ICD-10: M34.0 (diffuse form) M 34.1 (Limited Crest Form)
Contents
Diagnosis is based on
- Disease history
- Medical Examination Findings
- Antibodies
Disease history
Time of symptom debut (Raynaud's phenomenon and other symptoms) and for diagnosis
* (Star) marks the symptoms and research findings that are included in ACR-EULAR Criteria (2013):
Symptoms
- *Raynaud's phenomenon (three-phase)
- Non-Raynaud's manifestations
- *Sclerodactylia (hard skin on fingers)
- Proximal / distal for MCP joints
- *"Puffy Hands" (Fingers)
- *Ulcera or pitting scars (substance defect) at the fingertips
- * "Pitting scars" (scars and scabs) after sores on fingerpulpa
- *Telangiectasia (limited form)
- * Capillaroscopy pathological
- * Pulmonary hypertension
- *Interstitial lung disease (ILD)
- *Antibodies CENP (restricted form) or Scl70 / topoisomerase (diffuse form), RNA polymerase III (risk of kidney / renal crisis)
- Other disease manifestation
- Gastro-oesophageal reflux
- Ingestion (esophagus, dysmotility)
- Breathing Problems
- Heavy breath at load
- Dry Cough)
- Calcinate subcutaneously (limited form, CREST) (other calcinesis causes here)
- Hard skin proximal for elbows / knees or on the chest, stomach or back (systemic sclerosis, diffuse form)
- Abdominal pain and / or low hemoglobin (anemia), GAVE (gastral anterior ventricular ectasia) (by diffuse form of systemic sclerosis)
- Fecal pattern (bacterial overgrowth)
- Fecal incontinence
- Renal crisis with malignant blood pressure increase and increasing kidney failure (systemic sclerosis, diffuse form)
- Dry eyes, mouth or vaginal (secondary Sjögren's syndrome)
- Dry cough, stress dyspnoea (lung involvement)
- Malabsorption, diarrhea, fecal incontinence.
- Weight Loss (Kg) Last 3 Months?
- Treated with Nifedipine (Adalat), Proton pump inhibitor, Sildenafil (Revatio, Viagra), Bosentan (Tracleer) and / or immunosuppressive drugs mycophenolate (CellCept), cyclophosphamide (Sendoxan), HMAS
Clinical examination
- Blood pressure and weight
- Heart
- Auscultation
- Lungs
- Auscultation
- Edema or dyspnoea (functional failure: heart (Pulmonary hypertension), lungs, kidneys)
- Abdomen meteoristic (bacterial overgrowth)?
- Skin
- Visible Raynaud's phenomenon
- Sclerodactylia
- Hard, tight skin on your fingers
- "Puffy Hands"
- Dense, diffuse swollen fingers
- Diffuse form
- Proximal distribution of elbows and knees, truncus, face and throat
- Tendon friction rub (late-onset, diffuse form)
- Limited form
- Spread on hands, maximal up to elbows and feet maximum up to knees
- Tight and wrinkled around the mouth (Carp Mouth)
- Telangiectasia
- Calcinosis (X-rays of hands?)
- Rodnan skin score
- Visible Raynaud's phenomenon
- Ulcera or pitting scars (Substance defect on fingertips)
- Dryness disorders from the eyes and mouth (Secondary Marine Border Syndrome)
Supplementary medical investigations
Radiological examinations
- X-ray examination of the esophagus (dynamic with ingestion of contrast agent) or manometry (dysmotility, stenoses, reflux)
- CT examination / HRCT of lungs (milk glass, fibrosis or extended pulmonary artery (more than 3,5cm) at suspected Pulmonary hypertension)
- More than 20% lung tissue infection suggests serious lung involvement
- MRI of the heart
- Can detect fibrosis in the heart muscle
Lung function tests
- Lung function tests (FEV1, FVC, DLCO% of expected)
- Severe lung manifestation: FVC less than 70% and CT examination show more than 20% attacked lung tissue. Similarly, if FVC and DLCO fall by 10% and 15%, respectively, each year
- Immunosuppressive therapy with cyclophosphamide or mycophenolate should be considered
- Severe lung manifestation: FVC less than 70% and CT examination show more than 20% attacked lung tissue. Similarly, if FVC and DLCO fall by 10% and 15%, respectively, each year
Capillaroscopy
- Mega capillaries, bleeding, "bushy" capillaries
6-minute walk test
- The distance traveled during the 6 minute maximum, but even times
- Can be done in a corridor with a measured distance of trained health personnel with emergency medical access
- Registration of oxygen saturation and Borg's Dyspne Index (scale 0-10) before and immediately after the test
- Reference: ATS statement: guidelines for the six-minute walk test
Echocardiography
- Pulmonary hypertension: In echocardiography estimated pressure over 35mmHg (systolic)
Cardiac examination
- Pulmonary hypertension: Medium pressure at right cardiac cat examination shows above 25mmHg
Blood tests
- Antibodies
- ANA, ENA
- CENP / anti-centromer: limited form,
- SCL-70 / topoisomerase I: diffuse form
- PM-Scl 100 or PM-Scl 70 indicates Myositis-overlapp, Scleromyositis and (check muscle power, atrophy and creatine kinase (CK), if any) electromyography (EMG) and MRI examination of thigh muscles). Consider doing a muscle biopsy
- U3-RNP increased risk of Pulmonary hypertension
- RNA polymerase III: increased risk of renal crisis
- Creatinine in serum
- CK in serum (myositis overlap, scleroderma myositis)
Follow-up for systemic sclerosis
Referral to specialist
Local rheumatology department should be preferred. In Oslo: Rheumatology Department, Rikshospitalet: Postbox 4950 Nydalen, 0424 OSLO. The department receives patients from the Health Region South East and from all over the country when the capacity allows.
Uncertain diagnosis?
Norwegian Directorate of Health (Supervisor for referrals)
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