Table 8.

Response assessment and disease surveillance recommendations for LCH in adults

Type of LCHFirst response assessmentSubsequent assessmentsComments
Skin only Skin exam at each visit Skin exam at each visit  
Bone only FDG-PET/CT in 2-3 mo FDG-PET/CT every 3-6 mo*  
Pituitary and hypothalamus only Endocrine evaluation in 3 mo Endocrine evaluation annually MRI of pituitary/hypothalamus if initially involved 
Other unifocal LCH FDG-PET/CT or CT/MRI in 2-3 mo FDG-PET/CT or CT/MRI every 3-6 mo*  
Single-system PLCH PFTs and HRCT chest in 3-6 mo PFTs and HRCT chest 6-12 mo TTE and right-heart catheterization may be needed if suspicion of pulmonary hypertension 
Multisystem LCH FDG-PET/CT in 2-3 mo FDG-PET/CT every 3-6 mo* Organ-specific imaging (CT or MRI) may be needed depending on organ involvement 
Type of LCHFirst response assessmentSubsequent assessmentsComments
Skin only Skin exam at each visit Skin exam at each visit  
Bone only FDG-PET/CT in 2-3 mo FDG-PET/CT every 3-6 mo*  
Pituitary and hypothalamus only Endocrine evaluation in 3 mo Endocrine evaluation annually MRI of pituitary/hypothalamus if initially involved 
Other unifocal LCH FDG-PET/CT or CT/MRI in 2-3 mo FDG-PET/CT or CT/MRI every 3-6 mo*  
Single-system PLCH PFTs and HRCT chest in 3-6 mo PFTs and HRCT chest 6-12 mo TTE and right-heart catheterization may be needed if suspicion of pulmonary hypertension 
Multisystem LCH FDG-PET/CT in 2-3 mo FDG-PET/CT every 3-6 mo* Organ-specific imaging (CT or MRI) may be needed depending on organ involvement 

PFT, pulmonary function tests; TTE, transthoracic echocardiogram.

*

Once the best response is achieved and disease stabilized, the frequency of testing and imaging studies can be individualized to longer intervals.

Chest radiography can be performed instead of HRCT for follow-up in cases that are mild and do not require treatment. Serial lung HRCT is recommended in patients who exhibit changes in their clinical, functional, or chest radiography findings during follow-up.

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