scleroderma

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

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

Imaging of

intestinal vasculitis focusing on MR and CT enterography: a two-way street between radiologic findings and clinical data. Intestinal vasculitis and benign intestinal pneumatosis in a 52-year-old male with a history of known Scleroderma presenting with abdominal pain. Physical examination revealed generalized abdominal tenderness without rebound or guarding. Upright CXR (A) was done in the ER. Pneumoperitoneum (thick black arrows) was noted at CXR in the absence of signs or symptoms of peritonitis. Due to this discrepancy, abdominal CT scan (B, C) was performed and confirmed pneumoperitoneum (thin black arrow) along with extensive intestinal pneumatosis in the transverse colon (black arrowheads) and jejunum (thick white arrows). The patient underwent conservative treatment. He was discharged one week later with improved signs/symptoms. After three weeks, MRE was obtained and displayed no pneumoperitoneum. Axial T2-W image (D) demonstrates segmental jejunal mural thickening (white dotted oval) with hypointense appearance. Coronal T2-W image (E) shows bowel dilation and the classic hidebound sign (black dotted oval) in the ileum related to known scleroderma. The T2 hypointense segment of thick jejunum is also seen (curved arrows), suggestive of chronic mural thickening perhaps due to fibrosis

insightsimaging.springeropen.com • CC-by-4.0

Systemic

sclerosis HRCT image. Provided as-is. Please feel free to categorise, add description, crop.

Wikipedia • CC BY-SA 3.0

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

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

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

Scleroderma

• Scleroderma - hand manifestations - Ganzer Fall bei Radiopaedia

Nemattalla, W. Scleroderma - hand manifestations. Case study, Radiopaedia.org. (accessed on 27 Oct 2022) https://doi.org/10.53347/rID-7409 • CC by-nc-sa 3.0 (modified)

Systemic

sclerosis HRCT image. Provided as-is. Please feel free to categorise, add description, crop.

Wikipedia • CC BY-SA 3.0

Scleroderma

• Scleroderma - small bowel involvement - Ganzer Fall bei Radiopaedia

Dixon, A. Scleroderma - small bowel involvement. Case study, Radiopaedia.org. (accessed on 27 Oct 2022) https://doi.org/10.53347/rID-9597 • CC by-nc-sa 3.0 (modified)

Scleroderma

• Scleroderma with interstitial fibrosis - Ganzer Fall bei Radiopaedia

Yang, N. Scleroderma with interstitial fibrosis. Case study, Radiopaedia.org. (accessed on 27 Oct 2022) https://doi.org/10.53347/rID-8604 • CC by-nc-sa 3.0 (modified)

Scleroderma

• Scleroderma - musculoskeletal and gastrointestinal manifestations - Ganzer Fall bei Radiopaedia

Radswiki, T. Scleroderma - musculoskeletal and gastrointestinal manifestations. Case study, Radiopaedia.org. (accessed on 27 Oct 2022) https://doi.org/10.53347/rID-11919 • CC by-nc-sa 3.0 (modified)

Scleroderma

• Scleroderma - Ganzer Fall bei Radiopaedia

Radswiki, T. Scleroderma. Case study, Radiopaedia.org. (accessed on 27 Oct 2022) https://doi.org/10.53347/rID-11918 • CC by-nc-sa 3.0 (modified)

Scleroderma

• Progressive systemic sclerosis involving the knee - Ganzer Fall bei Radiopaedia

Skalski, M. Progressive systemic sclerosis involving the knee. Case study, Radiopaedia.org. (accessed on 27 Oct 2022) https://doi.org/10.53347/rID-25124 • CC by-nc-sa 3.0 (modified)

Scleroderma

• Scleroderma with acro-osteolysis - Ganzer Fall bei Radiopaedia

Dixon, A. Scleroderma with acro-osteolysis. Case study, Radiopaedia.org. (accessed on 27 Oct 2022) https://doi.org/10.53347/rID-62023 • CC by-nc-sa 3.0 (modified)

Scleroderma

• Scleroderma-related interstitial lung disease - Ganzer Fall bei Radiopaedia

Niknejad, M. Scleroderma-related interstitial lung disease. Case study, Radiopaedia.org. (accessed on 27 Oct 2022) https://doi.org/10.53347/rID-94807 • CC by-nc-sa 3.0 (modified)

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

Scleroderma

• Scleroderma - Ganzer Fall bei Radiopaedia

Farooq, S. Scleroderma. Case study, Radiopaedia.org. (accessed on 27 Oct 2022) https://doi.org/10.53347/rID-9735 • CC by-nc-sa 3.0 (modified)

Systemic

sclerosis HRCT image. Provided as-is. Please feel free to categorise, add description, crop.

Wikipedia • CC BY-SA 3.0

Acro-osteolysis

• Scleroderma with acro-osteolysis - Ganzer Fall bei Radiopaedia

Dixon A, Scleroderma with acro-osteolysis. Case study, Radiopaedia.org (Accessed on 29 Aug 2023) https://doi.org/10.53347/rID-62023 • CC by-nc-sa 3.0 (modified)

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

CREST syndrome
other connective tissue disorders:

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% .