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Interlobular septal thickening

MedGen UID:
1779791
Concept ID:
C5539418
Finding
Synonyms: Interlobular lines (pulmonary CT finding); Interlobular septal thickening on pulmonary HRCT; Peripheral lines (pulmonary CT finding); Septal lines (pulmonary CT finding); Septal thickening (pulmonary CT finding); Short lines (pumonary CT finding)
 
HPO: HP:0030879

Definition

Presence of thickening of the interlobular septa of the lungs as seen on a CT scan. [from HPO]

Conditions with this feature

Surfactant metabolism dysfunction, pulmonary, 1
MedGen UID:
368844
Concept ID:
C1968602
Disease or Syndrome
Inborn errors of pulmonary surfactant metabolism are genetically heterogeneous disorders resulting in severe respiratory insufficiency or failure in full-term neonates or infants. These disorders are associated with various pathologic entities, including pulmonary alveolar proteinosis (PAP), desquamative interstitial pneumonitis (DIP), or cellular nonspecific interstitial pneumonitis (NSIP) (Clark and Clark, 2005). A clinically similar disorder characterized by respiratory distress (267450) can affect preterm infants, who show developmental deficiency of surfactant. Acquired PAP (610910) is an autoimmune disorder characterized by the presence of autoantibodies to CSF2 (138960). Genetic Heterogeneity of Pulmonary Surfactant Metabolism Dysfunction See also SMDP2 (610913), caused by mutation in the SPTPC gene (178620) on 8p21; SMDP3 (610921), caused by mutation in the ABCA3 gene (601615) on 16p13; SMDP4 (300770), caused by mutation in the CSF2RA gene (306250) on Xp22; and SMDP5 (614370), caused by mutation in the CSF2RB gene (138981) on 22q12.
Surfactant metabolism dysfunction, pulmonary, 5
MedGen UID:
482204
Concept ID:
C3280574
Disease or Syndrome
Pulmonary surfactant metabolism dysfunction-5 (SMDP5) is an autosomal recessive lung disorder manifest clinically and pathologically as pulmonary alveolar proteinosis (PAP). PAP is a rare lung disease characterized by the ineffective clearance of surfactant by alveolar macrophages. This results in the accumulation of surfactant-derived lipoproteinaceous material in the alveoli and terminal bronchioles, causing respiratory failure (summary by Greenhill and Kotton, 2009). For a general phenotypic description and a discussion of genetic heterogeneity of pulmonary surfactant metabolism dysfunction, see SMDP1 (265120).
Pulmonary venoocclusive disease 1
MedGen UID:
854500
Concept ID:
C3887658
Disease or Syndrome
Pulmonary venoocclusive disease primarily affects the postcapillary venous pulmonary vessels and may involve significant pulmonary capillary dilation and/or proliferation. PVOD is an uncommon cause of pulmonary artery hypertension (PPH; see 178600), a severe condition characterized by elevated pulmonary artery pressure leading to right heart failure and death. PVOD accounts for 5 to 10% of 'idiopathic' PPH and has an estimated incidence of 0.1 to 0.2 cases per million. The pathologic hallmark of PVOD is the extensive and diffuse occlusion of pulmonary veins by fibrous tissue, with intimal thickening present in venules and small veins in lobular septa and, rarely, larger veins. Definitive diagnosis of PVOD requires histologic analysis of a lung sample, although surgical lung biopsy is often too invasive for these frail patients. Patients with PVOD respond poorly to available therapy, therefore it is crucial to distinguish PVOD from other forms of PPH. Radiologic characteristics suggestive of PVOD on high-resolution CT of the chest include nodular ground-glass opacities, septal lines, and lymph node enlargement. In addition, because PVOD mainly affects postcapillary vasculature, it causes chronic elevation of pulmonary capillary pressure and thus promotes occult alveolar hemorrhage, which may be a characteristic feature of PVOD (summary by Montani et al., 2008). Genetic Heterogeneity of Pulmonary Venoocclusive Disease See also PVOD2 (234810), caused by mutation in the EIF2AK4 gene (609280) on chromosome 15q15.
Interstitial lung disease 1
MedGen UID:
1794231
Concept ID:
C5562021
Disease or Syndrome
Interstitial lung disease (ILD) comprises a heterogeneous group of rare diseases affecting the distal part of the lung and characterized by a progressive remodeling of the alveolar interstitium. The manifestations form a spectrum ranging from idiopathic interstitial pneumonia (IIP) or pneumonitis to the more severe idiopathic pulmonary fibrosis (IPF). IPF is associated with an increased risk of developing lung cancer, which occurs in a subset of patients with ILD. Clinical features of ILD include dyspnea, clubbing of the fingers, and restrictive lung capacity. Imaging typically shows ground glass opacities and inter- and intraseptal thickening, while histologic studies usually show a pattern consistent with 'usual interstitial pneumonia' (UIP) (summary by Nathan et al., 2016, Doubkova et al., 2019). Genetic Heterogeneity of Interstitial Lung Disease See also ILD2 (178500), caused by mutation in the SFTPA2 gene (178642) on chromosome 10q22.

Professional guidelines

PubMed

Zhou J, Liao X, Cao J, Ling G, Ding X, Long Q
Clin Imaging 2021 Jan;69:318-323. Epub 2020 Oct 6 doi: 10.1016/j.clinimag.2020.09.012. PMID: 33045476Free PMC Article
Xie S, Lei Z, Chen X, Liu W, Wang X, Dong Y, Guo Y, Duan Y, Cao H, Qin J, Lin B
Br J Radiol 2020 Aug;93(1112):20200243. Epub 2020 Jun 2 doi: 10.1259/bjr.20200243. PMID: 32450727Free PMC Article
Sebastiani M, Manfredi A, Vacchi C, Cassone G, Faverio P, Cavazza A, Sverzellati N, Salvarani C, Luppi F
Clin Exp Rheumatol 2020 Mar-Apr;38 Suppl 124(2):221-231. Epub 2020 Apr 22 PMID: 32324122

Recent clinical studies

Etiology

Sebastiani M, Manfredi A, Vacchi C, Cassone G, Faverio P, Cavazza A, Sverzellati N, Salvarani C, Luppi F
Clin Exp Rheumatol 2020 Mar-Apr;38 Suppl 124(2):221-231. Epub 2020 Apr 22 PMID: 32324122
Duan YN, Zhu YQ, Tang LL, Qin J
Eur Radiol 2020 Aug;30(8):4427-4433. Epub 2020 Apr 14 doi: 10.1007/s00330-020-06860-3. PMID: 32291501Free PMC Article
Song F, Shi N, Shan F, Zhang Z, Shen J, Lu H, Ling Y, Jiang Y, Shi Y
Radiology 2020 Apr;295(1):210-217. Epub 2020 Feb 6 doi: 10.1148/radiol.2020200274. PMID: 32027573Free PMC Article
Schulert GS, Yasin S, Carey B, Chalk C, Do T, Schapiro AH, Husami A, Watts A, Brunner HI, Huggins J, Mellins ED, Morgan EM, Ting T, Trapnell BC, Wikenheiser-Brokamp KA, Towe C, Grom AA
Arthritis Rheumatol 2019 Nov;71(11):1943-1954. Epub 2019 Oct 1 doi: 10.1002/art.41073. PMID: 31379071Free PMC Article
Balko R, Edriss H, Nugent K, Test V
Respir Med 2017 Nov;132:203-209. Epub 2017 Oct 20 doi: 10.1016/j.rmed.2017.10.015. PMID: 29229098

Diagnosis

Donuru A, Torigian DA, Knollmann F
Tomography 2024 Apr 17;10(4):574-608. doi: 10.3390/tomography10040045. PMID: 38668402Free PMC Article
Sun JD, Shum T, Behzadi F, Hammer MM
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Zheng Q, Lu Y, Lure F, Jaeger S, Lu P
J Xray Sci Technol 2020;28(3):391-404. doi: 10.3233/XST-200687. PMID: 32538893Free PMC Article
Sebastiani M, Manfredi A, Vacchi C, Cassone G, Faverio P, Cavazza A, Sverzellati N, Salvarani C, Luppi F
Clin Exp Rheumatol 2020 Mar-Apr;38 Suppl 124(2):221-231. Epub 2020 Apr 22 PMID: 32324122
Schulert GS, Yasin S, Carey B, Chalk C, Do T, Schapiro AH, Husami A, Watts A, Brunner HI, Huggins J, Mellins ED, Morgan EM, Ting T, Trapnell BC, Wikenheiser-Brokamp KA, Towe C, Grom AA
Arthritis Rheumatol 2019 Nov;71(11):1943-1954. Epub 2019 Oct 1 doi: 10.1002/art.41073. PMID: 31379071Free PMC Article

Therapy

Lechartier B, Boucly A, Solinas S, Gopalan D, Dorfmüller P, Radonic T, Sitbon O, Montani D
Eur Respir Rev 2024 Jan 31;33(171) Epub 2024 Jan 17 doi: 10.1183/16000617.0156-2023. PMID: 38232988Free PMC Article
Nakamura J, Tsujino I, Shima H, Nakaya T, Sugimoto A, Sato T, Watanabe T, Ohira H, Suzuki M, Tsuneta S, Hisada R, Kato M, Konno S
Am J Cardiovasc Drugs 2023 May;23(3):329-338. Epub 2023 Mar 30 doi: 10.1007/s40256-023-00577-6. PMID: 36995544
Lu J, Ma M, Zhao Q, Meng F, Wang D, Cai H, Cao M
Sci Rep 2018 May 8;8(1):7300. doi: 10.1038/s41598-018-25670-8. PMID: 29740120Free PMC Article
Wang T, Lazar CA, Fishbein MC, Lynch JP 3rd
Semin Respir Crit Care Med 2012 Oct;33(5):498-508. Epub 2012 Sep 21 doi: 10.1055/s-0032-1325160. PMID: 23001804
Collins J
Radiol Clin North Am 2001 Nov;39(6):1115-35. doi: 10.1016/s0033-8389(05)70334-1. PMID: 11699664

Prognosis

Eggleston RH, Hartman TE, Walkoff LA, Yi ES, Ryu JH, Baqir M
Respir Med 2022 Apr;194:106761. Epub 2022 Feb 8 doi: 10.1016/j.rmed.2022.106761. PMID: 35217402
Sebastiani M, Manfredi A, Vacchi C, Cassone G, Faverio P, Cavazza A, Sverzellati N, Salvarani C, Luppi F
Clin Exp Rheumatol 2020 Mar-Apr;38 Suppl 124(2):221-231. Epub 2020 Apr 22 PMID: 32324122
Duan YN, Zhu YQ, Tang LL, Qin J
Eur Radiol 2020 Aug;30(8):4427-4433. Epub 2020 Apr 14 doi: 10.1007/s00330-020-06860-3. PMID: 32291501Free PMC Article
Balko R, Edriss H, Nugent K, Test V
Respir Med 2017 Nov;132:203-209. Epub 2017 Oct 20 doi: 10.1016/j.rmed.2017.10.015. PMID: 29229098
Wang T, Lazar CA, Fishbein MC, Lynch JP 3rd
Semin Respir Crit Care Med 2012 Oct;33(5):498-508. Epub 2012 Sep 21 doi: 10.1055/s-0032-1325160. PMID: 23001804

Clinical prediction guides

Dumea E, Lazar M, Barbu EC, Chitu CE, Ion DA
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Curr Med Imaging 2022;18(12):1311-1317. doi: 10.2174/1573405618666220509212035. PMID: 35579138
Sebastiani M, Manfredi A, Vacchi C, Cassone G, Faverio P, Cavazza A, Sverzellati N, Salvarani C, Luppi F
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Gorkem SB, Köse S, Lee EY, Doğanay S, Coskun AS, Köse M
Pediatr Pulmonol 2017 Apr;52(4):494-499. Epub 2016 Oct 31 doi: 10.1002/ppul.23614. PMID: 27797454
Yoon CG, Kim SJ, Kim K, Lee JE, Jhun BW
J Korean Med Sci 2016 Feb;31(2):247-53. Epub 2016 Jan 26 doi: 10.3346/jkms.2016.31.2.247. PMID: 26839479Free PMC Article

Recent systematic reviews

Osman M, Klopfenstein T, Belfeki N, Gendrin V, Zayet S
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Clin Radiol 2020 Nov;75(11):876.e33-876.e39. Epub 2020 Aug 12 doi: 10.1016/j.crad.2020.07.025. PMID: 32861461Free PMC Article
Ojha V, Mani A, Pandey NN, Sharma S, Kumar S
Eur Radiol 2020 Nov;30(11):6129-6138. Epub 2020 May 30 doi: 10.1007/s00330-020-06975-7. PMID: 32474632Free PMC Article
Bao C, Liu X, Zhang H, Li Y, Liu J
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