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CC May 2018 Sarcoidosis

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echoecho's version from 2018-05-02 04:41

Section

Question Answer
Define sarcoidosis?1) from the Greek "fleshy" 2) multi system disease 3) presence of multiple, noncaseating GRANULOMAS located throughout the body affecting various organs
List the most common locations?1) Mediastinal lymph nodes (95-98%) 2) lungs (> 90%) 3) liver (50-80%) 4) spleen (40-80%) 5) skin (25%)
About 80% of cases occur between the ages of ___ to ___ years, with a second peak occurrence between ages ____ to ____ years?20; 50; 50; 65
The lifetime incidence of sarcoidosis is ____% in AA and ____% in Caucasians?2.4%; 0.85%
It is more common in individuals of _________ancestry?Scandinavian
A family hx of sarcoidosis is seen in ___ % to ____% of patients?4; 10
The lifetime mortality due to sarcoidosis is < ___%, usually due to what causes?5; pulmonary disease or cardiac failure
Although the cause is unknown, the underlying _______ process results in formation of noncaseating granulomas with accumulated macrophages and activated T-lymphocytes?inflammation
The macrophages and T-lymphocytes, in turn, produce ______ and ______ ______ _____ _______, that amplify the inflammatory response?1) cytokines 2) tumor necrosis factor-alpha
Due to the increased risk in patients w/ a positive family hx of sarcoidosis, it is proposed that disease develops in ______ ________ individuals who may respond to specific antigens and infections?genetically susceptible
The presentation of sarcoidosis is dependent upon the affected _____ systems?organ
What are the commonly initial symptom?1) unexplained cough 2) dyspnea 3) nonspecific constitutional symptoms (fever, weight loss and fatigue)
Asymptomatic patients may have what on CXR performed for an unrelated reason?hilar adenopathy
List two classic constellations of symptoms ?1) Lofgren syndrome 2) Heerfordt syndrome
List the constellations of symptoms found in the Lofgren syndrome?1) erythema nodosum 2) b/l hilar adenopathy on CXR 3) fever 4) polyarthritis 5) uveitis
List the constellation of symptoms found in the Heerfordt syndrome?1) uveitis 2) parotitis 3) fever 4) facial nerve palsy
Sarcoidosis is associated w/ production of 1,25 (OH)2-vitamin D by the granulomas resulting in what?increased intestinal calcium absorption and possible hypercalcemia
With hypercalcemia, what may the patient present with?1) calcium-containing kidney stones 2) constipation 3) nausea 4) polyuria 5) lethargy 6) weakness
List the differential diagnosis for sarcoidosis?1) drug-induced hypersensitivity reactions 2) heavy metal pneumoconiosis 3) chronic variable immunodeficiency 4) TB 5) pulmonary fungal infections 6) HIV 7) lymphoma 8) vasculitis
If sarcoidosis is suspected, what is the initial work-up?1) CBC 2) lyres 3) liver chemistries 4) serum calcium 5) renal function tests 6) UA 7) HIV 8) TB testing 9) CXR 10) pulmonary function tests 11) echo
*** Diagnosis of sarcoidosis requires 3 criteria, list these?1) a compatible clinical and radiologic presentation 2) pathological evidence of noncaseating granuloma 3) exclusion of other diseases with similar findings
List the 5 different stages for radiologic staging of sarcoidosis with the rate of spontaneous remission and recommended evaluation schedule?stages 0, I, II, III, IV
What does the CXR, rate of spontaneous remission and recommended evaluation schedule looke like for stage 0 in the staging of sarcoidosis?CXR = normal; Rate of spontaneous remission = N/A; Recommended evaluation schedule = N/A
What does the CXR, rate of spontaneous remission and recommended evaluation schedule looke like for stage I in the staging of sarcoidosis?CXR = b/l hilar lymphadenopathy; Rate of spontaneous remission = 55-99%; Recommended evaluation schedule = every 6 months; may go to annual if symptoms are stable
What does the CXR, rate of spontaneous remission and recommended evaluation schedule looke like for stage II in the staging of sarcoidosis?CXR = b/l hilar lymphadenopathy AND pulmonary infiltrates; Rate of spontaneous remission = 40-70%; Recommended evaluation schedule = every 3-6 months
What does the CXR, rate of spontaneous remission and recommended evaluation schedule looke like for stage III in the staging of sarcoidosis?CXR = pulmonary infiltrates WITHOUT hilar lymphadenopathy; Rate of spontaneous remission = 10-20%; Recommended evaluation schedule = every 3-6 months
What does the CXR, rate of spontaneous remission and recommended evaluation schedule looke like for stage IV in the staging of sarcoidosis?CXR = pulmonary fibrosis; Rate of spontaneous remission = 0-5%; Recommended evaluation schedule = every 3-6 months
*** List the reliable biomarkers for diagnosing sarcoidosis?there are none
Levels of _____ ______ enzyme may be elevated in up to 75% of patients, but it is not specific for the disease and levels do not correlate the severity of the symptoms?angiotensin-converting enzyme (ACE)
When biopsy confirmation is required, the use of what instrument is often recommended due to the frequency of pulmonary and hilar involvement and the high diagnostic yield using this instrument sampling and low risk of complication?flexible bronchoscope
Biopsy of what lymph nodes has a high diagnostic yield but is associated with a higher rate of complication?mediastinal
In general, topical therapy for sarcoidosis (typically what medication) is indicated for skin lesions, anterior uveitis, cough or nasal polyps?corticosteroids
Asymptomatic stage I and II disease does not require treatment because what is common?spontaneous remission
Systemic therapy is indicated when?1) significant pulmonary disease (persistent pulmonary infiltrates or decline in lung function ) 2) cardiac disease 3) neurosarcoidosis 4) ocular disease that does not respond to topical therapy 5) symptomatic hypercalcemia 6) lupus pernio (cutaneous sarcoidosis involving the nose, ears, cheeks and lips)
First line systemic therapy for symptomatic patients and for those w/ progressive disease is oral _______?corticosteroids
Of note, while some studies suggest that pulmonary findings on CXR improve with steroids, is there any evidence that mortality, lung function or disease progression are affected?no
There are no validated protocols for corticosteroid therapy, but usual practice is to use what?an extended course of prednisone (20-40 mg daily) x 4-6 weeks, followed by a slow taper if symptoms stabilize or improve.
Are inhaled steroids effective in the treatment of pulmonary sarcoidosis?have not been shown to be effective
If patients do not improve after ____ months of steroid therapy, they are unlikely to respond to it, and second-line therapy should be considered?3
*** When is second-line therapy considered?1) for those who decline or are intolerant of steroids 2) develop significant side effects (hyperglyecemia, osteoporosis) 3) who have progression of their disease despite adequate doses and duration of treatment
List the second-line therapies?1) Methotrexate (Trexall) 2) Azathioprine (Imuran) 3) Leflunomide (Arabia) 4) Hydroxychloroquine (Plaquenil) 5) Infliximab (Remicade) 6) Adalimumab (Humira)
There is no evidence to support the use of one drug over another in second-line therapies, however, which drug is recommended by experts for second-line therapy?Methotrexate
What does effect does Methotrexate and Azathioprine have that allows dose reductions of maintenance prednisone? What function is improved?1) steroid-sparing effects 2) pulmonary
There is a higher infection rate with what of the second-line therapies?Azathioprine (Imuran)
What surgery has been used in patients with severe, progressive pulmonary sarcoidosis that is resistant to medical therapy?lung transplantation
*** SUMMARY = What is the etiology of sarcoidosis?unknown
*** SUMMARY = What is sarcoidosis characterized by?noncaseating granulomas that can affect any organ but predominantly involves the lungs and hilar lymph nodes
*** SUMMARY = Sarcoidosis is suspected when there is what symptoms?1) unexplained cough 2) fever 3) weight loss 4) dyspnea 5) other systemic symptoms
*** SUMMARY = Sarcoidosis is particularly seen in what 2 ethnic groups?1) AA 2) Scandinavian origin
*** SUMMARY = Why is the diagnosis of sarcoidosis challenging?1) a single, reliable diagnostic test is not available 2) patients may be asymptomatic 3) initial symptoms are variable
*** SUMMARY = For symptomatic patients, what is the first line therapy?oral prednisone
*** SUMMARY = For symptomatic patients, what is the most commonly used second-line drug?Methotrexate (Trexall)
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