| Idiopathic Pulmonary Fibrosis
| Diffuse Parenchymal Lung disease
| Asbestos Related Lung disease
| Coal Worker's Pneumoconiosis
| Farmer's Lung
| Cryptogenic Organising Pneumonia (COP-BOOP)
| Extrinsic Allergic alveolitis (Hypersensitivity)
| Cor Pulmonale
Staging is really all based on the CXR findings. Many need no treatment. Topical steroids when possible.
- Non caseating granulomatous lung disease
- Protean manifestations affecting many organs
- Blacks > whites. Females >Males. Onset Age 20-40
- Cutaneous anergy - lack of a skin reaction to various antigens e.g. tuberculin
- Diminished cell-mediated immune response to antigens
- Reduced lymphocytes and low circulating T cells as they are sequestered in lungs
- Mildly increased B cells in peripheral blood and increased bronchial CD4 cells
Clinical- There are broadly 2 different presentations
- Acute Disease with a good prognosis with Erythema nodosum, Arthralgia, Bilateral hilar lymphadenopathy
- Chronic - worse prognosis - Gradual increasing dyspnoea, Pulmonary fibrosis and systemic complications
Other Clinical findings
- Asymptomatic disease in 30% with only CXR changes
- Arthritis: Feet and hands and large joints may be affected too
- General: Lymphadenopathy, Hypercalcaemia in less than 10% and rarely severe.
- Eye: Early on - Anterior uveitis with blurred vision and eye pain, Later - Posterior uveitis with progressive visual loss and blindness
- Cardiac: Heart block, VT, ECG changes, Cardiomyopathy (v rare) and CCF may be seen. Consider ECHO.
- Uveoparotid fever: Heerfordt's syndrome - Uveitis + Parotids enlarged + VIIth palsy + Fever
- Lofgren's syndrome : BHL + EN + arthritis/arthralgia + fever
- Skin: 25%. Erythema nodosum, Lupus pernio (red/blue nose), Nodules, scar infiltration
- CNS: Chronic granulomatous meningitis, Fits, Peripheral/cranial neuropathy
- Endocrine: Hypothalamic lesions - diabetes insipidus and Hypercalcaemia
- Haematological: Generalised lymphadenopathy, low CD4 count
- Liver: granulomas on biopsy and mild deranged LFT's
- Bone: cystic lesions can develop
- Renal:interstitial nephritis, Glomerulonephritis, Renal stones (high Calcium)
- Often the patients are female of childbearing age and pregnancy usually improves symptoms.
- Tuberculosis - check smear for open TB and tuberculin testing
- Lymphoma - do HRCT
- Lung cancer with localised spread
- The ESR and CRP may be elevated with active disease
- Raised serum ACE: from epithelioid cells of the granulomas
- Raised 1,25(OH)2 D3
- The Calcium (Blood and urine) is elevated in only 10%
- Tuberculin tests are usually negative
- Bronchoscopy - Cobble stoning of mucosa and lavage shows an increased CD4:CD8 T cell ratio
- Transbronchial Biopsies - Non-caseating granulomata on biopsy
- Liver Biopsies - Non-caseating granulomata on biopsy
- ECG or 24 hr tape: heart blocks
- Kveim test was formerly used and was an injection of splenic extract from a sarcoid patient injected intradermally and then biopsied but now cannot be done with risks of infections.
- Exclude open (smear-positive) TB before formal lung function tests or risks contaminating the equipment.
- Reduced transfer factor, Restrictive (small lung) PFT's and reduced DLCO
- High-resolution CT can show the extent of the fibrosis - ground glass appearance suggests active disease which may be steroid responsive
- Gallium-67 scan: The "panda sign" (localization in the lacrimal and salivary glands, giving a typical panda appearance to the face) is suggestive of sarcoidosis
Staging is really all based on the CXR findings
- 0: CXR normal bit may have EN and arthralgia. Most cases resolve in 1 year
- I: BHL with no lung infiltrates 80% resolve spontaneously. EN and arthralgia.
- II: BHL + pulmonary infiltrate 50% resolve spontaneously. Breathlessness and cough.
- III: No BHL but Pulmonary infiltrates 10-25% resolve
- IV: Advanced fibrosis with evidence of honeycombing, hilar retraction, bullae, cysts, and Emphysema. 0% resolve. Cor pulmonale
- Good: younger, erythema nodosum
- Poor: Age > 40, Afro-Caribbean, Continuous symptoms over 6 months, Lupus pernio, Involvement of over 3 organs, CXR stages III-IV,
- Manage joint pains with analgesia and NSAIDS and even a short course of steroids. No treatment is needed for simple BHL e.g. Lofgren's syndrome except NSAIDs as analgesia for joint involvement. Simple follow up is all that is required.
- Confirm disease with least invasive biopsy. Steroids are given for active disease e.g. Prednisolone 20-30 mg od for 6 weeks and then a reducing dose. Indications are progressive lung disease based on symptoms and/or radiology and falling TLCO, Hypercalcaemia, Other organ damage (Eyes, CNS, Liver, Heart). Topical steroids for uveitis.
- More severe diseases may need Methotrexate (10-20 mg/week) or Azathioprine (50-150 mg/day) and anti-TNF alpha inhibitors.
- Thalidomide, Chloroquine and Hydroxychloroquine also used for cutaneous disease
- Hypercalcaemia usually respond to a course of steroids. These decrease 1,25(OH)2D production and restore the calcium concentration to normal.
- Lung transplantation in severe cases with progressive lung fibrosis