- Systemic (multi-system) granulomatous inflammatory disease characterized by non-caseating granulomas (small inflammatory nodules) on lung…
- Granulomas most often appear in the lungs or the lymph nodes, but virtually any organ can be affected. Normally the onset is gradual/insidious.
- Sarcoidosis may be asymptomatic or chronic and may cause death.
- Cause = idiopathic! Some causes may include = genetics, propionibacterium acnes, vit D dysregulation, hyperprolactinaemia, thyroid disease, autoimmune.
- 19 in 100,000 in UK
- Uncommon in Japan
- Bilateral hilar lymphadenopathy
- Pulmonary infiltration
- Skin and eye lesions (erythema nodosum)
- Keratoconjunctivitis sicca
- Metabolic manifestations
- Central nervous system
- o Lead to severe neurological disease
- Bone and joint involvement
- o Arthralgia without erythema nodosum seen in 5% of cases
- o Bone cysts are found
- Cardiac involvement
- o Ventricular dysrhythmias and sudden death
- o Conduction defects
- o Cardiomyopathy (dilated/restrictive) with congestive cardiac failure
- Typical sarcoid granulomas consist of focal accumulations of epithelioid cells, macrophages and lymphocytes, mainly T cells.
- There is depressed cell-mediated reactivity to tuberculin.
- There is overall lymphopenia: circulating T lymphocytes are low but B cells are slightly increased
- Bronchoalveolar lavage shows a great increase in the number of cells; lymphocytes are greatly increased (particularly CD4 helper cells).
- The number of alveolar macrophages (=’Kupfer cells’) is increased but they represent a reduced percentage of the total number of bronchoalveolar lavage cells.
- Transbronchial biopsies show infiltration of the alveolar walls and interstitial spaces with leucocytes, mainly T cells, prior to granuloma formation.
- Langhan’s cells
- Asteroid bodies
- Schaumann bodies
- Epitheloid macrophages
- o CXR
- Full blood count
- o Mild normochomic, normocytic anaemia with raised ESR
- Serum biochemistry
- o Serum calcium is raised
- o Hypergammaglobulinaemia
- Transbronchial biopsy
- o Most useful investigation
- o Positive results seen in 90% of cases
- o Pulmonary non-caeseating granulomas are found
- Serum level of ACE
- o Raised
- Lung function tests
- o Restrictive lung defect
- o Decrease in FEV1 and FVC
- Severe symptoms are generally treated with steroids, and steroid-sparing immunomodulating agents such as azathioprine and methotrexate are often used.
- The disease can remit spontaneously or become chronic, with exacerbations and remissions. In some patients, it can progress to pulmonary fibrosis and death.
- Approximately half of the cases resolve or can be cured within 12–36 months and most within 5 years.
- Where the heart is involved, the prognosis is poor
- Much more severe in American blacks – death rates are 10%
- Disease remits in 2/3rds patients with hilar lymphadenopathy alone