Sarcoid Flashcards
What are the common symptoms / clinical presentation of sarcoidosis?
Usually asymptomatic, picked on CXR (30%)
Basically: 90% presents with intrathoracic lymphadenopathy, pulmonary involvement, skin and ocular signs.
Top from bottom:
Systemic: fatigue, night sweats, weight loss, lymphadenopathy (20%)
Skin: erythema nodosum, Lupus pernio
MSK: Arthralgia - even frank arthritis can occur (50%)
Brain - psychiatric / seizures
Eye - uveitis, photophobia, sicca symptoms
Face - facial nerve palsy
Cardiac: conduction problems (VT, CHB - 5%)
Lung: SOB/Cough - ILD (15%), Bilateral hilar lymphadenopathy
GI: Cholestasis & derranged LFTs, Hepatosplenomegaly (20-40%)
Renal: stones due to hypercalcaemia (rare)
Lofgren’s syndrome? (4)
Bilateral hilar LN
Fever
Polyarthralgia (migratory)
Erythema nodosum
Examination for Sarcoidosis - findings to report.
Skin: maculopapular rash, Lupus pernio, anterior uveitis (+papilloedema)
CVS: arrhythmia, RVF
Resp: ILD
Abdo: Hepatosplenomegaly
Haem: Lymphadenopathy
Neuro: 7th CN palsy
MSK: arthralgia
Diagnostic approach for Sarcoid?
Definitive diagnostic test does not exist.
Following 3 generally required
- Compatible clinical + radiographic features (i.e. hypercalcaemia, bilateral hilar enlargement)
- Exclusion of other diseases that may present similarly
- Histologic detection of non-caseating granulomas
So ultimately biopsy is required. Exceptions are…
Bilateral hilar lymphadenopathy in asymptomatic patient (Stage 1)
Classic Lofgren’s (fever, arthralgia, erythema nodosum and bilateral hilar LN)
What is your approach to investigating suspected Sarcoidosis in this patient?
T: no diagnostic test. CXR (bilateral hilar LN), CT chest, Calcium, ACEi (raised in 2/3) but ultimately require bronchoscopy/open/close lung biopsy or LN for non-caseating granuloma (consider biopsying other organs if involved - e.g. LN, skin, liver) and BAL (increased CD4+ T-cells). PET is helpful in identifying sites to biopsy
E: Lung Ca (biopsy), TB (IGRA and BAL for MCS/AFB/TB culture), septic work-up
S: depending on systems involved. For pulmonary - CXR (?stage), HRCT - look for active alveolitis (GG changes, any evidence of infection as alternative diagnosis), inflammatory markers, ABG, PFT, serum Calcium.
T: HBA1C (as steroids maybe needed), lipid proflie, full set of blood
S: 24-h urine excretion of calcium, resistant organisms (e.g. Aspergillous), 24-Holter (conduction), TTE (cardiomyopathy, pHTN), consider Cardiac MRI (best for delineating cardiac involvement of disease), HRCT (ILD)
How would you stage Sarcoidosis?
Stage 1: bilateral hilar LN
Stage 2: bilateral hilar LN + infiltration
Stage 3: infiltration alone
4: fibrotic bands, bullae, bronchiectasis
Would you treat this patient with Sarcoidosis? what are indications of treatment (3)
What are pharmacologic options and how would you follow this patient up?
Non-progressive disease does not require treatment. Asymptomatic patient with pulmonary sarcoid but no extrapulmonary involvement - no treatment (high rate of spontaneous remission, especially in context of side Fx of long course of steroids)
Progressive disease or Active disease - progressive CXR changes (stage II,III), worsening lung function or significant systemic disease require treatment.
Treatment:
- Prednisolone 1mg/kg up to 6 weeks (UTD suggest 0.5mg/kg, 20-40mg/d). If no improvement, continue for another 4-6 weeks at same dose. Once improvement is seen, taper down for following few months. Then maintenance dose for further 6-8 months, total ~ 12 months.
- For those with cough but otherwise do not meet the criteria for PO steroids - consider inhaled steroids (budesonide 800-1600mcg BD) - 2B, consider as an alternative to long-term low dose prednisolone.
- Regular follow-up for monitoring - physical exam, CXR/CT, spirometry, PFT, Sats every few months. Monitor carefully for steroid complications.
- Steroid refractory disease - addition of immunosuppressive can be considered (MTX, AZA, Leflunomide). These agents can be used in setting of steroid sparing agent.
- Anti-TNF
