Dr. Tuomela presented a case of a 37 yo M w/ minimal PMH who developed bilateral ankle swelling with overlying erythema, purple-brown plaques overlying his shins, and a persisting high grade fever + leukocytosis, all of which were refractory to PO antibiotics. After an extensive workup, this patient was found to have a specific variant of sarcoidosis: Lofgren Syndrome!
Sarcoidosis
Epi
•Age: most commonly 2—40 years
•Women > men
•Highest incidence: N Europe 5-40/100,000 people/year
•AA 3x more likely than Caucasian (11/100,000)
•Not transmissible
Path:
Inflammatory disease of unknown cause characterized by non-caseating granulomas in multiple organs and tissues
•Initial immune response by T-helper cells, followed by accumulation of activated monocytes
•T-cell activation leads to increased cytokine release à IL-2, IFN-gamma, TNF
•20% develop chronic disease (Cardiac, Neurologic, Fibrotic pulmonary disease)
Pathology in HIV
Untreated HIV who lack T helper cells rarely develop sarcoid
•Can be unmasked after anti-retroviral therapy
•Granulomas can resolve with or without therapy
Clin Presentation
•HIGHLY VARIABLE PRESENTATION: Asymptomatic to Multiorgan failure
•Most common symptoms are cough and dyspnea (2-4 weeks)
•90% have lung involvement
•Constitutional symptoms
•Cutaneous symptoms
Lupus Pernio
•Bridge of nose, beneath the eyes and cheeks
•Diagnostic for chronic sarcoid
Erythema Nodosum
•red, painful, tender lumps on lower legs
•More common in women as well as Caucasian and Puerto Rican patients
Maculopapular lesions
•Maculopapular lesions are most common
•Overlooked because non-painful and chronic
•Purple papules and indurated
•Made of non-caseating granulomas (biopsy)
Dx
•There is no definitive test.
Criteria include:
•Compatible clinical and radiographic manifestations
•Exclusion of other diseases that present similarly
•Histopathologic detection of non-caseating granulomas
•Biopsy Exceptions:
•Asymptomatic patient (Stage I) can be monitored
•Lofgren = fever, erythema nodosum, arthralgias, bilateral hilar lymphadenopathy
Staging Pulmonary Sarcoid - Characterized by chest radiograph (not CT)
•Stage I: hilar adenopathy alone
•Stage 2: Adenopathy + infiltrate
•Stage 3: Infiltrate alone
•Stage 4: Fibrosis
•Restrictive pattern vs obstructive pattern vs normal
•Airway hyperreactivity --> some will respond to bronchodilators and inhaled glucocorticoids
Pulm HTN Complication:
•At least 5% of patients
•Either direct vascular involvement of the PA or consequence of fibrotic changes in the lung
•70% of those with fibrosis waiting for transplant will have pHTN
•Median survival of 3 years
Rheum Manifestations of Sarcoid
•Periarthritis of multiple joints (ankles typically)
•Can also develop erosive damage, deformity, and dactylitis
•Bone involvement, usually associated with poorer prognosis
•Myopathy, chronic proximal weakness and ultimately atrophy or contractures
Specific Syndromes of Sarcoid:
Lofgren Syndrome = triad of:
•Bilateral hilar lymphadenopathy
•Erythema nodosum
•Migratory polyarthralgia
•If all three are present, 95% specific for sarcoidosis
•Prognosis is good, remits in 2-16 weeks
•>90% resolve within 2 years
•Goal is symptom reduction
•NSAIDs, low-dose glucocorticoids, colchicine, and hydroxychloroquine
Heerfordt Syndrome: AKA Uveparotid fever:
= fever, uveitis, and parotitis with or without cranial nerve VII palsy
•presents similar to Sjogren’s
Prognosis
•Variable – age, race, organ involvement
•Majority improve or stabilize in 2 years
•2/3 achieve spontaneous remission within 5 years. ~30% chronic disease
•<5% recurrence
•Progressive sarcoid leads to death in <5% of patients
•Usually due to respiratory failure (pulm fibrosis and rarely hemorrhage due to aspergilloma), cardiomyopathy, neurologic involvement
Comments