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Scleroderma (systemic sclerosis)

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Connective tissue hardens — recognised early, organs can be better protected.

Also known as: scleroderma · systemic sclerosis · sclerodactyly · CREST syndrome

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This compilation is for information and replaces neither medical diagnosis nor treatment. If you have symptoms, see a doctor.

An autoimmune disease in which connective tissue hardens and thickens. In the fingers this shows as taut, shiny, tethered skin (sclerodactyly) – the fingers become stiff; a Raynaud phenomenon (white-blue-red fingers in the cold) often precedes it. There are skin-limited forms and forms affecting internal organs. Early rheumatology assessment matters (antibodies such as ANA, anti-Scl-70, anti-centromere; nailfold capillaroscopy).

Levers & pathways

studiedKeep the fingers warm

Gloves even at the chilled shelf, keep the whole body warm rather than only the hands, avoid jumps in temperature. Every prevented vessel spasm is a piece of tissue that stays supplied.

studiedSmoking acts twice over here

Nicotine narrows exactly the small vessels that are already set too tight. Stopping smoking is one of the few levers in this illness that lie entirely in one’s own hands.

studiedShow fingertip wounds early

Small, very painful ulcers on the tips heal badly and can become infected. There are vessel-widening treatments for them — the earlier, the more substance is kept.

studiedThe heartburn belongs to the picture

The gullet stiffens too and closes less well at the bottom. Raise the head of the bed, last meal three hours before lying down, smaller portions — usually together with an acid-blocking drug.

studiedCheck lungs and heart yearly

A lung function test and a heart ultrasound once a year find the two involvements that shape the course most — and both can be treated today if they are noticed early.

studiedMeasure the blood pressure yourself

A suddenly and sharply rising blood pressure with headache is, in this illness, a reason to call the same day. There is an effective treatment for it, and it is time-critical.

studiedRule out the threadworm before immune therapy

A threadworm can stay silent in the gut for decades and, under cortisone, biologicals or other immune suppression, flood lung and bloodstream. Anyone who ever went barefoot in the tropics or subtropics is looked for beforehand with a blood test — one effort, and it prevents the worst course this worm has.

infoWhat the medicine does — and what it does not

No drug makes hardened skin soft again. Treatment goes organ by organ: calcium channel blockers and, in severe cases, sildenafil or iloprost for the white fingers; proton pump inhibitors for reflux, often at double dose; nintedanib and mycophenolate for lung scarring; ACE inhibitors in renal crisis — there they changed the outlook most. Higher-dose cortisone can trigger the renal crisis and is therefore used sparingly.

infoNumbers and timeframes

About two to three in ten thousand people are affected, women four to five times as often, usually starting between thirty and fifty. In more than ninety in a hundred, Raynaud’s comes first — often years before anything else. The renal crisis almost always occurs in the first five years, which is why blood pressure is measured at home regularly during that time. Lung function and heart ultrasound are checked yearly.

Possible complaints

studiedWhite-blue-red fingers in the cold

In almost all this phenomenon stands at the beginning, often years before everything else: fingers that go white in cold or excitement, then bluish, then painfully red on warming.

studiedTaut, shiny skin on the fingers

First swollen sausage fingers in the morning, later skin that can no longer be pinched up, and fingers that bend less well. The change travels from the fingertips inwards.

studiedHeartburn and trouble swallowing

The gullet stiffens too: food gets stuck, acid comes up when lying down, a cough at night. That is the most common involvement of the inner organs and it can be treated well.

studiedSores on the fingertips

Small, very painful ulcers or pitted scars on the tips show that the small vessels are strongly affected. They belong shown early, because there are vessel-widening treatments.

studiedBreathlessness and blood pressure as quiet signs

A dry cough and breathlessness on stairs point to the lung, a suddenly sharply rising blood pressure with headache to the kidney. Both begin quietly and are looked for yearly, precisely because they begin quietly.

Blood values

associatedANA

Antinuclear antibodies – screening; often positive. Specific ones: anti-Scl-70 and anti-centromere.

Nutrients

associatedVitamin D

With chronic inflammatory and autoimmune pictures low levels are common — partly because the illness itself lowers the level, partly because less time is spent outdoors. Whether correcting it changes the course is being studied; for bone and muscle it is worthwhile regardless.

studiedVitamin B12

Taken up right at the end of the small bowel and needing a helper from the stomach. If either place is affected a deficiency arises — often only after years, because the liver holds a store.

associatedOmega-3 fatty acids

They give rise to messengers that tend to end inflammatory reactions rather than fuel them. Oily sea fish twice a week is the simplest route; capsules are an option but replace no treatment.

studiedIron

Taken up in the upper small bowel and needing stomach acid. Acid blockers, an inflamed lining and silent blood losses often come together here.

associatedZinc

Needed for steering the defences and for the healing of tissue. A lack is more common with chronic diarrhoea, little meat and pulses, and under cortisone.

Supplements

studiedVitamin D3 (+K2)

Low levels are the rule here, for two reasons: less sun because of the sensitivity to cold, and an absorption problem when the gut is involved. Measured and replaced; on the hardening of the skin it does not act.

studiedVitamin B12

If the small intestine becomes sluggish, bacteria settle there that belong further down — and they use up B12 before it is taken up. A bloated belly, diarrhoea alternating with constipation and a falling B12 value therefore belong together here.

mixedOmega-3 (EPA/DHA)

Examined for the white-finger attacks, with a small effect in individual studies — distinctly weaker than what warmth and leaving off nicotine bring. Nicotine narrows exactly the vessels at issue; that is here the largest single lever.

infoIron

Clearly effective with proven deficiency, useless without. Take only with a documented deficiency — excess is harmful.

Hormones

associatedEstradiol

Women are affected four to nine times more often, mostly between thirty and fifty. Why is open — the difference is so large that it has to be described, even without an explanation.

associatedT4 (thyroxine)

Autoimmune thyroid conditions come along here more often. The value belongs measured once — tiredness and cold sensitivity otherwise fit both and get attributed to the wrong illness.

studiedNoradrenaline

Almost all those affected have Raynaud’s phenomenon, and for many it is the first sign — years before anything else. A newly appearing Raynaud beyond thirty therefore belongs looked at.

Food

studiedSmall meals with oesophageal involvement

The oesophagus stiffens, reflux is common. Smaller portions, staying upright after eating and the last bite 3 hours before lying down is the usual approach.

infoKeep weight up

Swallowing trouble and gut involvement often lead to weight loss. Small energy-dense meals and regular weighing matter more than any elimination diet.

Alternative approaches — what people use

studiedPhysiotherapy

Daily stretching of hands and face keeps mobility while the skin tightens. Mobility once lost is hard to regain — hence early and regularly.

traditionalHeat & cold

Warmth on the hands eases the Raynaud attacks that nearly always come with it. Cold stimuli are the opposite of helpful here.

Frequencies — what is on file

traditionalScleroderma

Listed values: 110 · 174 · 285 · 4370 · 6300 Hz