Flashcards in Sarcoidosis -Goya Deck (11):
What is sarcoidosis?
a multisystem granulomatous disorder of unknown etiology that is characterized by the presence of noncaseating granulomas in the involved organs
Commonly presents with bilateral hilar lymphadenopathy, pulmonary infiltration & skin lesions.
Symptoms related to Lung, Skin, Eyes, Peripheral nerves, Liver, Kidney, Heart …are possible.
more common in North American blacks (women > men) and North Europen whites
3rd or 4th decade
spontaneous remission in majority of cases
What is the pathogenesis of sarcoidosis?
noncaseating granulomas composed of a central core of epithelioid histocytes and multinucleate giant cells
activated T cells and macrophages accumulate at site of inflammation
release chemoattractants and growth factor --> cell proliferation and granuloma formation
progressive granulomatous inflammation leads to injury, dysfunction and destruction of affected organs
What is the most common organ system affected by sarcoidosis?
respiratory tract (SOB, nonproductive cough, nonspecific CP, hemoptysis)
present asymptomatically 40% of the time
What are some other presenting symptoms in sarcoidosis?
-skin manifestations--> erythema nodosum =raised skin lesions over the shins (Lofgren's syndrome)
-occular involvement (35% get conjunctivitis)
-arthralgias and arthritis
-neuro: poor prognosis. Cranial nerve palsy, paresthesias, seizures.
A 42-year-old female comes to the physician’s office with persistent cough from last 1 year. She had unprotected sex with multiple partners in last 1 year. Four months ago she noticed red bumps on her legs. She also had swelling of her joints. Her HIV test is negative. Her chest x-ray shows bilateral hilar masses. Which of the following is most likely diagnosis?
A. Acute HIV disease
What chest x-ray findings are associated with the different stages of sarcoidosis?
-Bilateral hilar lymphadenopathy with or without paratracheal adenopathy
-Normal lung parenchyma
-50% present with this as asymptomatic
-bilateral hilar adenopathy with lung involvement
-25% present in this stage.
-symptomatic (1/3 will progress to fibrosis)
stage 3: (lung only)
-significant fibrosis without bilateral hilar adenopathy
-15% present with this
-pulmonary fibrosis with honeycombing
-worse in upper lobes
What lab abnormalities are seen in sarcoidosis? Are these specific?
elevated ACE in 50-80% of pts
*not specific to sarcoidosis
What is the diagnostic criteria for sarcoidosis?
appropriate clinical picture, histologic findings & exclusion of other potential diseases capable of producing a similar picture
Fiberoptic bronchoscopy is the most common method for obtaining a diagnosis
Biopsy is 50-60% in patients without parenchymal disease
90-95% in patients with parenchymal disease
What are the indications for steroid use in sarcoidosis? Do pts with stage 1 disease an normal lung function require treatment with steroids? Stage 2-3?
Progressive pulmonary impairment or respiratory symptoms
Persistent hypercalcemia/hypercalicuria with renal insufficiency
*Patients with stage 1 disease with or without erythema nodosum & with normal lung function (VC, DLCO) do not require treatment with corticosteroids
stage 2-3 with symptoms and impaired lung functions should be treated with steroids
What features suggest poor prognosis in sarcoidosis?
Insidious onset with cutaneous, eye or bone involvement
CXR stages 2 or 3
Alveolar or fibrotic changes on CXR
Progression of organ involvement beyond 1 year