Sarcoidosis Flashcards
definition of sarcoidosis
mutlisystem inflammatory disaease with protean manifestations. large spectrom of organ system invovlement with variable course and prognsis and unclear indcations for treatment.
etiology of sarcoidosis
genetic influence (80% among MZ twins) combined with environmental factors (smoking actually protective).BTNL2 gene may increase risk. HL class II type may be associated with disease development, phenotype, and environmental interactions
stages of immunopathogenesis of sarcoidosis
- initiation: antigen stimulates T cell activation. TNF probably plays a role
- granuloma formation: macrophages differentiate into the core, T cells are recruited, fiboblast and collagen also encase cells.
- disease either goes into remission or becomes chronic.
3 features for sarcoidosis diagnosis
- presence of clinical and radiographic picture consistent with sarcoidosis
- non-caseating granulomas present
- exclusion of other causes
features of pulmonary sarcoidosis
dyspnea, dry cough, chest pain, fatigue. exam seems normal. on function testing, you will see more restriction than obstruction and a decrease in DLCO. see adenopathy.
may also see pulmonary hypertension and hypoxia
sarcoidosis staging based on X-ray
O- IV 0: no lung involvement 1: bilateral lympadenopathy (LAD) II: LAD + infiltrates III: parenchymal disease but no LAD IV: pulmonary fibrosis
How are stages useful for sarcoidosis?
Stage 0 and 1 most likely to have sponateous remission; III and IV are least likely. stages don’t predict flares and are not progressive
sarcoidosis staging based on CT
I, II: nodes
III: confluent nodules
IV: bronchiectasis, fibrosis, and air trapping
asymmetric LAD, cavitated/spiculated nodules, and pleural effusions all unusual
Lofgren’s syndrome
fever, erythema nodosum, polyarthritis, bilateral hilar adenopathy. form of acute sarcoidosis. increases chances of spontaneous remission
Warning systems for sarcoidosis
eye, heart, kidney, neuro
sarcoidosis of skin
many manifestation. erythema nodosum is most common: painful. no granulomas on biopsy
lupus pernio is another form and requires immediate treatment
eye involvement in sarcoidosis
anterior uveitis more than posterior. 10% go completely blind. consider cataracts too
Cardiac involvement in sarcoidosis
2nd leading cause of death, including sudden cardiac death. many ppl have baseline cardiac fivindings. but this is hard to diagnose (look at interventricular septum)
Kidney involvement in sarcoidosis
hypercalcemia frequent complication
neurosarcoid
uncommon. can invovle brain base, cranial nerves (esp VII) hypothalamus, pituitary. may be central or peripheral.
What is signficicance to CD4: CD8 ratio?
if BAL CD4> CD8, probably sarcoid. specific but not sensitive.
What other diseases present like sarcoidosis?
mycobacteria, fungus, esp. histoplasmosis, hypersensitivity pneumonitis, beryllium and other heavy metal exposure, lymphoma, Wegener’s and Churg-Strauss
prognostic predictors
chest x-ray. African americans have worse prognosis. E nodosa and lofgrens and arthritis, isolated uveitis, and isolated CNVII all have good prognosis. worse prognosis: age over 40, cardiac problems, hypercalcemia, chronic uveitis, lupus pernio, nephrocacinosis, neurosarcoidosis, progressive pulmonary fibrosis, pulmonary HTN.
when is sarcoidosis treatment warranted?
III and IV disease. sometimes for stage II. indicated with problems iwth lung function, cardiac/neuro involvement, uveitis that doesn’t respond to steroids, hypercalcemia.
relapse is 75% if treatment is stopped after starting it.
treatment for acute sarcoidosis
predinso 20-40 mg/day; more for cardiac/neuro attacks. consider alternate agent if not better after 3 months. taper dose after 3 months but expect to keep pt on meds for at LEAST a year. steroids treat symptoms but don’t change long term progression
Non-steroidal sacoidosis treatment
methotrexate, esp for lung, CNS, eye, skin
anti TNF agents (infliximab): last line agent
azathioprine for hepatic/ocular
leflunomide for lung
