Skin and joint manifestations
In the early stages, the skin becomes edematous, acquires a doughy consistency and a glossy appearance. Microscopic examination of the dermis reveals perivascular infiltrates of CD4+ T lymphocytes and plasma cells, along with disorganization of connective tissue. The walls of capillaries and arterioles thicken, and their lumen narrows.
Subsequently, severe dermal sclerosis, vascular hyalinosis, and atrophy of the epidermis with its appendages (sweat and sebaceous glands, hair follicles) develop. Calcinosis cutis is a specific feature, especially periarticularly on the fingers. Ultimately, a mask-like face, sclerodactyly, and severe trophic disorders (ulcers, alopecia) develop, sometimes leading to self-amputation of the fingers.
Joint involvement presents as polyarthritis of the small joints of the hands. Lymphoplasmacytic infiltration and synoviocyte hyperplasia occur in the synovial membrane. Unlike rheumatoid arthritis, tissue destruction is not typical — the process ends with synovial sclerosis and vascular obliteration.
Kidney involvement
Occurs in 75% of patients and is one of the main threats to life. It is based on scleroderma microangiopathy, primarily affecting interlobular arteries.
Morphological features:
- Concentric intimal hyperplasia.
- Mucoid swelling.
- Fibrinoid necrosis of vessel walls with luminal thrombosis.
These changes lead to renal infarctions. The «true scleroderma kidney» syndrome develops — a severe condition combining infarctions and acute kidney injury. Additionally, 30% of patients develop arterial hypertension due to renal artery involvement. Chronic glomerulonephritis is rare in this context.
Lung and heart changes
The lungs are affected in more than 50% of cases. The early phase is represented by alveolitis with tissue infiltration by macrophages, lymphocytes, and band neutrophils. In the late phase, basal diffuse interstitial pneumofibrosis develops, which is inevitably accompanied by pulmonary hypertension. Pleural fibrosis is frequently found, and fibrinous pleuritis is less common.
In 30% of patients, «scleroderma heart» develops. Pathomorphologically, this manifests as productive vasculitis, as well as micro- and macrofocal cardiosclerosis. Sclerosis of the parietal and valvular endocardium, as well as chordae tendineae, leads to the development of specific scleroderma heart disease.
Gastrointestinal tract and liver
Gastrointestinal pathology is observed in 90% of patients. The most severe changes occur in the esophagus:
- Sclerosis of the muscular layer (predominantly in the lower third).
- Motility disorders and reflux esophagitis.
- Formation of strictures and peptic ulcers.
- Epithelial metaplasia (Barrett's esophagus).
Motility disorders, sclerosis, and mucosal atrophy also develop in other parts of the gastrointestinal tract, clinically manifesting as malabsorption syndrome. Diverticula formation and diverticulitis may occur in the colon.
In CREST syndrome, the liver is involved in the pathological process, with the development of primary biliary cholangitis (PBC).
Outcomes
Progression of systemic sclerosis leads to severe organ and system failure. The main causes of death are uremia (against the background of renal failure), as well as cardiovascular and cardiopulmonary failure.
Briefly: answers to topical questions
What specific autoantibodies are markers of systemic sclerosis?
Markers of systemic sclerosis include antinuclear antibodies and a number of highly specific autoantibodies.
- Anticentromere antibodies — a highly specific marker of the disease.
- Antitopoisomerase antibodies (anti-Scl70) — antibodies to topoisomerase I.
- Antifibrillarin antibodies — a specific serological marker.
- Anti-PM-Scl antibodies — detected in the immunological profile.
- Anti-RNA polymerase I or III antibodies — highly specific markers.
- Antibodies to fibroblast cell membranes — indicate impaired humoral immunity.
- Antibodies to type I and IV collagen — detected in the disease.
What is the pathogenesis of pulmonary hypertension in systemic sclerosis?
The pathogenesis of pulmonary hypertension in systemic sclerosis involves several diverse mechanisms.
- Interstitial lung disease — development of basal diffuse interstitial pneumofibrosis.
- Left ventricular diastolic dysfunction — occurs against the background of myocardial fibrosis.
- Proliferative vasculopathy — involvement of small arterioles and venules of the pulmonary circulation.
- Pulmonary veno-occlusive disease phenotype — forms as a result of pathological vascular changes.
In the diffuse form of the disease, hypertension is more often caused by interstitial disease, while in the limited form, it is caused by vasculopathy.
To remember the main visceral targets, use the K-L-H-G rule (Kidneys, Lungs, Heart, GI tract). It is the failure of these systems that determines the outcome of the disease.
Class and exam questions
How does joint involvement in scleroderma differ from rheumatoid arthritis?
In systemic sclerosis, joint tissue destruction is not typical. The process ends with sclerosis of the synovial membrane and vascular obliteration without destruction of cartilage and bone.
What is the «true scleroderma kidney»?
This is a life-threatening complication in which microangiopathy and fibrinoid necrosis of vessels lead to multiple renal infarctions, resulting in acute kidney injury.
Which part of the gastrointestinal tract is most frequently affected and how does it manifest?
The most pronounced changes occur in the esophagus. Sclerosis of the muscular layer of the lower third develops, motility is impaired, and reflux esophagitis, strictures, and Barrett's metaplasia occur.
What else to study on this topic
- Morphogenesis of scleroderma microangiopathy
- Pathogenesis of pulmonary hypertension in interstitial pneumofibrosis
- Differences between scleroderma heart disease and rheumatic heart disease
- CREST syndrome: clinical and morphological characteristics
- Mechanisms of malabsorption syndrome development in scleroderma
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