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Necrotizing pulmonary granulomatosis

MedGen UID:
1785597
•Concept ID:
C5539676
•
Disease or Syndrome
Synonym: Caseating pulmonary granulomatosis
 
HPO: HP:0033550

Definition

A granuloma that is associated with necrotic changes. Caseation necrosis is defined as a region in granulomas with eosinophilic, granular and cheese-like cellular debris with necrosis. The word caseous itself means pertaining or related to cheese, and comes from the Latin word caseus, meaning cheese. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNecrotizing pulmonary granulomatosis

Professional guidelines

PubMed

Pulmonary vasculitis: diagnosis and treatment.
Anaev EK, Baranova IA, Belevsky AS
Ter Arkh 2018 Apr 19;90(3):99-106. doi: 10.26442/terarkh201890399-106. PMID: 30701864
Granulomatosis with polyangiitis (Wegener): clinical aspects and treatment.
Comarmond C, Cacoub P
Autoimmun Rev 2014 Nov;13(11):1121-5. Epub 2014 Aug 20 doi: 10.1016/j.autrev.2014.08.017. PMID: 25149391
EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria.
Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, Buoncompagni A, Lazar C, Bilge I, Uziel Y, Rigante D, Cantarini L, Hilario MO, Silva CA, Alegria M, Norambuena X, Belot A, Berkun Y, Estrella AI, Olivieri AN, Alpigiani MG, Rumba I, Sztajnbok F, Tambic-Bukovac L, Breda L, Al-Mayouf S, Mihaylova D, Chasnyk V, Sengler C, Klein-Gitelman M, Djeddi D, Nuno L, Pruunsild C, Brunner J, Kondi A, Pagava K, Pederzoli S, Martini A, Ruperto N; Paediatric Rheumatology International Trials Organisation (PRINTO)
Ann Rheum Dis 2010 May;69(5):798-806. doi: 10.1136/ard.2009.116657. PMID: 20413568

Recent clinical studies

Etiology

Childhood-Onset ANCA- Associated Vasculitis: single center experience from Central California.
Singh D, Sukumaran S
Pediatr Rheumatol Online J 2023 Jul 3;21(1):66. doi: 10.1186/s12969-023-00853-4. PMID: 37400890Free PMC Article
Diagnosing and treating ANCA-associated vasculitis: an updated review for clinical practice.
Chevet B, Cornec D, Casal Moura M, Cornec-Le Gall E, Fervenza FC, Warrington KJ, Specks U, Berti A
Rheumatology (Oxford) 2023 May 2;62(5):1787-1803. doi: 10.1093/rheumatology/keac623. PMID: 36315063
Pulmonary vasculitis: diagnosis and treatment.
Anaev EK, Baranova IA, Belevsky AS
Ter Arkh 2018 Apr 19;90(3):99-106. doi: 10.26442/terarkh201890399-106. PMID: 30701864
EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria.
Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, Buoncompagni A, Lazar C, Bilge I, Uziel Y, Rigante D, Cantarini L, Hilario MO, Silva CA, Alegria M, Norambuena X, Belot A, Berkun Y, Estrella AI, Olivieri AN, Alpigiani MG, Rumba I, Sztajnbok F, Tambic-Bukovac L, Breda L, Al-Mayouf S, Mihaylova D, Chasnyk V, Sengler C, Klein-Gitelman M, Djeddi D, Nuno L, Pruunsild C, Brunner J, Kondi A, Pagava K, Pederzoli S, Martini A, Ruperto N; Paediatric Rheumatology International Trials Organisation (PRINTO)
Ann Rheum Dis 2010 May;69(5):798-806. doi: 10.1136/ard.2009.116657. PMID: 20413568
Epidemiology of systemic vasculitis.
Lane SE, Watts R, Scott DG
Curr Rheumatol Rep 2005 Aug;7(4):270-5. doi: 10.1007/s11926-005-0036-5. PMID: 16045829

Diagnosis

Diagnosing and treating ANCA-associated vasculitis: an updated review for clinical practice.
Chevet B, Cornec D, Casal Moura M, Cornec-Le Gall E, Fervenza FC, Warrington KJ, Specks U, Berti A
Rheumatology (Oxford) 2023 May 2;62(5):1787-1803. doi: 10.1093/rheumatology/keac623. PMID: 36315063
Pathology of Granulomatous Pulmonary Diseases.
Rosen Y
Arch Pathol Lab Med 2022 Jan 2;146(2):233-251. doi: 10.5858/arpa.2020-0543-RA. PMID: 33905479
Sarcoidosis: A Clinical Overview from Symptoms to Diagnosis.
Sève P, Pacheco Y, Durupt F, Jamilloux Y, Gerfaud-Valentin M, Isaac S, Boussel L, Calender A, Androdias G, Valeyre D, El Jammal T
Cells 2021 Mar 31;10(4) doi: 10.3390/cells10040766. PMID: 33807303Free PMC Article
Lung involvement in ANCA-associated vasculitis.
Sacoto G, Boukhlal S, Specks U, Flores-Suárez LF, Cornec D
Presse Med 2020 Oct;49(3):104039. Epub 2020 Jul 7 doi: 10.1016/j.lpm.2020.104039. PMID: 32650042
Cavitary Lung Diseases: A Clinical-Radiologic Algorithmic Approach.
Gafoor K, Patel S, Girvin F, Gupta N, Naidich D, Machnicki S, Brown KK, Mehta A, Husta B, Ryu JH, Sarosi GA, Franquet T, Verschakelen J, Johkoh T, Travis W, Raoof S
Chest 2018 Jun;153(6):1443-1465. Epub 2018 Mar 6 doi: 10.1016/j.chest.2018.02.026. PMID: 29518379

Therapy

Proteinase 3-specific antineutrophil cytoplasmic antibody-associated vasculitis.
Falde SD, Fussner LA, Tazelaar HD, O'Brien EK, Lamprecht P, Konig MF, Specks U
Lancet Rheumatol 2024 May;6(5):e314-e327. Epub 2024 Apr 1 doi: 10.1016/S2665-9913(24)00035-3. PMID: 38574742
Diagnosing and treating ANCA-associated vasculitis: an updated review for clinical practice.
Chevet B, Cornec D, Casal Moura M, Cornec-Le Gall E, Fervenza FC, Warrington KJ, Specks U, Berti A
Rheumatology (Oxford) 2023 May 2;62(5):1787-1803. doi: 10.1093/rheumatology/keac623. PMID: 36315063
Granulomatosis with polyangiitis (Wegener's).
Puéchal X
Joint Bone Spine 2020 Dec;87(6):572-578. Epub 2020 Jun 17 doi: 10.1016/j.jbspin.2020.06.005. PMID: 32562697
Granulomatosis with polyangiitis (Wegener): clinical aspects and treatment.
Comarmond C, Cacoub P
Autoimmun Rev 2014 Nov;13(11):1121-5. Epub 2014 Aug 20 doi: 10.1016/j.autrev.2014.08.017. PMID: 25149391
Pulmonary vasculitis.
Leavitt RY, Fauci AS
Am Rev Respir Dis 1986 Jul;134(1):149-66. doi: 10.1164/arrd.1986.134.1.149. PMID: 2873770

Prognosis

Diagnosing and treating ANCA-associated vasculitis: an updated review for clinical practice.
Chevet B, Cornec D, Casal Moura M, Cornec-Le Gall E, Fervenza FC, Warrington KJ, Specks U, Berti A
Rheumatology (Oxford) 2023 May 2;62(5):1787-1803. doi: 10.1093/rheumatology/keac623. PMID: 36315063
Granulomatosis with polyangiitis (Wegener's).
Puéchal X
Joint Bone Spine 2020 Dec;87(6):572-578. Epub 2020 Jun 17 doi: 10.1016/j.jbspin.2020.06.005. PMID: 32562697
Granulomatosis with polyangiitis (Wegener): clinical aspects and treatment.
Comarmond C, Cacoub P
Autoimmun Rev 2014 Nov;13(11):1121-5. Epub 2014 Aug 20 doi: 10.1016/j.autrev.2014.08.017. PMID: 25149391
Pulmonary capillaritis.
Franks TJ, Koss MN
Curr Opin Pulm Med 2000 Sep;6(5):430-5. doi: 10.1097/00063198-200009000-00008. PMID: 10958235
Pulmonary vasculitis.
Leavitt RY, Fauci AS
Am Rev Respir Dis 1986 Jul;134(1):149-66. doi: 10.1164/arrd.1986.134.1.149. PMID: 2873770

Clinical prediction guides

The Role and Pitfall of F18-FDG PET/CT in Surveillance of High Grade Pulmonary Lymphomatoid Granulomatosis.
Yang M, Rosenthal AC, Ashman JB, Craig FE
Curr Probl Diagn Radiol 2021 May-Jun;50(3):443-449. Epub 2019 Feb 10 doi: 10.1067/j.cpradiol.2019.02.002. PMID: 30826140
Pathogenesis of ANCA-Associated Pulmonary Vasculitis.
Alba MA, Jennette JC, Falk RJ
Semin Respir Crit Care Med 2018 Aug;39(4):413-424. Epub 2018 Nov 7 doi: 10.1055/s-0038-1673386. PMID: 30404109Free PMC Article
Granulomatosis with polyangiitis (Wegener): clinical aspects and treatment.
Comarmond C, Cacoub P
Autoimmun Rev 2014 Nov;13(11):1121-5. Epub 2014 Aug 20 doi: 10.1016/j.autrev.2014.08.017. PMID: 25149391
Coexistent pulmonary granulomatosis with polyangiitis (Wegener granulomatosis) and Crohn disease.
Vaszar LT, Orzechowski NM, Specks U, Ytterberg SR, Loftus EV Jr, Mark EJ, Tazelaar HD
Am J Surg Pathol 2014 Mar;38(3):354-9. doi: 10.1097/PAS.0000000000000135. PMID: 24525506
Vasculopathy and pulmonary arterial hypertension.
Guillevin L
Rheumatology (Oxford) 2009 Jun;48 Suppl 3:iii54-7. doi: 10.1093/rheumatology/ken484. PMID: 19487226

Recent systematic reviews

Efficacy of Rituximab on Refractory Organ Involvements in Granulomatosis with Polyangiitis: A Systematic Review of Case Reports.
Hajishah H, Amini MJ, Chadeganipour AS, Salehi SA, Kazemi D
Curr Rheumatol Rev 2024;20(3):270-283. doi: 10.2174/0115733971263964231020072640. PMID: 37907486

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