Disease history
- Debut Age 10-40 Year (80%)
- Pulmonary symptoms
- Irritant cough
- Dyspnea
- Pain in the lung area
- General health status
- Night sweat / some fever
- Exhaustion
- Weight Loss
- The eye (5-20%) (Eyelid)
- Anterior uveitis (iridocyclitis or iritis)
- Posterior uveitt (chorioretinitt)
- Retinal vasculitis
- Keratoconjunctivitis
- Conjunctive follicles
- Skin (25%)
- Erytema nodosum
- Subcutaneous lesions (often in the throat / face and in the old scars)
- Growing, pink knots
- Red well-defined rash
- Enlarged lymph nodes
- Hepatosplenomegaly
- Heart failure and / or rhythm disturbances
Examinations
- Auscultation of lungs
- Crepitations over the lungs
- X-ray examination or CT examination of lung
- Hilus lymph nodes
- Knots and opacities
- Frosted glass in lung tissue
- Traction brochiectation
- Cysts
- Fibrosis
- Lung function tests including DLCO
- PET / CT can map different organ affection
- Heart
- Auscultation
- ECG
- MRI examination of the heart with contrast agent (gadolinium)
- Echocardiography (left ventricular failure with EF <40%)
- Granuloma of proximal septum (may be misinterpreted as hypertrophic cardiomyopathy), atrial graumomas may resemble myxoma (Reference Vikse J, 2018)
- Blood tests
- Blood sedimentation rate (ESR) and CRP (moderate increase)
- Leukocytes (Leukopenia 5-10%)
- ACE (high in generalized disease)
- Reference area (adult 20-100, child 9-13 years up to 40% higher: 20-140 at OUS laboratory)
- IgG (low at 80%)
- Calcium (increased)
- Calcium in urine (increased)
- Alkaline phosphatase (increased) and liver enzymes
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