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Pulmonary fibrosis

MedGen UID:
11028
Concept ID:
C0034069
Disease or Syndrome
Synonyms: Fibroses, Pulmonary; Fibrosis, Pulmonary; Pulmonary Fibroses; Pulmonary Fibrosis
SNOMED CT: Fibrosis of lung (51615001); Pulmonary fibrosis (51615001); Cirrhosis of lung (51615001)
 
HPO: HP:0002206
Monarch Initiative: MONDO:0002771

Definition

Chronic progressive interstitial lung disorder characterized by the replacement of the lung tissue by connective tissue, leading to progressive dyspnea, respiratory failure, or right heart failure. Causes include chronic inflammatory processes, exposure to environmental irritants, radiation therapy, autoimmune disorders, certain drugs, or it may be idiopathic (no identifiable cause). [from NCI]

Conditions with this feature

Idiopathic pulmonary hemosiderosis
MedGen UID:
9403
Concept ID:
C0020807
Disease or Syndrome
Idiopathic pulmonary hemosiderosis is a respiratory disease due to repeated episodes of diffuse alveolar hemorrhage without any underlying apparent cause, most often in children. Anemia, cough, and pulmonary infiltrates on chest radiographs are found in majority of the patients.
Dyskeratosis congenita, X-linked
MedGen UID:
216941
Concept ID:
C1148551
Disease or Syndrome
Dyskeratosis congenita (DC), a telomere biology disorder, is characterized by a classic triad of dysplastic nails, lacy reticular pigmentation of the upper chest and/or neck, and oral leukoplakia. The classic triad may not be present in all individuals. People with DC are at increased risk for progressive bone marrow failure (BMF), myelodysplastic syndrome (MDS) or acute myelogenous leukemia (AML), solid tumors (usually squamous cell carcinoma of the head/neck or anogenital cancer), and pulmonary fibrosis. Other findings can include: abnormal pigmentation changes not restricted to the upper chest and neck, eye abnormalities (epiphora, blepharitis, sparse eyelashes, ectropion, entropion, trichiasis), and dental abnormalities (caries, periodontal disease, taurodauntism). Although most persons with DC have normal psychomotor development and normal neurologic function, significant developmental delay is present in the two variants in which additional findings include cerebellar hypoplasia (Hoyeraal Hreidarsson syndrome) and bilateral exudative retinopathy and intracranial calcifications (Revesz syndrome). Onset and progression of manifestations of DC vary: at the mild end of the spectrum are those who have only minimal physical findings with normal bone marrow function, and at the severe end are those who have the diagnostic triad and early-onset BMF.
Idiopathic Pulmonary Fibrosis
MedGen UID:
321462
Concept ID:
C1800706
Disease or Syndrome
Idiopathic pulmonary fibrosis is one of a family of idiopathic pneumonias sharing clinical features of shortness of breath, radiographically evident diffuse pulmonary infiltrates, and varying degrees in inflammation, fibrosis, or both on lung biopsy. In some cases, the disorder can be rapidly progressive and characterized by sequential acute lung injury with subsequent scarring and end-stage lung disease. Although older studies included several forms of interstitial pneumonia under the term 'idiopathic pulmonary fibrosis,' the clinical label of 'idiopathic pulmonary fibrosis' should be reserved for patients with a specific form of fibrosing interstitial pneumonia referred to as usual interstitial pneumonia (Gross and Hunninghake, 2001). It is estimated that 0.5 to 2.2% of cases of idiopathic pulmonary fibrosis are familial (Marshall et al., 2000). Pulmonary fibrosis can also be a feature in patients with mutations in the TERT (187270) or the TERC (602322) gene; see PFBMFT1 (614742) and PFBMFT2 (614743). Some patients with surfactant protein C deficiency (610913) who survive to adulthood manifest features of pulmonary fibrosis.
Hermansky-Pudlak syndrome 2
MedGen UID:
374912
Concept ID:
C1842362
Disease or Syndrome
Hermansky-Pudlak syndrome (HPS) is characterized by oculocutaneous albinism, a bleeding diathesis, and, in some individuals, pulmonary fibrosis, granulomatous colitis, or immunodeficiency. Ocular findings include reduced iris pigment with iris transillumination, reduced retinal pigment, foveal hypoplasia with significant reduction in visual acuity (usually in the range of 20/50 to 20/400), nystagmus, and increased crossing of the optic nerve fibers. Hair color ranges from white to brown; skin color ranges from white to olive and is usually a shade lighter than that of other family members. The bleeding diathesis can result in variable bruising, epistaxis, gingival bleeding, postpartum hemorrhage, colonic bleeding, and prolonged bleeding with menses or after tooth extraction, circumcision, and other surgeries. Pulmonary fibrosis, a restrictive lung disease, typically causes symptoms in the early thirties and can progress to death within a decade. Granulomatous colitis is severe in about 15% of affected individuals. Neutropenia and/or immune defects occur primarily in individuals with pathogenic variants in AP3B1 and AP3D1.
Dyskeratosis congenita autosomal recessive 1
MedGen UID:
341705
Concept ID:
C1857144
Disease or Syndrome
Dyskeratosis congenita (DC), a telomere biology disorder, is characterized by a classic triad of dysplastic nails, lacy reticular pigmentation of the upper chest and/or neck, and oral leukoplakia. The classic triad may not be present in all individuals. People with DC are at increased risk for progressive bone marrow failure (BMF), myelodysplastic syndrome (MDS) or acute myelogenous leukemia (AML), solid tumors (usually squamous cell carcinoma of the head/neck or anogenital cancer), and pulmonary fibrosis. Other findings can include: abnormal pigmentation changes not restricted to the upper chest and neck, eye abnormalities (epiphora, blepharitis, sparse eyelashes, ectropion, entropion, trichiasis), and dental abnormalities (caries, periodontal disease, taurodauntism). Although most persons with DC have normal psychomotor development and normal neurologic function, significant developmental delay is present in the two variants in which additional findings include cerebellar hypoplasia (Hoyeraal Hreidarsson syndrome) and bilateral exudative retinopathy and intracranial calcifications (Revesz syndrome). Onset and progression of manifestations of DC vary: at the mild end of the spectrum are those who have only minimal physical findings with normal bone marrow function, and at the severe end are those who have the diagnostic triad and early-onset BMF.
Sarcoidosis 2
MedGen UID:
436694
Concept ID:
C2676468
Finding
Riddle syndrome
MedGen UID:
394368
Concept ID:
C2677792
Disease or Syndrome
RIDDLE is an acronym for the major features of this syndrome: radiosensitivity, immunodeficiency, dysmorphic facies, and learning difficulties (Stewart et al., 2007).
Sarcoidosis 1
MedGen UID:
394568
Concept ID:
C2697310
Finding
Osteomyelitis, sterile multifocal, with periostitis and pustulosis
MedGen UID:
411230
Concept ID:
C2748507
Disease or Syndrome
A rare severe genetic autoinflammatory syndrome characterized by usually neonatal onset of generalized neutrophilic cutaneous pustulosis and severe recurrent multifocal aseptic osteomyelitis with marked periostitis, typically affecting distal ribs, long bones and vertebral bodies. High levels of acute-phase reactants (with no fever associated) and onychosis are frequently observed additional features. Caused by homozygous mutation in the IL1RN gene on chromosome 2q14.
Hermansky-Pudlak syndrome 1
MedGen UID:
419514
Concept ID:
C2931875
Disease or Syndrome
Hermansky-Pudlak syndrome (HPS) is characterized by oculocutaneous albinism, a bleeding diathesis, and, in some individuals, pulmonary fibrosis, granulomatous colitis, or immunodeficiency. Ocular findings include reduced iris pigment with iris transillumination, reduced retinal pigment, foveal hypoplasia with significant reduction in visual acuity (usually in the range of 20/50 to 20/400), nystagmus, and increased crossing of the optic nerve fibers. Hair color ranges from white to brown; skin color ranges from white to olive and is usually a shade lighter than that of other family members. The bleeding diathesis can result in variable bruising, epistaxis, gingival bleeding, postpartum hemorrhage, colonic bleeding, and prolonged bleeding with menses or after tooth extraction, circumcision, and other surgeries. Pulmonary fibrosis, a restrictive lung disease, typically causes symptoms in the early thirties and can progress to death within a decade. Granulomatous colitis is severe in about 15% of affected individuals. Neutropenia and/or immune defects occur primarily in individuals with pathogenic variants in AP3B1 and AP3D1.
Immunodeficiency, ovarian dysgenesis, and pulmonary fibrosis
MedGen UID:
461506
Concept ID:
C3150156
Disease or Syndrome
Lung fibrosis-immunodeficiency-46,XX gonadal dysgenesis syndrome is characterised by immune deficiency, gonadal dysgenesis and fatal lung fibrosis. So far, it has been described in two sisters born to consanguineous parents. Both karyotypes were normal female (46,XX). No genetic anomalies could be identified by comparative genome hybridization analysis of their genomes or by analysis of genes known to be associated with these types of anomalies.
Dyskeratosis congenita, autosomal dominant, 2
MedGen UID:
462793
Concept ID:
C3151443
Disease or Syndrome
Dyskeratosis congenita (DC), a telomere biology disorder, is characterized by a classic triad of dysplastic nails, lacy reticular pigmentation of the upper chest and/or neck, and oral leukoplakia. The classic triad may not be present in all individuals. People with DC are at increased risk for progressive bone marrow failure (BMF), myelodysplastic syndrome (MDS) or acute myelogenous leukemia (AML), solid tumors (usually squamous cell carcinoma of the head/neck or anogenital cancer), and pulmonary fibrosis. Other findings can include: abnormal pigmentation changes not restricted to the upper chest and neck, eye abnormalities (epiphora, blepharitis, sparse eyelashes, ectropion, entropion, trichiasis), and dental abnormalities (caries, periodontal disease, taurodauntism). Although most persons with DC have normal psychomotor development and normal neurologic function, significant developmental delay is present in the two variants in which additional findings include cerebellar hypoplasia (Hoyeraal Hreidarsson syndrome) and bilateral exudative retinopathy and intracranial calcifications (Revesz syndrome). Onset and progression of manifestations of DC vary: at the mild end of the spectrum are those who have only minimal physical findings with normal bone marrow function, and at the severe end are those who have the diagnostic triad and early-onset BMF.
Dyskeratosis congenita, autosomal dominant, 3
MedGen UID:
462795
Concept ID:
C3151445
Disease or Syndrome
Dyskeratosis congenita (DC), a telomere biology disorder, is characterized by a classic triad of dysplastic nails, lacy reticular pigmentation of the upper chest and/or neck, and oral leukoplakia. The classic triad may not be present in all individuals. People with DC are at increased risk for progressive bone marrow failure (BMF), myelodysplastic syndrome (MDS) or acute myelogenous leukemia (AML), solid tumors (usually squamous cell carcinoma of the head/neck or anogenital cancer), and pulmonary fibrosis. Other findings can include: abnormal pigmentation changes not restricted to the upper chest and neck, eye abnormalities (epiphora, blepharitis, sparse eyelashes, ectropion, entropion, trichiasis), and dental abnormalities (caries, periodontal disease, taurodauntism). Although most persons with DC have normal psychomotor development and normal neurologic function, significant developmental delay is present in the two variants in which additional findings include cerebellar hypoplasia (Hoyeraal Hreidarsson syndrome) and bilateral exudative retinopathy and intracranial calcifications (Revesz syndrome). Onset and progression of manifestations of DC vary: at the mild end of the spectrum are those who have only minimal physical findings with normal bone marrow function, and at the severe end are those who have the diagnostic triad and early-onset BMF.
Hermansky-Pudlak syndrome 4
MedGen UID:
483344
Concept ID:
C3484357
Disease or Syndrome
Hermansky-Pudlak syndrome (HPS) is characterized by oculocutaneous albinism, a bleeding diathesis, and, in some individuals, pulmonary fibrosis, granulomatous colitis, or immunodeficiency. Ocular findings include reduced iris pigment with iris transillumination, reduced retinal pigment, foveal hypoplasia with significant reduction in visual acuity (usually in the range of 20/50 to 20/400), nystagmus, and increased crossing of the optic nerve fibers. Hair color ranges from white to brown; skin color ranges from white to olive and is usually a shade lighter than that of other family members. The bleeding diathesis can result in variable bruising, epistaxis, gingival bleeding, postpartum hemorrhage, colonic bleeding, and prolonged bleeding with menses or after tooth extraction, circumcision, and other surgeries. Pulmonary fibrosis, a restrictive lung disease, typically causes symptoms in the early thirties and can progress to death within a decade. Granulomatous colitis is severe in about 15% of affected individuals. Neutropenia and/or immune defects occur primarily in individuals with pathogenic variants in AP3B1 and AP3D1.
Pulmonary fibrosis and/or bone marrow failure, telomere-related, 1
MedGen UID:
766531
Concept ID:
C3553617
Disease or Syndrome
Shortened telomeres can cause a wide variety of clinical features that constitute a phenotypic spectrum. The most severe form is dyskeratosis congenita (see, e.g., 127750), characterized by early childhood onset of skin abnormalities, bone marrow failure, predisposition to malignancy, and risk of pulmonary and hepatic fibrosis. Adult-onset pulmonary fibrosis is the most common manifestation of mutant telomerase genes. Other manifestations include aplastic anemia due to bone marrow failure, hepatic fibrosis, and increased cancer risk, particularly myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML). Phenotype, age at onset, and severity are determined by telomere length, not just telomerase mutation (summary by Armanios, 2009). The genetic diagnosis of telomere-related bone marrow failure and pulmonary fibrosis has implications for treatment because affected individuals generally do not respond to immunosuppression and may be at increased risk for fatal complications after bone marrow or lung transplantation (Parry et al., 2011). Genetic Heterogeneity of Telomere-Related Pulmonary Fibrosis and/or Bone Marrow Failure Also see PFBMFT2 (614743), caused by mutation in the TERC gene (602322) on chromosome 3q26; PFBMFT3 (616373), caused by mutation in the RTEL1 gene (608833) on chromosome 20q13; PFBMFT4 (616371), caused by mutation in the PARN gene (604212) on chromosome 16p13; and PFBMFT5 (618674), caused by mutation in the ZCCHC8 gene (616381) on chromosome 12q24.
Pulmonary fibrosis and/or bone marrow failure, telomere-related, 2
MedGen UID:
766536
Concept ID:
C3553622
Disease or Syndrome
Poikiloderma, hereditary fibrosing, with tendon contractures, myopathy, and pulmonary fibrosis
MedGen UID:
816655
Concept ID:
C3810325
Disease or Syndrome
Hereditary fibrosing poikiloderma with tendon contractures, myopathy, and pulmonary fibrosis (POIKTMP) is characterized by the skin findings of poikiloderma (typically beginning in the first six months and mainly localized to the face), hypohidrosis with heat intolerance, mild lymphedema of the extremities, chronic erythematous and scaly skin lesions on the extremities, sclerosis of the digits, and mild palmoplantar keratoderma. Scalp hair, eyelashes, and/or eyebrows are typically sparse. Muscle contractures are usually seen in childhood and can be present as early as age two years. The majority of affected individuals develop progressive weakness of the proximal and distal muscles of all four limbs. Some adults develop progressive interstitial pulmonary fibrosis, which can be life threatening within three to four years after respiratory symptoms appear. Other features are exocrine pancreatic insufficiency, liver impairment, hematologic abnormalities, relative short stature, and cataract.
Sting-associated vasculopathy, infantile-onset
MedGen UID:
863159
Concept ID:
C4014722
Disease or Syndrome
STING-associated vasculopathy with onset in infancy is an autoinflammatory vasculopathy causing severe skin lesions, particularly affecting the face, ears, nose, and digits, and resulting in ulceration, eschar formation, necrosis, and, in some cases, amputation. Many patients have interstitial lung disease. Tissue biopsy and laboratory findings show a hyperinflammatory state, with evidence of increased beta-interferon (IFNB1; 147640) signaling (summary by Liu et al., 2014).
Pulmonary fibrosis and/or bone marrow failure, telomere-related, 3
MedGen UID:
901644
Concept ID:
C4225346
Disease or Syndrome
Pulmonary fibrosis and/or bone marrow failure, telomere-related, 4
MedGen UID:
903928
Concept ID:
C4225347
Disease or Syndrome
Retinal dystrophy with or without extraocular anomalies
MedGen UID:
934647
Concept ID:
C4310680
Disease or Syndrome
An autosomal recessive condition caused by mutation(s) in the RCBTB1 gene, encoding RCC1 and BTB domain-containing protein 1. It is characterized by severe retinal dystrophy. Associated extraocular abnormalities may or may not be present.
Dyskeratosis congenita, autosomal dominant 1
MedGen UID:
1645250
Concept ID:
C4551974
Disease or Syndrome
Dyskeratosis congenita (DC), a telomere biology disorder, is characterized by a classic triad of dysplastic nails, lacy reticular pigmentation of the upper chest and/or neck, and oral leukoplakia. The classic triad may not be present in all individuals. People with DC are at increased risk for progressive bone marrow failure (BMF), myelodysplastic syndrome (MDS) or acute myelogenous leukemia (AML), solid tumors (usually squamous cell carcinoma of the head/neck or anogenital cancer), and pulmonary fibrosis. Other findings can include: abnormal pigmentation changes not restricted to the upper chest and neck, eye abnormalities (epiphora, blepharitis, sparse eyelashes, ectropion, entropion, trichiasis), and dental abnormalities (caries, periodontal disease, taurodauntism). Although most persons with DC have normal psychomotor development and normal neurologic function, significant developmental delay is present in the two variants in which additional findings include cerebellar hypoplasia (Hoyeraal Hreidarsson syndrome) and bilateral exudative retinopathy and intracranial calcifications (Revesz syndrome). Onset and progression of manifestations of DC vary: at the mild end of the spectrum are those who have only minimal physical findings with normal bone marrow function, and at the severe end are those who have the diagnostic triad and early-onset BMF.
Immunodeficiency 60
MedGen UID:
1681890
Concept ID:
C5193072
Disease or Syndrome
Immunodeficiency-60 (IMD60) is an autosomal dominant primary immunologic disorder characterized by inflammatory bowel disease and recurrent sinopulmonary infections. The age at symptom onset is highly variable, ranging from infancy to mid-adulthood. Laboratory studies show dysregulation of both B and T cells, with variably decreased immunoglobulin production, decreased T-regulatory cells, and overall impaired lymphocyte maturation (summary by Afzali et al., 2017).
Pulmonary fibrosis and/or bone marrow failure, telomere-related, 5
MedGen UID:
1684878
Concept ID:
C5231457
Disease or Syndrome
Individuals with PFBMFT5 have an age-dependent, rapidly progressive phenotype of pulmonary fibrosis and/or bone marrow failure with short telomeres and low levels of TERC (602322), a specialized noncoding RNA that provides the template for telomere repeat addition (Gable et al., 2019).
Fanconi renotubular syndrome 5
MedGen UID:
1711127
Concept ID:
C5394473
Disease or Syndrome
Fanconi renotubular syndrome-5 (FRTS5) is a mitochondrial disorder characterized by proximal renotubular dysfunction from birth, followed by progressive kidney disease and pulmonary fibrosis. It occurs only in individuals of Acadian descent (Crocker et al., 1997 and Hartmannova et al., 2016). For a discussion of genetic heterogeneity of Fanconi renotubular syndrome, see FRTS1 (134600).
Autosomal recessive chronic granulomatous disease 5
MedGen UID:
1710326
Concept ID:
C5394542
Disease or Syndrome
Autosomal recessive chronic granulomatous disease-5 (CGD5) is a primary immunodeficiency characterized by onset of recurrent infections and severe colitis in the first decade of life. Patients often present with features of inflammatory bowel disease and may show granulomata on biopsy. Patients are particularly susceptible to catalase-positive organisms, including Burkholderia cepacia, Legionella, and Candida albicans. Some patients may develop autoinflammatory symptoms, including recurrent fever in the absence of infection, hemolytic anemia, and lymphopenia. Additional features may include short stature, viral infections, cutaneous abscesses, pulmonary infections, and lymphadenitis. Hematopoietic bone marrow transplant is curative. The disorder results from impaired oxidative burst via the NAPDH oxidative complex in macrophages and neutrophils (summary by Arnadottir et al., 2018 and Thomas et al., 2019). For a discussion of genetic heterogeneity of CGD, see the X-linked form (CGDX; 306400).
Immunodeficiency 73b with defective neutrophil chemotaxis and lymphopenia
MedGen UID:
1740566
Concept ID:
C5436549
Disease or Syndrome
Immunodeficiency-73B with defective neutrophil chemotaxis (IMD73B) is an autosomal dominant immunologic disorder characterized by onset of recurrent infections in infancy or early childhood. Affected individuals develop respiratory infections, cellulitis, and severe invasive infections or sepsis; organisms include bacteria such as Staphylococcus, as well as viruses, fungi, and mycobacterial species. Laboratory studies show variable abnormalities, including B- and T-cell lymphopenia, decreased immunoglobulin subsets, decreased TRECs and dysfunctional T cells, decreased NK cells, neutropenia, and impaired neutrophil chemotaxis. Hematopoietic stem cell transplantation is curative (summary by Hsu et al., 2019; review by Lougaris et al., 2020).
Hermansky-Pudlak syndrome 6
MedGen UID:
854714
Concept ID:
C3888007
Disease or Syndrome
Hermansky-Pudlak syndrome (HPS) is characterized by oculocutaneous albinism, a bleeding diathesis, and, in some individuals, pulmonary fibrosis, granulomatous colitis, or immunodeficiency. Ocular findings include reduced iris pigment with iris transillumination, reduced retinal pigment, foveal hypoplasia with significant reduction in visual acuity (usually in the range of 20/50 to 20/400), nystagmus, and increased crossing of the optic nerve fibers. Hair color ranges from white to brown; skin color ranges from white to olive and is usually a shade lighter than that of other family members. The bleeding diathesis can result in variable bruising, epistaxis, gingival bleeding, postpartum hemorrhage, colonic bleeding, and prolonged bleeding with menses or after tooth extraction, circumcision, and other surgeries. Pulmonary fibrosis, a restrictive lung disease, typically causes symptoms in the early thirties and can progress to death within a decade. Granulomatous colitis is severe in about 15% of affected individuals. Neutropenia and/or immune defects occur primarily in individuals with pathogenic variants in AP3B1 and AP3D1.

Recent clinical studies

Etiology

Rumende CM, Susanto EC, Sitorus TP
Acta Med Indones 2021 Apr;53(2):233-241. PMID: 34251354
Li X, Shen C, Wang L, Majumder S, Zhang D, Deen MJ, Li Y, Qing L, Zhang Y, Chen C, Zou R, Lan J, Huang L, Peng C, Zeng L, Liang Y, Cao M, Yang Y, Yang M, Tan G, Tang S, Liu L, Yuan J, Liu Y
Respir Res 2021 Jul 9;22(1):203. doi: 10.1186/s12931-021-01798-6. PMID: 34243776Free PMC Article
Xue M, Zhang T, Chen H, Zeng Y, Lin R, Zhen Y, Li N, Huang Z, Hu H, Zhou L, Wang H, Zhang XD, Sun B
Int J Biol Sci 2021;17(6):1565-1573. Epub 2021 Apr 10 doi: 10.7150/ijbs.58825. PMID: 33907520Free PMC Article
Tanni SE, Fabro AT, de Albuquerque A, Ferreira EVM, Verrastro CGY, Sawamura MVY, Ribeiro SM, Baldi BG
Expert Rev Respir Med 2021 Jun;15(6):791-803. Epub 2021 Apr 27 doi: 10.1080/17476348.2021.1916472. PMID: 33902377
Wang Z, Li X, Chen H, Han L, Ji X, Wang Q, Wei L, Miu Y, Wang J, Mao J, Zhang Z
Aging (Albany NY) 2021 Jan 20;13(3):4605-4616. doi: 10.18632/aging.202420. PMID: 33495418Free PMC Article

Diagnosis

Rumende CM, Susanto EC, Sitorus TP
Acta Med Indones 2021 Apr;53(2):233-241. PMID: 34251354
Li X, Shen C, Wang L, Majumder S, Zhang D, Deen MJ, Li Y, Qing L, Zhang Y, Chen C, Zou R, Lan J, Huang L, Peng C, Zeng L, Liang Y, Cao M, Yang Y, Yang M, Tan G, Tang S, Liu L, Yuan J, Liu Y
Respir Res 2021 Jul 9;22(1):203. doi: 10.1186/s12931-021-01798-6. PMID: 34243776Free PMC Article
Xue M, Zhang T, Chen H, Zeng Y, Lin R, Zhen Y, Li N, Huang Z, Hu H, Zhou L, Wang H, Zhang XD, Sun B
Int J Biol Sci 2021;17(6):1565-1573. Epub 2021 Apr 10 doi: 10.7150/ijbs.58825. PMID: 33907520Free PMC Article
Tanni SE, Fabro AT, de Albuquerque A, Ferreira EVM, Verrastro CGY, Sawamura MVY, Ribeiro SM, Baldi BG
Expert Rev Respir Med 2021 Jun;15(6):791-803. Epub 2021 Apr 27 doi: 10.1080/17476348.2021.1916472. PMID: 33902377
Hino T, Lee KS, Han J, Hata A, Ishigami K, Hatabu H
Korean J Radiol 2021 May;22(5):811-828. Epub 2020 Dec 21 doi: 10.3348/kjr.2020.1132. PMID: 33543848Free PMC Article

Therapy

Wang Q, Liu J, Hu Y, Pan T, Xu Y, Yu J, Xiong W, Zhou Q, Wang Y
Theranostics 2021;11(14):7110-7125. Epub 2021 May 13 doi: 10.7150/thno.61085. PMID: 34093874Free PMC Article
Yuan J, Li P, Pan H, Xu Q, Xu T, Li Y, Wei D, Mo Y, Zhang Q, Chen J, Ni C
Ecotoxicol Environ Saf 2021 Sep 1;220:112372. Epub 2021 May 31 doi: 10.1016/j.ecoenv.2021.112372. PMID: 34082245
Jiang F, Li S, Jiang Y, Chen Z, Wang T, Liu W
Mol Med Rep 2021 Jun;23(6) Epub 2021 Mar 31 doi: 10.3892/mmr.2021.12044. PMID: 33786626Free PMC Article
Zhang P, Wang J, Luo W, Yuan J, Cui C, Guo L, Wu C
Am J Respir Cell Mol Biol 2021 Jul;65(1):54-69. doi: 10.1165/rcmb.2020-0320OC. PMID: 33761308
Zhang C, Wu Z, Li JW, Tan K, Yang W, Zhao H, Wang GQ
J Med Virol 2021 Mar;93(3):1378-1386. Epub 2020 Nov 1 doi: 10.1002/jmv.26634. PMID: 33107641

Prognosis

Li X, Shen C, Wang L, Majumder S, Zhang D, Deen MJ, Li Y, Qing L, Zhang Y, Chen C, Zou R, Lan J, Huang L, Peng C, Zeng L, Liang Y, Cao M, Yang Y, Yang M, Tan G, Tang S, Liu L, Yuan J, Liu Y
Respir Res 2021 Jul 9;22(1):203. doi: 10.1186/s12931-021-01798-6. PMID: 34243776Free PMC Article
Xue M, Zhang T, Chen H, Zeng Y, Lin R, Zhen Y, Li N, Huang Z, Hu H, Zhou L, Wang H, Zhang XD, Sun B
Int J Biol Sci 2021;17(6):1565-1573. Epub 2021 Apr 10 doi: 10.7150/ijbs.58825. PMID: 33907520Free PMC Article
Hino T, Lee KS, Han J, Hata A, Ishigami K, Hatabu H
Korean J Radiol 2021 May;22(5):811-828. Epub 2020 Dec 21 doi: 10.3348/kjr.2020.1132. PMID: 33543848Free PMC Article
Zhang S, Chen H, Yue D, Blackwell TS, Lv C, Song X
J Gene Med 2021 Mar;23(3):e3318. Epub 2021 Feb 11 doi: 10.1002/jgm.3318. PMID: 33533071Free PMC Article
Zhang C, Wu Z, Li JW, Tan K, Yang W, Zhao H, Wang GQ
J Med Virol 2021 Mar;93(3):1378-1386. Epub 2020 Nov 1 doi: 10.1002/jmv.26634. PMID: 33107641

Clinical prediction guides

Li X, Shen C, Wang L, Majumder S, Zhang D, Deen MJ, Li Y, Qing L, Zhang Y, Chen C, Zou R, Lan J, Huang L, Peng C, Zeng L, Liang Y, Cao M, Yang Y, Yang M, Tan G, Tang S, Liu L, Yuan J, Liu Y
Respir Res 2021 Jul 9;22(1):203. doi: 10.1186/s12931-021-01798-6. PMID: 34243776Free PMC Article
Xue M, Zhang T, Chen H, Zeng Y, Lin R, Zhen Y, Li N, Huang Z, Hu H, Zhou L, Wang H, Zhang XD, Sun B
Int J Biol Sci 2021;17(6):1565-1573. Epub 2021 Apr 10 doi: 10.7150/ijbs.58825. PMID: 33907520Free PMC Article
Jiang F, Li S, Jiang Y, Chen Z, Wang T, Liu W
Mol Med Rep 2021 Jun;23(6) Epub 2021 Mar 31 doi: 10.3892/mmr.2021.12044. PMID: 33786626Free PMC Article
Hino T, Lee KS, Han J, Hata A, Ishigami K, Hatabu H
Korean J Radiol 2021 May;22(5):811-828. Epub 2020 Dec 21 doi: 10.3348/kjr.2020.1132. PMID: 33543848Free PMC Article
Chen X, Wu Y, Wang Y, Chen L, Zheng W, Zhou S, Xu H, Li Y, Yuan L, Xiang C
Stem Cell Res Ther 2020 Nov 11;11(1):477. doi: 10.1186/s13287-020-01926-x. PMID: 33176882Free PMC Article

Recent systematic reviews

Koo BS, Park KY, Lee HJ, Kim HJ, Ahn HS, Yim SY, Jun JB
Arthritis Res Ther 2021 Apr 6;23(1):100. doi: 10.1186/s13075-021-02494-y. PMID: 33823923Free PMC Article
Lee JYT, Tikellis G, Corte TJ, Goh NS, Keir GJ, Spencer L, Sandford D, Khor YH, Glaspole I, Price J, Hey-Cunningham AJ, Maloney J, Teoh AKY, Watson AL, Holland AE
Eur Respir Rev 2020 Jun 30;29(156) Epub 2020 Apr 29 doi: 10.1183/16000617.0125-2019. PMID: 32350085
Igai Y
Jpn J Nurs Sci 2019 Jul;16(3):241-252. Epub 2018 Nov 14 doi: 10.1111/jjns.12242. PMID: 30430734
Bahri S, Ben Ali R, Abidi A, Jameleddine S
Biomed Pharmacother 2017 Sep;93:666-673. Epub 2017 Jul 5 doi: 10.1016/j.biopha.2017.06.052. PMID: 28688290
Srour N, Thébaud B
Stem Cells Transl Med 2015 Dec;4(12):1500-10. Epub 2015 Oct 22 doi: 10.5966/sctm.2015-0121. PMID: 26494779Free PMC Article

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