Sarcoidosis Flashcards
What is sarcoidosis?
- Multisystem granulomatous disorder of unknown aetiology
- Characterised by pathological non-caseating granulomas in involved organs.
What is the epi of sarcoidosis?
~10-20/100000
3-4x more in Black Americans
Age of onset: 20-60y
Rare in Asia:
Spore – 0.56/100000, predominantly Indians (49.2%), bimodal age distribution
What is the mortality risk and complications of sarcoidosis?
- 5-year survival is 93–95%
- Mortality higher in individuals with more severe disease at diagnosis and if more organs are involved
- Higher risk of infection, CCF, CVA, VTE, autoimmune disease, malignancy
Complications: Aspergilloma, Pulmonary HTN
What are the Sx of sarcoidosis?
Pulmonary: coughs, dyspnoea, wheeze (if with endobronchial involvement or traction bronchiectasis)
Others: chest pain, fatigue, malaise, fever, LOW, skin lesions, visual changes, dry eyes or mouth, parotid swelling, palpitations, syncope, joint pain, muscle weakness
Children: skin rash, erythema nodosum, arthritis, uveitis, lymph nodes, hyperglobulinemia, hyperCa
What are the investigations for sarcoidosis?
Requires:
- Compatible clinical & radiographic manifestation
- Exclusion of other similar disease
Laboratory testing:
FBC: anaemia, leukopenia, eosinophilia, thrombocytopenia
Ca (noted Hypercalciuria >common than hyperCa)
Cr, ALP
Serum Vit D
Hypergammaglobulinemia
Mantoux test
Elevated ACE level but poor sensitivity and specificity
Histopathology detection of noncaseating granulomas, except in:
Lӧfgren syndrome (Erythema nodosum + bilat hilar adenopathy + fever + arthritis)
Heerfordt syndrome (uveitis + parotiditis + fever)
Imaging:
CXR
HRCT
MRI scan – extrapulmonary
FDG-PET scan -
Gallium-67 scan showing uptake in parotid and lacrimal glands (Panda sign) with right paratracheal and bilateral hilar uptake (Lambda sign)
TTE
Others:
PFT
Opthalmic exam
Cardiac Ax (ECG)
What are the CXR stages of pulm sarcoidosis?
Stage I: bilateral hilar adenopathy
Stage II: bilateral hilar adenopathy and reticular opacities
Stage III: reticular opacities in the upper zones with shrinking hilar nodes
Stage IV: reticular opacities in the upper zones with volume loss
What are the CT changes of sarcoidosis
String of beads
Galaxy appearance
What are the DDx of sarcoidosis?
Mycobacterial infection – MTB, NTM
Fungal infection – histoplasmosis, blastomycosis, PJP
Hypersensitivity pneumonitis
Pneumoconiosis
Drug-induced hypersensitivity
Pulmonary histiocytic disorders
Foreign body granulomatosis
What are the Rx for sarcoidosis?
Depending on the organ involved
Most cases do not require Rx
Initial Rx: oral glucocorticoid 4-6w, then taper the dose if improving, inhaled glucocorticoid for coughs
Refractory to steroid: MTX, Aza, leflunomide, MMF
- Consider trial of combination of 2 immunosuppression, or adding TNF-a antagonist (infliximab, adalimumab)
What are the monitoring required in sarcoidosis?
Symptomatic: 1-2 monthly
Asx: 3-4 monthly for 1st year, then yearly
In sarcoidosis showing progression and fibrosis, what should be done?
Add on antifibrotic nintedanib, and stop other meds which are not effective
Dosage of meds used in sarcoidosis
ERS 2022 Clinical practice guideline for Mx of sarcoidosis
What is Lofgren syndrome?
Erythema nodosum + bilat hilar adenopathy + fever + arthritis
What is Heerfordt syndrome?
uveitis + parotiditis + fever
What are the complications from sarcoidosis?
VTE
Aspergilloma
Pulmonary HTN
What are the adverse prognostic factors in sarcoidosis?
DDx of granuloma on lung Bx
Reference: Oxford Resp Med Handbook
- Sarcoidosis
- tuberculosis
- hypersensitivity pneumonitis
- Granulomatosis with polyangiitis (Wegener’s)
- Drug reactions
- non-tuberculous mycobacteria infection
- Fungal infections:
- Cryptococcosis
- aspergillosis
- Coccidioidomycosis * Blastomycosis
- aspiration of foreign material
- primary biliary cirrhosis
- Sarcoid-like reaction to malignancy.
How does sarcoidosis cause hyperCa?
Hypercalcemia in sarcoidosis is due to the uncontrolled synthesis of 1,25-dihydroxyvitamin D3 by macrophages. 1,25-dihydroxyvitamin D3 leads to an increased absorption of calcium in the intestine and to an increased resorption of calcium in the bone.
Indications for immunosuppressive Rx in sarcoidosis
- Increasing symptoms, deteriorating pFts, and worsening CXr infiltrates
- Cardiac sarcoidosis
- neurosarcoidosis
- Sight-threatening ocular sarcoidosis
- hypercalcaemia
- Lupuspernio
- Splenic, hepatic, or renal sarcoidosis.
BTS guidelines for the management of sarcoidosis (2008)
- Rx not indicated in asymptomatic or mildly abnormal stage I, II & III disease because of high rates of remission.
- 1st line: OCS in patients with progressive disease determined by radiology or on lung function, significant symptoms, or extrapulmonary disease requiring treatment
- prednisolone (or equivalent) 0.5 mg/kg/day for 4 weeks, then reduced to a maintenance dose which will control symptoms and disease progression, for 6–24 months
- Give high doses, such as 30 mg prednisolone/day, to control active disease. rarely need >40 mg/day. Usually give this high dose for 2–3 weeks, and then reduce if there has been a response
- Maintenance dose of around 5–20 mg to control symptoms and prevent progression of disease. Leave on this dose for a few months, and then slowly reduce steroid dose further.
- Maintain on low dose of prednisolone (5–7.5 mg/day or alternate days) for prolonged period of up to 12 months to consolidate resolution, before considering complete withdrawal.
- remember bone protection with a bisphosphonate (avoid routine calcium and vitamin D supplementation)
- Bisphosphonates should be used to minimize steroid-induced
osteoporosis - Inhaled corticosteroids may be considered for
symptom control (cough) in a subgroup of patients - other immunosuppressive or anti-inflammatory treatments only
have a limited role but should be considered in patients when corticosteroids are not controlling the disease or side effects are intolerable. at present, methotrexate is the treatment of choice - Lung transplantation should be considered in end-stage pulmonary sarcoidosis.
If steroid treatment fails or sarcoidosis life-threatening, other immunosuppressive regimes may be indicated e.g. pulsed high-dose intravenous (IV) methylprednisolone, esp for neurosarcoidosis.
If steroid side effects cannot be tolerated, other immunosuppressive drugs should be considered. possibilities include azathioprine and methotrexate.
