Scleroderma systemic M34.0

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 16.10.2021

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Synonym(s)

progressive scleroderma; Progressive systemic scleroderma; PSS; Scleroderma; Scleroderma diffuse; Scleroderma progressive systemic; Sclerodermia diffusa seu progressiva; Scleropoicilodermia; Sclerosis systemic; SSc; Systemic Scleroderma; Systemic sclerosis; System sclerosis

Definition
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Autoimmunological multisystem fibrosis belonging to the so-called collagenoses with fibrosis of skin, subcutaneous tissue and numerous other organs (vessels, lung, intestine, skeleton, kidney, heart, liver) of varying degrees as well as obliterating angiopathy with fibrosis and obliteration of small vessels (onion skin angiopathy with intimal proliferation) with skin and organ infarctions.

Classification
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  • A distinction is made between limited and diffuse forms of progression.
  • The so-called CREST syndrome (Calcinosis, Raynaud's phenomenon, Esophageal hypomotility, Scleroderma, Teleangiectasis) is a subform of limited systemic scleroderma and is a common pattern of the disease.
  • Classification of systemic scleroderma according to disease activity:
    • Acute inflammatory (malignant) systemic scleroderma with rapid progression of sclerosis, with or without organ symptoms.
    • Chronic inflammatory (often vascular) systemic scleroderma with intermittent progression, with or without organ symptoms.
    • Aphlegmatic systemic scleroderma, with or without organ symptoms.
    • Burnt-out systemic scleroderma in its final sclerotic state, without signs of activity.
    • Systemic scleroderma without scleroderma (without sclerotic signs of skin).
  • Clinically, ADF is divided into three main types:
    • Type I: Acral type (the most frequent form with a marked vascular component).
    • Type II: Proximal ascending type with ascending sclerosis of forearms and upper arms. Involvement of the digestive tract, lungs, kidneys and liver (primary biliary cirrhosis).
    • Type III: Stem scleroderma (stem type) with centrifugal spread. Raynaud's phenomenon may be absent. Involvement of joints and internal organs (heart, kidney, digestive tract). Febrile temperatures.
  • The American Rheumatism Association (ARA) distinguishes:
    • Limited systemic scleroderma.
    • Diffuse systemic scleroderma.

Occurrence/Epidemiology
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Worldwide, common to all populations. Blacks and Hispanics are generally observed to have higher severity levels than whites.

  • Incidence: 0,5-1,5/100.000 inhabitants/year
  • Prevalence: 10-15/100,000 inhabitants/year.

Etiopathogenesis
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Systemic disease with humoral and cellular immune phenomena, e.g. depression of T-lymphocytes, occurrence of clonal T-cells (about 30% of patients), evidence of antinuclear factors.

Genetic disposition (correlation with HLA-B8, HLA-DR5), disturbance of microcirculation (initially functional, then lesions of endothelial cells), dysregulation of connective tissue metabolism (increased biosynthesis of proteoglycans and collagen) are discussed.

It is possible that stimulating antibodies against the PDGF receptor (= receptor of the "Platelet-Derived Growth Factor" [see also growth factors and tyrosine kinases]) as well as an abnormal TGF-beta signal transduction play a role; this would explain the clinical effect of tyrosine kinase inhibitors. Furthermore it could be shown that in the skin of patients with systemic scleroderma the chemokine CCL13 is increasingly expressed. Also the serum level of this chemokine (diagnostic marker) is increased.

Manifestation
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Usually occurring between 30 and 50 LJ. Women are 10-15 times more frequently affected than men.

Clinical features
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Prodromes: Uncharacteristic disturbance of the general condition, acrocyanosis, livedo racemosa, sensitivity to cold in the cold season. Raynaud's phenomenon occurs in 60-90% of cases, often as the first symptom of PSS. Type I is also referred to as a limited form, types II and III as diffuse forms (in Anglo-American literature).

Skin symptoms: acra: Raynaud's syndrome (anamnestic, possibly cold provocation), acrosclerosis(sclerodactyly), oedematous swelling, hyperpigmentation, spatter-like scarring, sclerosed cuticle, pathological capillaries in nail fold dermatoscopy (pathological structures and reduced density <7 capillaries/mm), teleangiectasia, haemorrhages. In the late stage, the most impressive features are tight atrophy, sclerosis (= Hoick-Gottron sign), bending contractures with formation of a claw hand, nail dystrophies as well as extremely therapy-resistant digital ulcers (so-called rat bite necrosis occurs in about 50% of patients) and circumscribed calcinosis.

Face: Amimia, facial reduction (comparison with previous pictures), pointed nose, narrow lips (microcheilie), microstomy (measurement of the mouth opening), radial mouth wrinkling, frenulum sclerosis, atrophic smooth tongue. Stem: sclerosis, coarse edema, telangiectasia, hypopigmentation and hyperpigmentation.

Other: poikiloderma (scleropoiciloderma), ulcerations, alopecia, calcinosis, atrophy of skin appendages, mucous membrane changes (sclerotherapy of genital mucous membrane).

Panniculitis: Is rarely diagnosed, but is relatively often detectable with subtle inspection of the patient.

Involvement of internal organs:

Digestive tract (in 90%): mainly oesophagus, stomach, small and large intestine.

Lungs (in about 70%): pulmonary fibrosis, pulmonary hypertension with clinical signs such as dyspnoea, cough, cyanosis. The triad with dyspnoea, quasi normal chest x-ray and unremarkable lung function is typical. If there are complaints of unspecific symptoms such as rapid decrease in performance, chest pain, dizziness or syncope, a careful diagnosis should be made: echocardiography is the screening method of choice and also excludes left ventricular dysfunction or heart disease. The ECG is pathological in only about two thirds of the patients. Radiological: In more than 90% of the cases a dilated pulmonary segment and a widening of the descending right pulmonary artery are detectable. The peripheral vessels are often rarefied. With Doppler echocardiography pulmonary hypertension can be confirmed and relatively well quantified. Angiography and cardiac catheterization should be reserved for specialized centers to plan the optimal therapy.

The concordant occurrence of systemic sclerosis and silicosis (see also silicosis and comorbidities) is of expert opinion.

Larynx: Hoarse, husky voice.

Heart: Cardiac myopathy.

Kidney: proteinuria (careful control of urine status with microelectrophoresis!), renal insufficiency, hypertension.

Eyes: cataract.

Locomotor system: osteolysis, osteoporosis, cystic lightening, arthralgias, tendovaginitis.

Muscles: weakness, pain.

S.a. Thibièrge-Weissenbach syndrome, CREST syndrome.

Laboratory
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Nonspecific signs of inflammation are present.

Rheumatoid factors are positive in 20 to 30% of cases.

Syphilis serology is false positive in 5% of cases.

Cold agglutinins are detectable in 25% of cases. Leukocyturia, erythrocyturia or proteinuria may also be present. Electrophoresis also shows elevated gamma immunoglobulins.

Antinuclear antibodies (ANA) are positive in approximately 94% of cases.

Autoantibodies may be detectable against Scl-70 (topoisomerase I, 36-70% positive), centromeres (ACA) (up to 70%), PM-Scl (approx. 3%), fibrillarin, Ku (up to 50%, indicating overlap syndrome), RNA polymerase I (8%) or single-stranded DNA (4%).

The significance of functional autoantibodies has not yet been clarified.

Histology
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  • Early stage: Discrete perivascular and periadnexial (especially around the eccrine sweat glands) and diffuse round cell infiltrates with lymphocytes, plasma cells and few macrophages. Edema of the dermis. Only minor dermal fibrosis. The epidermis and the vessels of the dermis are inconspicuous.
  • Late stage: The dermis is widened, vessels are rarified. Perivascular round cell infiltrates are visible in the deep dermis and the adjacent subcutis. Homogenized broadened collagen fibre bundles run parallel to the skin surface. The adnexa are atrophic, the vessels are narrowed in a slit-like manner. Eccrine sweat glands are walled in.
  • Adipose tissue: Primary septal panniculitis, which may spread to the periphery of the lobules, resulting in the picture of a mixed septal/lobular panniculitis. The septa are edematous, dilated and infiltrated mainly lymphoid cells. The subcutaneous fatty tissue is partially reduced at the expense of the spreading connective tissue.

Diagnosis
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Clinical, histology, chest X-ray, esophageal manometry and scintigraphy, lung function, ECG, cardiac echo, upper abdomen sonography, EMG, skeletal scintigraphy.

Differential diagnosis
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  • Circumscript scleroderma (Morphea) especially when it appears as generalized circumscript scleroderma (absence of serological autoimmune phenomena and absence of organ involvement).
  • Generalized lichen sclerosus et atrophicus (histology is differentiated; no serological autoimmune phenomena; no systemic infestation).
  • Autoimmune diseases that may be associated with sclerosing of the skin. These include: dermatomyositis; lupus erythematosus, systemic (always serological autoimmune phenomena detectable; histological and immunohistological differentiation is important). With SLE and dermatomyositis overlapping clinical pictures are observed(overlap syndrome).
  • Of great differential diagnostic relevance is the group "scleroderma-like diseases", the pseudoscleroderma.
  • Nephrogenic systemic fibrosis (after administration of gadolinium-containing contrast agents)
  • Scleroderma-like clinical pictures due to chemical noxae (vinyl chloride, silicon dioxide - see also silicosis)

Complication(s)
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Risk factors for organ involvement (according to WHO):
  • Pulmonary arterial hypertension (PAH)
  • HIV infection
  • Appetite suppressants (Aminorex, Fenfluramine, Dexfenfluramine)
  • Female sex
  • Portals hypertension in liver cirrhosis
  • Proteinuria (acute nephrogenic crisis)
  • Congenital left-right shunt
  • L-tryptophan intake
  • Thyroid diseases
  • Pregnancy
  • Systemic hypertension
  • Chemotherapeutics
  • Metamphetamine and cocaine.

Therapy
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Inflammatory PSS with rapid progression (BSG, ANA, rheumatoid factor pos., polyarthritis):

  • Immunosuppressive therapy: Basic therapy is the immunosuppressive combination therapy with glucocorticoids and azathioprine. Prednisone (e.g. Decortin), initial 75-100 mg/day i.v., maintenance dose 5.0-10.0 mg/day p.o., or Cloprednol (e.g. Syntestan) continuous dose of 1.25-2.5 mg/day p.o. are combined with Azathioprine (e.g. Imurek) 1-2 mg/kg bw/day.
  • In case of further disease progression exchange of azathioprine against Ciclosporin A (Sandimmun) 2.5-5.0 mg/kg bw/day. Cave! Strict monitoring of kidney function!
  • Alternative or complementary: Extracorporeal photopheresis can be used as a complementary procedure with few side effects (method is only used in a few centres). As an immunomodulating procedure, this therapy shows encouraging individual results.
  • Alternative or complementary: Plasmapheresis: Is primarily used for the detection of circulating immune complexes or high-titre antibodies.
  • Experimental: Encouraging results are reported from the use of the tyrosine kinase inhibitor Imatinib (Gleevec). It is possible that this effect is due to the blockage of the PDGF (= Platelet Derived Growth Factor, see below) or its receptor.
  • Colchicine has no safe value; neither does potassium paraaminobenzoate (Potaba Glenwood). Less active PSS in the sclerosing final state: No generally valid therapy modalities can be listed for this PSS which has already been running for years or decades. The therapy depends on the respective organ symptoms and must be individually adapted to these.
  • Clinical trials:
    • Individual case reports are available on positive effects of tocilizumab, an Il-6 receptor AK. A phase II study is currently being conducted.
    • Serotinin: In a proof of concept study the 5-HT 2b-receptor antagonist terguride was tested. Results remain to be seen.
    • Stem cell transplantation: In severe rapidly progressive systemic scleroderma, myeloablative haematopoietic autologous stem cell transplantation is a worth considering option. In a study with 36 patients, stem cell transplantation proved to be superior to cyclophosphamide (Sullivan KM et al. 2018).
    • Soluble guanylcyclase (sGC): The sGC stimulator riociguat follows a dual principle: The treatment of the vascular and fibrosing components (Distler O et al. 2017).
    • Lysophosphatidic acid (LPA): LPA acts profibrotically via LPA1 receptors. In a phase IIa study the effect of LPA1-AK on organ fibrosis is being investigated.

Arterial occlusive disease:

  • Early infusion of prostaglandin E (e.g. prostavasin) intra-arterial (1-2 times/day 10 μg Alprostadil in 25 ml physiological saline solution for 60-120 min.) or intravenous (40 μg Alprostadil in 50-250 ml physiological saline solution for 2 hours 2 times/day).
  • Alternatively, iloprost (ilomedin) 0.5 ng/kg bw/min i.v. can be administered over 6 hours 21 days. In the case of radiographically proven, circumscribed stenoses of the ulnar and radial arteries, the constricted section of the vessel can be bridged or replaced by a piece of vein or a replacement vessel made of special plastic.

Fingertip necroses (FKN):

  • In the case of uncomplicated FKN dry wound dressings; in the case of infected FKN polyvidon-iodine ointment dressings, if necessary internal antibiotics after antibiogram. In individual cases with pronounced keratotic marginal wall formation of ulcerations, hydrocolloidal wound dressings are indicated (e.g. Varihesive extra thin can be adapted very well to fingertips, is self-adhesive).
  • Therapy of choice: Bosentan. Results of the RAPIDS-1 study (level of evidence Ib) show a positive effect on digital ulcers (improved healing rate, lower rate of new necroses compared to the placebo group). Therapy of the 1st choice!
  • Alternative: Even under iloprost (dosage see above) there is a reduction of digital ulcerations.

Calcinosis of the skin:

  • Frequently found in the area of the fingertips or above the finger joints. Millet grain-sized, painful, rough, skin-coloured or reddened nodules.
  • Conservative approach: etidronic acid, coumarin derivatives, minocycline (50-100 mg/day p.o.).
  • Surgical: Removal in LA; curettage if superficially positioned, excision if deeply positioned to protect the tissue. Good results can be achieved with the CO2 laser. Dry wound dressings. S.a.u. CREST syndrome.

Raynaud's symptomatology:

  • Calcium antagonists: Nifedipine (e.g. Adalat) 1-3 times/day 5-10 mg p.o. (low initial dose, as headaches often occur in the adaptive phase); find individual maintenance dose!
  • Alternatively pentoxifylline (e.g. Trental) 0.4-0.8 g/day i.v., later 0.4-0.6 g/day p.o., possibly in combination with nifedipine (results are not very convincing!).
  • Alternative: Low-dose (0.1-0.3 g/day p.o.) acetylsalicylic acid (e.g. Aspirin protect 100). It makes sense to apply ointments containing nitrate locally (e.g. Isoket ointment, 1-2 g evenly distributed on both hands).

Carpal tunnel syndrome:

  • Symptoms: First symptoms are the "painful falling asleep of the hands, especially at night. In advanced cases, persistent numbness of fingers 1-4 and problems with fine handwork are added. Clarification and therapy by neurologists.

Sicca symptoms: see below Sjögren's syndrome.

Changes of the stomatognathic system:

  • Consistent prophylaxis and maintenance therapy; in the case of dentures in the form of crown and bridge prosthetics, exact analysis of the periodontal situation of the abutment teeth. Changes of the periodontal system in PSS. Difficulties with suction dentures often persist due to pronounced atrophy of the jaws. The aim is to achieve a dental prosthesis by inserting implants.
  • Microstomy: If food intake is considerably more difficult, a plastic surgery of the corner of the mouth can provide relief. This procedure, known from burn surgery, involves an incision of several millimetres at both corners of the mouth. The red of the lips can be reconstructed by protruding the oral mucosa outwards (vermillon plastic surgery).

Hypertensive circulatory situation:

  • ACE inhibitor (e.g. captopril or tensobone) 25-75 mg/day p.o. Alternatively: α receptor blocker e.g. prazosin (Duramipress) 10-15 mg/day p.o.
  • Proteinuria: Early use of ACE inhibitors (e.g. Captopril or Tensobon) 25-75 mg/day p.o.

Pain:

  • For chronic pain, especially joint pain, administration of NSA such as acetylsalicylic acid (e.g. aspirin), metamizole (e.g. Novalgin) or paracetamol (e.g. Ben-u-ron). If the pain symptoms are severe, switch to weak opioids such as tramadol (e.g. Tramal) or dihydrocodeine (DHC).

Pulmonary hypertension:

  • Patients with pulmonary hypertension in scleroderma have significantly elevated endothelin levels. Previous therapy with conventional vasodilators and anticoagulants has been effective in only a few patients. Synthetic prostacyclin analogues are more effective in both primary pulmonary hypertension and secondary pulmonary hypertension due to systemic scleroderma. Good effects are achieved with the endothelin receptor antagonist bosentan.

Pulmonary fibrosis:

  • Studies conducted so far with cyclophosphamide (1-2 mg/kg bw/day) have not shown satisfactory results. Subjective symptoms such as dyspnoea are said to have improved underneath.

General therapy
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The therapy of PSS basically depends on the acuteity of the disease (see Table 2) and the type of organ infestation. It consists of a combination of system therapy, local therapy, physiotherapy and psychotherapy.

Radiation therapy
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UVA1 whole-body irradiation has proven successful in both inflammatory accentuated and fibrosed scleroderma. In addition to the anti-inflammatory effect, the induction of collagenase softens existing fibroses. See below phototherapy.

Progression/forecast
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Mostly protracted, 5-20 year course, with low self-healing tendency. In type III: unfavourable prognosis, death often occurs within 3-5 years. Acute malignant variant (rare): death within a few months (malignant scleroderma).

Naturopathy
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  • Physiotherapy: To be carried out only by physiotherapists who are familiar with PSS!
  • Stimulation or reflex therapy: Has become increasingly important in recent years. Acupuncture is indicated as an additional method for chronic pain.
  • BMS ( biomechanical stimulation ): The method of biomechanical stimulation leads to a loosening of the musculature in addition to an increase in peripheral blood circulation and can be usefully applied here in scleroderma. The effectiveness of this therapy is currently not scientifically proven and is not covered by health insurance.
  • TENS (transcutaneous electrical nerve stimulation): excitation of peripheral nerves by electrical skin stimulation, to activate various pain-inhibiting mechanisms.
  • Physiotherapy: The aim is to counteract contractures and tendencies to shrinkage of the muscle and tendon apparatus and to avoid stiffening of joints.
    • Active physiotherapy: The patient's own activity and initiative are required.
    • Passive physiotherapy: Passive stretching, e.g. of muscles and joints.

Note! Strictly avoid painful exercises! No isometric exercises (involving exertion)!

  • Massages. Massage principle for PSS: start therapy very carefully, increase intensity slowly, maintain therapy regularly over a long period of time.
  • Heat therapy, sauna

Tables
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Progressive systemic scleroderma ? Organ involvement

Clinic

Involvement

Skin changes

> 95%1

Oral mucosa and dental organs

> 90%

Circulatory disorders

> 90%

Immune system (autoantibodies)

> 90%

Esophageal motility disorders

70-80%

Arthritides

40-60%

Pulmonary fibrosis

40-60%

Cardiac involvement

30-40%

Muscle involvement

10-20%

Intestinal involvement

< 10%

Kidney involvement

< 10%

Liver involvement (PBC)

> 5%

Practical tools for collagenosis patients

Article

to be obtained from

Heat pads for body, shoes, gloves (disposable articles)

Frankonia Hunting, Randersackerer Straße 3-5, D-97064 Würzburg, Germany

Pocket stove (with lighter fuel)

Patent pocket stove (with solid fuel)

Heatable Thinsulate gloves, battery operated

Heatable socks, battery operated

Shoe warmer, insert warms shoes before putting them on

Shoe heating system, sole with heating elements, heated shoes while wearing, battery operation

Thermo Soles Feldgasse 34 7072 Mörbisch am SeeAustria
, https://thermosoles.eu

Heat and cold packs (Lavatherm), can be used as often as desired; different sizes. Lavatherm contains liquid sodium acetate, which is transformed into a solid state by bending an activator rod. During this process the packing heats up to about 54 °C.

Helga Kandora, Bergschlagweg 38, D-46569 Hünxe

Ruhewohl warmth cushion, for multiple use

Sissel company

Viscoped foot insole, silicone foot insole for pressure relief

Bauerfeind GmbH & Co, Arnoldstr. 15, D-47906 Kempen, Phone: 02152-2080, Fax: 02152-52 36 3

Practical tips for the scleroderma patient in daily life

Article

to be obtained from

Writing aid, triangular handle extension which is put over pen, standard and oversize

Lehrmittelhaus Riedel GmbH, Unter den Linden 15, D-72762 Reutlingen, Phone: 07121-515350, Fax: 07121-370143

Universal opener, for opening bottles, glasses and crowns with limited mobility of the hands

Medication box, facilitates removal of small tablets/capsules

Bottle open er (especially for screw caps)

Merck Company, Frankfurter Str. 250, D-64293 Darmstadt, Germany

What the scleroderma patient should pay attention to

  • Avoid cold steering wheel covers (e.g. made of plastic or leather); use wool or fur covers instead

  • Avoid handling frozen goods (freezer); put on household gloves if unavoidable

  • Do not wash dishes cold with unprotected hands; dishwasher is best

  • Discuss the specifics of the illness with the employer; if possible, always take intervals of rest.

  • If possible, avoid all work with an increased risk of injury (e.g. household chores with knives and scissors, handicrafts, heavy manual work).

Physiotherapeutic measures for PSS

Applications to increase peripheral blood circulation (the prescriptions are to be understood as serial applications) *

Carbonic acid baths (as partial or full baths)

Two- or four-cell baths (electrotherapeutic application with direct current, intensity: 5-15-25 mA, treatment time: 12-18-24 min.)

Whole-body heat applications, e.g. sauna (contraindications: severe heart and lung diseases); after sauna, short cold applications, e.g. cold showers, are well tolerated (recommended as vascular training)

Indication: sclerotic non-inflammatory-oedematous phase of PSS

Examples of physiotherapeutic exercises for PSS

  • Training of vascular regulation of arms and legs (Ratschow exercises)

  • Stretching exercises of the hands (spreading the hands, drawing contours with a pencil, stretching beyond the drawn contour)

  • Kneading exercises of the hands (squeeze soft sponge or rubber ball with the hands)

  • Stretching exercises of the wrists: press hands together in prayer position

  • Raise elbows, take a book in hand and rotate wrists as far as possible

  • Breathing therapy with stretching positions and thorax massage

Massages for SSc

  • Large-surface stroking and light shifting of the skin

  • Small-scale shifting of the skin against its support (e.g. with a fingertip)

  • Attempt to lift the skin folds from the support

  • Underwater massages (especially for patients with muscle complaints)

  • Connective tissue massages: Here the non-sclerotic skin is treated with strong massaging grips.

Heat therapy for acral circulatory disorders

  • Warm up a bowl with about 50 g of hulled millet for about 1 min. in the microwave, knead the warm millet with your hands for 5-10 min.

  • Warm the bowl with warm sand in the oven. Hands and feet can be bathed and moved in the warm sand.

  • Mud or silt packs (applications possible at home). Medium pre-made in glove or boot form.

  • Paraffin baths: use of warmed paraffin for hand and foot baths.

  • Whole body treatments, e.g. infrared hyperthermia.

Psychotherapeutic measures for PSS

Conversational and behavioural therapies

  • Biofeedback

  • Active relaxation methods such as autogenic training

  • Progressive muscle relaxation according to Jacobson

  • NLP (neurolinguistic programming) as pain processing and coping therapy

Rules of thumb for a wholesome diet (half-pound rule according to Kluthe)

Food

Daily

½ pound of potatoes

½ pound of bread and other whole grain products

½ pound of vegetables

½ pound fruit juice

½ pound of milk or sour milk products

Weekly

½ pound of meat or fish

½ pound of sausage products

½ pound of cheese

½ pound eggs (4-5 pieces)

½ pound of fat (100 g butter, 100 g margarine, 50 g oil)

½ pound rice or pasta

½ pound of cereal grains (whole or ground) and cereals

½ pound of honey or jam and other sweets

Diet/life habits
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  • Practical behavioural measures for scleroderma patients:
    • Absolute ban on nicotine!
    • Clothing: Warm and airtight clothing, always wear warm gloves in transitional weather, no tight shoes (prolonged pressure reduces circulation; danger of general reduced circulation of the feet and ulceration at pressure points).
    • Showering/care: Long, warm showers in the morning, short cold interval showers are possible (vascular training). Apply oil/water emulsions to the moist skin (Sebamed Lotion, Bepanthen Lotio etc.). Sauna sessions- not too hot, cave heart involvement - and short cooling down.
    • Nail care: Cutting nails may be difficult due to hardening of finger and toe nails; bathe hands and feet with warm soapy water for 10 minutes before cutting nails, then cut carefully. Avoid injury.
    • Oral hygiene: Careful dental hygiene, clean teeth with a soft toothbrush.
    • Face: Teleangiectasias in the area of the face can be covered with a covering make-up (e.g. waterproof Dermacolor, Stiefel company); if necessary laser therapy (e.g. with pulsed dye laser, argon laser) or sclerotherapy.
    • Living rooms: Adequate temperature, with tendency to overheat. Do not place the bed on the weather side. Do not lay cold floors (stone floors), carpet floors are recommended. Use temperature-controlled waterbeds. If possible, move to ground floor flats or flats where lifts are available.
  • Sports: Endurance sports (light jogging) without acute overload are recommended (no constricting sportswear), swimming in well-tempered pools, gymnastics in well-heated rooms, dancing. Sports that can lead to injuries (judo, football, handball and volleyball) or sports that lead to unavoidable cooling of the skin (horse riding, sailing, etc.) are not recommended.
  • Self-training: In case of microstomies, slowly stretch the mouth opening with the fingers, practice whistling, speech training (loud, clear reading aloud), tense and relax facial muscles (make grimaces). In the case of motor disorders of the fingers, fine gardening, plucking weeds, training tactile skills (handicrafts, carefully kneading plasticine).
  • Vacation: Vacation in warm southern countries is recommended (avoid blazing sun!), vacation in the local winter period (principle: shorten cold period in the own country), active vacation with physiotherapeutic applications (e.g. thermal baths). Winter sports are not recommended because of the risk of chilling.
  • Occupation: Not recommended (as triggering by specific occupational noxae cannot be excluded): Mining (exposure to stone dust), work in quarries and sandblasting (exposure to stone dust), work with jackhammers or other vibrating machines (activation of vascular symptoms), work in occupations that lead to constant cooling of the skin (e.g. butchers, florists), painting occupations (organic solvents), dental laboratories (exposure to plastic dust when grinding or polishing dentures), handling liquid PVC and similar plastics (PVC disease).
  • Sexuality: For people with scleroderma, sexuality is an unjustly neglected topic. Scleroderma sufferers tend to have a low sense of self-respect due to their altered appearance. Sexual relationships begin with social relationships. To appear interesting oneself, one must be interested in others. A positive relationship with one's own body will help the sufferer to establish and maintain relationships with others.
    • Sexual intercourse: It is recommended to experiment with different techniques and methods; prefer lateral positions during sexual intercourse.
    • Scleroderma patient should be active partner in sex.
    • Joint complaints: Before sexual intercourse take a non-steroidal anti-inflammatory (e.g. 20 trp. Novalgin), warm bath; important is a warm bed and a warm room!
    • Women with sicca symptoms: use of water soluble lubricants (e.g. vaginal gel pH5 listed under NRF 25.3. is recommended.
  • Nutrition:
    • Eat varied but not too much (as many different foods as possible).
    • Eat spicy but not salty foods.
    • Easily digestible food; no flatulent foods (cabbage, legumes).
    • No constipating foods such as chocolate or bananas.
    • Avoid large portions; rather eat in small amounts and more frequently.
    • Always drink plenty, at least 1.5-2 l/day (mineral water, diluted fruit or vegetable juices; tea and coffee in small quantities).
    • Whole grain products are recommended.
    • Foods that are easy to chew and swallow, e.g. no dry bread rolls or dry meat; remove the crust from bread.
    • High protein and vitamin content.
    • Plenty of potatoes, fruit and vegetables.
    • Alcohol is allowed in small quantities (e.g. 1 glass of red wine, sparkling wine or beer with dinner).

Phytotherapy external
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Official rosemary oil (see below Rosmarinus officinalis leaf oil) has been proven to have a caring effect on the skin as well as a distinct vasodilatory (warming) component and can be recommended as an additive therapy (von Schoen-Angerer T et al. 2018).

Note(s)
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  • The connective tissues of the lungs, kidneys, esophagus, and heart are considered particularly at risk. Lung involvement is now the most common cause of death in systemic scleroderma. Scleroderma is not curable, but the course of the disease can be slowed or stopped with medication and specialized rehabilitation.
  • Notice. The former name"progressive systemic scleroderma" has therefore been changed in favour of the current name"systemic scleroderma".

  • Diagnosis and treatment of scleroderma require special medical experience with these diseases. The Ministry of Research and Technology (BMBF) has therefore named clinics and centres in Germany with sufficient expertise in this disease as part of a research programme.
  • It is recommended to join a self-help group to exchange practical experience for everyday problems ( http://www.sklerodermie-selbsthilfe.de/startseite.html).
  • In ANA-negative patients (about 10% of scleroderma collectives), males are in the majority, showing lower levels of skin sclerosis and vasculopathies, and increased frequency of malabsorption. Pulmonary involvement including pulomonal hypertension as well as mortality rates are comparable to the ANA-positive group.

Literature
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  1. Altmeyer P et al: Working group on scleroderma of the Arbeitsgemeinschaft Dermatologische Forschung (ADF) (1986) Clinic of progressive systemic scleroderma (PSS). Dermatologist 37: 320-324
  2. Baroni SS et al (2006) Stimulatory autoantibodies to the PDGF receptor in systemic sclerosis. N Engl J Med 354: 2667-2676
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Last updated on: 16.10.2021