sarcoid Flashcards
What baseline tests should you do?
FBC, UE (renal sarcoid), LFTS- ALP (hepatic sarcoid and treatment), Vit D and Ca, ECG, eye exam, CXR
What tests should be done if cardiac sarcoid suspected?
holter
echo: look for cardiac sarcoidosis (although with low sensitivity) prior to a more advanced imaging modality and also to look for any other cause of cardiac dysfunction which may be seen in sarcoidosis, eg LV heart disease or PH
cardiac MRI- gives prognostic and diagnostic info
Histology?
well-formed, concentrically arranged layers of immune cells, most prominent being the central core of macrophage aggregates and multinucleated giant cells.
An outer layer of loosely organized lymphocytes, mostly T cells, is often observed with a few interposed dendritic cells
often nonnecrotic; however, variants of sarcoidosis, particularly the nodular pulmonary sarcoidosis phenotype, can present with a mixture of necrotic and nonnecrotic granulomas
imaging findings that make sarcoid highly probable?
CXR Bilateral hilar adenopathy
CT Perilymphatic nodules, Osteolysis, cysts/punched-out lesion, trabecular pattern bone,
peribronchial tissue and interlobular septa are the most common sites of sarcoid lesions
MRI Gadolinium enhancement on MRI
probable:
Upper lobe or diffuse infiltrates (CXR, CT, and PET)
Peribronchial thickening (CT)
Two or more enlarged extra thoracic nodes (CT, MRI, and PET) Increased inflammatory activity in heart (MRI, PET, and
gallium)
Imaging showing enlargement or nodules in liver or spleen
(CT, PET, and MRI)
Inflammatory lesions in bone (gallium, PET, and MRI)
physical signs that make sarcoid highly probable?
Lupus pernio Uveitis
Optic neuritis Erythema nodosum
probable: Maculopapular, erythematous, or violaceous skin lesions Subcutaneous nodules
Scleritis
Retinitis
Lacrimal gland swelling
Granulomatous lesions on direct laryngoscopy Symmetrical parotid enlargement Hepato-/splenomegaly
What is Erdheim-Chester?
histiocytic disorder with clinical and radiologic features similar to sarcoidosis affecting parotids, may be differentiated from sarcoidosis on the basis of histopathologic staining for the CD68 marker
What should be screened annually?
Renal function for renal sarcoid
Liver function in hepatic sarcoid and monitor hepastotoxicity of drugs
Ca level- you want to prevent renal failure. it is steroid responsive, if RF happens- RF is normally irreversible
what is Lofgren’s?
triad of fever, erythema nodosum, and bilateral hilar adenopathy, mya have bilateral ankle arthritis
self limiting and doesn’t require treatment. if given treatment, there is a small subgroup that might get worse on steroids
need 3/12 follow up CXR and doens’t need CT as long as symptoms don’t change
Who to do image surveillance on?
Should be limited to patients with unexplained deterioration in symptoms or lung function, those with ‘red flags’ or when specific conditions are suspected such as aspergilloma
CD4:CD8 ratio from BAL?
CD4:CD8 ratio >4 in the absence of an increased proportion of other inflammatory cell types is highly specific (95%)
when should we start treatment?
PFTs
TLCO <65%, FVC <70%
or if there is a 10% drop from baseline FVC or 15% drop from baseline TLCO. The caveat is presence of inactive fibrotic disease which may have resulted in irreversible but non-progressive lung function loss
When would you give methylpred?
It is seldom warranted in pulmonary disease in isolation but may be required with rapidly progressive extrapulmonary disease (eg, cardiac sarcoidosis, neurosarcoid- osis, severe optic neuritis).
How much steroid? and how? and when to stop?
20 to 40 mg per day of prednisolone for 4 to 6 weeks, followed by slow tapering (eg, reducing by 5 mg every 2 weeks) to a maintenance dose, usually between 5 to 10mg per day
Attempts at withdrawing prednisolone should be made every 6 to 12 months
when would you give 2nd line therapy?
- Progression of disease or unacceptable symptom burden despite adequate steroid therapy.
- Intolerable steroid side effects.
- Inability to taper steroid below 10 to 15mg per day.
- The presence of major comorbidities likely to be adversely affected by steroid therapy (severe obesity, DM, OP, HTN)
- A strong patient aversion to steroids, in which case, a second-line agent may be used as initial therapy.
What treatment options are there for fatigue?
PR, inspiratory muscle training, d-methylphenidate or armodafinil for 8 weeks to tests its effect, low dose steroids +/- MTX
