Sarcoidosis, D Linger, DSA Flashcards
What is sarcoidosis
systemic granulomatous disease characterized by non-caseating granulomas
90% patients with sarcoidosis present with what
mediastinal hilar lymph node involvement or parenchymal lung disease
When is the peak incidence of sarcoidosis
between ages 20 and 39
demographic distribution of sarcoidosis
3.5x more common in black americans
women >men
What is Lofgrens syndrome
acute manifestation of the disease consisting of erythema nodosum, arthritis, and b/l hilar lymphadenopathy
prognosis of lofgrens syndrome
favorable
chronic sarcoidosis can lead to what
progressive pulmonary fibrosis and increased morbidity
What are the general Sx of sarcoidosis
fatigue, fever, night sweats and weight loss
what are Sx and signs of pulmonary involvement in sarcoidosis
cough, dyspnea with exertion, wheeze, chest discomfort
rales, expiratory wheezing
What are signs and Sx of skin involvement in sarcoidosis
rash, macules, papules, nodules, hyperpigmentation or hypopigmentation, erythema nodosum
infiltration of old scars and tattoos, maculopapular lesions of face, neck, upper back and extremities
lupus pernio of nose, cheeks, ears and lips. erythema nodosum
what are signs and Sx of ocular involvement in sarcoidosis
gritty or dry eyes, pain, redness and or blurred vision
uveitis, iritis, scleral plaques, enlarged lacrimal glands
what are signs and Sx of cardiac involvement in sarcoidosis
arrhythmia, palpitations, near-syncope, lower extremity edema
usually normal
what are signs and Sx of nervous system involvement in sarcoidosis
HA, blurred vision, numbness, weakness, instability, seizures
cranial nerves II VII VIII, neuropathies, gait instability, weakness
what are signs and Sx of GI involvement in sarcoidosis
epigastric pain, early satiety, RUQ pain, jaundice
HSM, jaundice
What are signs and Sx of MSK involvement in sarcoidosis
swelling and joint stiffness, joint pain, muscle pain
arthralgias, arthritis
What are Signs of lymphatic involvement in sarcoidosis
common sites of palpable adenopathy: cervical, supraclavicular, axillae, inguinal regions
how can you Dx sarcoidosis based of signs found around eye
yellow nodules of conjunctival involvement that can be biopsied to make Dx
what are the cardiac manifestations that are rare in sarcoidosis
AV block, ventricular arrhythmias, L ventricular dysfunction and sudden death
Most common CN involved in sarcoidosis
VII II and VIII
most common joints affected by sarcoidosis
ankles and knees
What occurs with Ca metabolism in sarcoidosis
disorders of vitamin D that may result in nephrolithiasis, nephrocalcinosis and hypercalcuria
sometimes symptomatic renal stone
How do you Dx sarcoidosis
clinical, radiographic and histologic evidence of non-caseating granulomas in one or more organs
what is pathoneumonic of sarcoidosis
lofgrens syndrome
what is show up on a bronchoalveolar lavage in sarcoidosis
lymphocytosis and elevated CD4/8 ratio
What enzyme will be elevated in sarcoid granulomas
ACE
What is on DDx for sarcoidosis
mycobacteria, fungi, spirochetes, bacteria, parasites
lymphoma, carcinoma
extrinsic allergic alveolitis (bird fanciers lung and farmers lung)
chemical:silica and beryllium
Immuno: crohns disease, primary biliary cirrhosis, vasculitis
What are the CXR staging for sarcoidosis
stage 0: normal
stage 1: b/l hilar adenopathy
stage 2: b/l hilar adenopathy and parenchymal infiltrates
stage 3: parenchymal infiltrates w/o lymphadenopathy
stage 4: advanced parenchymal disease with fibrosis
how will sarcoidosis present on a PFT
restrictive ventilatory defect, concurrent obstruction and decreased DLCO
What labs should you order if you suspect sarcoidosis
CXR PFT opthalmic exam EKG CBC CMP 24 urine serum ACE consider cardiac monitoring
When do you initiate Tx for sarcoidosis
symptomatic patients only
what is Tx for sarcoidosis
prednisone 20-40 mg daily for 3 mo
monitor for improvement in FVC and if improved wean 10-15mg daily for 6-9 mo then taper off
cytotoxic and immunosuppressives are used for those not responding to prednisone
Prognosis of sarcoidosis
spontaneous remission in 50% patients at 3 yrs
less than 5% die from sarcoidosis