
By reinterpreting the system, one can derive the closed-loop transfer function, which...
579
01:26Classification of Systems-I
554
Linearity is a system property characterized by a direct input-output relationship, combining homogeneity and additivity.
Homogeneity dictates that if an input x(t) is multiplied by a constant c, the output y(t) is multiplied by the same constant. Mathematically, this is expressed as:
Homogeneity dictates that if an input x(t) is multiplied by a constant c, the output y(t) is multiplied by the same constant. Mathematically, this is expressed as:
554
01:31Classification of Systems-II
461
Continuous-time systems have continuous input and output signals, with time measured continuously. These systems are generally defined by differential or algebraic equations. For instance, in an RC circuit, the relationship between input and output voltage is expressed through a differential equation derived from Ohm's law and the capacitor relation,
461
01:22Mechanical Systems
601
Mechanical systems are analogous to to electrical networks where springs and masses play similar roles to inductors and capacitors, respectively. A viscous damper in mechanical systems functions similarly to a resistor in electrical networks, dissipating energy. The forces acting on a mass in such systems include an applied force in the direction of motion, counteracted by forces from the spring, a viscous damper, and the mass's acceleration. This interplay of forces is mathematically...
601