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 (in Danish))
- 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
Uncertain diagnosis?
Norwegian Directorate of Health (Supervisor for referrals)
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