scleroderma

Systemic
sclerosis HRCT image. Provided as-is. Please feel free to categorise, add description, crop.
Wikipedia • CC BY-SA 3.0
Systemic
sclerosis HRCT image. Provided as-is. Please feel free to categorise, add description, crop.
Wikipedia • CC BY-SA 3.0
Lost bones:
differential diagnosis of acro-osteolysis seen by the pediatric rheumatologist. Frontal radiographs of the hands in a 13 year old male patient with systemic sclerosis show tuft resorption consistent with acro-osteolysis. No soft tissue calcifications present
ped-rheum.biomedcentral.com • CC-by-4.0
Clinical and
radiological features of lung disorders related to connective-tissue diseases: a pictorial essay. Axial (A, B) and coronal (C) HRCT images of an SSc patient demonstrate NSIP pattern with ground-glass opacities (black arrows), well depicted in the subpleural and basal regions of the lung; fine reticulations may be also observed. Coronal MPR image is useful to better delineate the presence of small bronchiectasis, which may be seen in the peripheral basal regions (white empty arrows)
insightsimaging.springeropen.com • CC-by-4.0
Clinical and
radiological features of lung disorders related to connective-tissue diseases: a pictorial essay. NSIP pattern with subpleural sparing in a female SSc patient. Axial (A) and sagittal (B) HRCT images demonstrate ground-glass opacities predominantly located in the basal pulmonary regions, with peripheral and subpleural distribution (white arrows). Pulmonary alterations may spare subpleural regions, as well demonstrated in A—namely in the left lower lobe
insightsimaging.springeropen.com • CC-by-4.0
Sklerodermie
mit Beteilung des Ösophagus: Im unteren Ösophagus (glatte Muskulatur!) deutliche Wandstarre mit passivem Durchfallen des Kontrastmittels / des Bolus (Glasrohr-Ösophagus).
Wikipedia • CC BY-SA 4.0
Sklerodermie
mit Beteiligung des Ösophagus: Während im kranialen Abschnitt des Ösophagus noch eine weitgehend normale Peristaltik vorhanden ist, ist der Ösophagus in den unteren zwei Dritteln dilatiert und deutlich hypomotil. Das Kontrastmittel fällt passiv durch das "Glasrohr". Die Passage durch die Cardia erfolgt aber weitgehend regelrecht, was die Abgrenzung zur Achalasie erlaubt.
Wikipedia • CC BY-SA 4.0
Systemic
sclerosis HRCT image. Provided as-is. Please feel free to categorise, add description, crop.
Wikipedia • CC BY-SA 3.0
Radiological
identification and analysis of soft tissue musculoskeletal calcifications. Systemic Sclerosis. Posteroanterior radiograph of the hand shows tumoural calcinosis in the soft tissue (arrow). Note acro-osteolysis (arrowhead) and atrophy of soft tissue (curved arrow), characteristic of systemic sclerosis
insightsimaging.springeropen.com • Open Access
Radiopaedia • CC-by-nc-sa 3.0 • de

Scleroderma, also known as systemic sclerosis, is an autoimmune connective tissue disorder characterized by multisystem fibrosis and soft tissue calcification. As such, it affects many separate organ systems, which are discussed separately:

The remainder of this article is a general discussion of scleroderma.

Epidemiology

Although presentation in childhood is indeed recognized , scleroderma is considered a disease of middle age (30-50 years of age) . There is a strong female predilection (F:M 3:1), even more pronounced during reproductive years (F:M 10:1) .

Clinical presentation

Clinical manifestation depends on which systems are predominantly involved. It should be noted that the majority of patients 70-97% develop an arthropathy that mostly affects the fingers, wrists, and ankles . Esophageal involvement is common, occurring in approximately 80% of patients . Although 90% of patients have pulmonary involvement histologically, only a minority are symptomatic .

Pathology

The disease is characterized by widespread deposition of collagen and other extracellular matrix proteins. This is believed to occur as a result of an abnormal immune response. Small vessels are involved early in the disease, accounting for the involvement of organs with a dense capillary network. This results in perivascular fibrosis and gradual luminal stenosis .

Markers

Serological markers include:

  • elevated erythrocyte sedimentation rate (ESR)
  • rheumatoid factor (RF): positive in 30-40%
  • antinuclear antibodies (ANA): 35-96%
    • anti SCL-70: 30-70% (particularly in diffuse disease)
    • anti-centromere antibodies: 20-40% (particularly in limited disease)
    • antitopoisomerase I
Associations

Radiographic features

These are best discussed under manifestations of individual systems. Please refer to respective subsections.

Treatment and prognosis

No cure for scleroderma exists. Treatment aims to reduce systemic involvement and target particular symptomatic organ systems. For targeted therapy, please refer to the articles that pertain to scleroderma and the particular body system.

Systemic medication aimed at reducing the deposition of fibrous tissue and at reducing inflammation has been tried with limited success .

Overall prognosis is variable, depending on the organ(s) involved and the severity. For patients with limited cutaneous involvement, 10-year survival is approximately 75%. However, patients with diffuse involvement have a 10-year survival closer to 55% .

Siehe auch:
und weiter: