Sarcoidosis
Rheumatology · Other Rheumatologic Diseases 🎯 Practice these cards
Löfgren syndrome consists of ____, ____, and migratory polyarthralgia, usually accompanied by fever.
Löfgren syndrome is an acute form of systemic sarcoidosis consisting of bilateral hilar lymphadenopathy, erythema nodosum, and migratory polyarthralgia (often with fever and ankle arthritis). It favors younger women and Scandinavians and carries an excellent prognosis with spontaneous resolution.
The complete Löfgren triad has a ____% diagnostic specificity for sarcoidosis, obviating the need for ____.
When all three components of the Löfgren triad are present, diagnostic specificity for sarcoidosis is 95%, obviating the need for tissue biopsy. Treatment is symptomatic (NSAIDs) because the syndrome is usually self-limited.
Approximately ____% of sarcoidosis patients have extrapulmonary involvement, and ____% present with only extrapulmonary disease.
Although sarcoidosis most commonly affects the lungs (>90%), approximately 50% of patients have extrapulmonary involvement and 2% have ONLY extrapulmonary involvement. In the U.S., Black persons are affected more commonly and more severely than White persons.
Silent cardiac involvement occurs in at least ____% of sarcoidosis cases; the preferred diagnostic imaging test is cardiac ____.
Silent cardiac involvement occurs in at least 25% of sarcoidosis cases (clinically apparent disease: <5% of U.S. patients, ~23% of Japanese patients). Every patient needs a baseline ECG; cardiac MRI is the preferred advanced imaging test. Sudden death can be the first manifestation.
Bone involvement, reported in up to ____% of patients, appears as cystic and ____ osteolytic lesions on hand radiographs and marks a chronic course.
Bone involvement, reported in up to 15% of patients, produces characteristic cystic and lacy reticular osteolytic lesions on hand radiographs and is associated with a chronic course and poorer prognosis (often alongside lupus pernio).
Hypercalcemia in sarcoidosis reflects unregulated macrophage production of ____ (active vitamin D); hypercalciuria occurs in up to ____% of patients.
Hypercalcemia (10–20% of patients; hypercalciuria up to 50%) results from unregulated macrophage 1-alpha-hydroxylase (CYP27B1) production of calcitriol, independent of PTH. Patients should limit vitamin D intake, avoid excessive sunlight, and stay hydrated; severe cases need glucocorticoids.
A bronchoalveolar lavage CD4:CD8 lymphocyte ratio greater than ____ supports a diagnosis of sarcoidosis.
Bronchoalveolar lavage in sarcoidosis characteristically shows lymphocytosis; a CD4:CD8 lymphocyte ratio >3.5 supports the diagnosis when combined with consistent clinical and radiographic findings (a normal ratio does not exclude disease). BAL also helps exclude infection.
Spontaneous resolution rates: Scadding stage I ____%, stage II 40–70%, stage III ____%, stage IV 0%.
Spontaneous resolution by Scadding stage: I (bilateral hilar lymphadenopathy alone) 55–90%; II (adenopathy + infiltrates) 40–70%; III (infiltrates without adenopathy) 10–20%; IV (fibrosis) 0%. Asymptomatic stage I disease is observed, not treated.
First-line sarcoidosis therapy is ____; the most recommended second-line steroid-sparing agent is ____; the best-evidenced third-line biologic is ____.
Stepwise therapy: first-line glucocorticoids (e.g., prednisone 40–60 mg/d for symptomatic extrapulmonary disease, tapered to the lowest effective dose); second-line methotrexate (most recommended), azathioprine, leflunomide, mycophenolate, thalidomide, or hydroxychloroquine (skin/hypercalcemia); third-line infliximab (best-evidenced) or adalimumab; cyclophosphamide for severe organ-threatening disease.
The TNF antagonist ____ has been shown to be ineffective in sarcoidosis and should not be used.
The TNF receptor antagonist etanercept has been shown to be ineffective in sarcoidosis (pulmonary and ocular trials) and should not be used. Only monoclonal anti-TNF antibodies (infliximab, adalimumab) are effective — and they can paradoxically induce sarcoid-like reactions.
Heerfordt syndrome consists of facial nerve palsy, fever, ____, and bilateral enlargement of the ____.
Heerfordt syndrome is an acute sarcoidosis presentation of facial nerve palsy, fever, anterior uveitis, and bilateral parotid enlargement. Like Löfgren syndrome, it carries an excellent prognosis with up to 80% spontaneous remission and may be diagnosed without biopsy.
At diagnosis, every sarcoidosis patient needs an ____ to screen for silent cardiac disease and an ____ examination to detect silent uveitis.
Every newly diagnosed sarcoidosis patient should undergo an ECG (to detect silent cardiac involvement) and an ophthalmologic examination (to detect clinically silent uveitis), plus PFTs, chest imaging, and labs (creatinine, alkaline phosphatase, calcium, CBC). Optic neuropathy is a vision-threatening emergency.
____ is a violaceous infiltrative lesion of the nasal tip and central face that marks chronic sarcoidosis with bone cysts and pulmonary fibrosis.
Lupus pernio is a distinctive chronic cutaneous sarcoidosis lesion: violaceous infiltrative plaques and nodules of the nasal tip and central face, most common in African-American women. It is frequently associated with bone cysts, pulmonary fibrosis, and a prolonged clinical course — an adverse prognostic marker.
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