Histologic and Fibroblastic Foci Score of Nicholson et al for Patients with Idiopathic Pulmonary Fibrosis

Description

Nicholson et al developed a histologic score for evaluating lung biopsies from patients with idiopathic pulmonary fibrosis. The score can be used to monitor the patient over time, while some components may predict disease progression. The authors are from the Royal Brompton Hospital in London and the Mayo Clinic in Scottsdale.

Parameters for the histologic score:

(1) fibroblastic foci (FF): distinct, somewhat nodular area of loose fibroblastic proliferation

(2) interstitial mononuclear cell infiltrate (IM)

(3) established fibrosis (EF)

(4) intra-alveolar macrophages (AM)

Parameter	Finding	Points
fibroblastic foci	none	0
	minimal	1
	mild	2
	mild to moderate	3
	moderate	4
	moderate to marked	5
	marked	6
interstitial mononuclear cell infiltrate	none	0
	minimal	1
	mild	2
	mild to moderate	3
	moderate	4
	moderate to marked	5
	marked (degree seen in lymphocytic interstitial pneumonia)	6
established fibrosis	none	0
	minimal	1
	mild	2
	mild to moderate	3
	moderate	4
	moderate to marked	5
	end stage (honeycomb lung)	6
intra-alveolar macrophages	none	0
	normal	1
	mild increase	2
	mild to moderate increase	3
	moderate increase	4
	moderate to marked increase	5
	marked accumulation (degree seen in desquamative interstitial pneumonia)	6

histologic score =

= SUM(points for all 4 parameters)

If more than one lung biopsy was taken, then the final histologic score is the average of the histologic scores for each biopsy.

Interpretation:

• minimum score: 0

• maximum score: 24

• A high fibroblastic foci score correlated with increased mortality.

• A rising interstitial mononuclear cell infiltrate is associated with functional decline (declining FVC and carbon monoxide diffusing capacity) at 6 months.

• A rising fibroblastic foci score is associated with functional decline (declining FVC and carbon monoxide diffusing capacity) at 6 and 12 months.

Performance:

• Inter-observer agreement was moderate to good (0.56 to 0.76).

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Description

Specialty

ICD-10