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Case Reports
. 2021 Feb;42(1):95-101.
doi: 10.1007/s00292-020-00904-7. Epub 2021 Jan 25.

[IgG4-associated lung disease with granulomatous lesions : Coexistence of two entities or the spectrum of one disease?]

[Article in German]
Christiane Kümpers  1 , Lars Tharun  2 , Florian Stellmacher  3 , Ulf Greinert  4 , Katharina May  5 , Sven Perner  2   3   6
Affiliations
Case Reports

[IgG4-associated lung disease with granulomatous lesions : Coexistence of two entities or the spectrum of one disease?]

[Article in German]
Christiane Kümpers et al. Pathologe. 2021 Feb.

Abstract
in English, German

A 54-year-old patient with a history of pulmonary tuberculosis and occupational exposure to dust in early adulthood presented with symptoms of coughing with sputum, weight loss, occasional night sweats, and thoracic pain. Non-necrotizing granulomatosis in lung and lymph-node biopsies indicated sarcoidosis. Combined immunosuppressive therapy did not lead to an improvement. An atypical lung resectate with fibroinflammatory changes and obliterative endothelialitis may finally lead to the diagnosis of IgG4-associated lung disease with a bronchovascular pattern of involvement. The question discussed here is whether this is a coexistence of IgG4-associated lung disease with sarcoidosis or the spectrum of one disease.

Ein 54 Jahre alter Patient mit anamnestisch im frühen Erwachsenenalter nicht austherapierter Lungentuberkulose und beruflicher Staubexposition wird mit dem Beschwerdebild von Husten mit Auswurf, Gewichtsverlust, gelegentlichem Nachtschweiß und thorakalen Schmerzen vorstellig. Eine nichtnekrotisierende Granulomatose in Lungen- und Lymphknotenbiopsaten weist auf eine Sarkoidose hin. Unter kombinierter immunsuppressiver Therapie kommt es zu keiner Befundbesserung. Anhand eines atypischen Lungenresektates mit fibroinflammatorischen Veränderungen und obliterativer Endothelialitis kann schlussendlich eine IgG4-assoziierte Lungenerkrankung vom bronchovaskulären Typ diagnostiziert werden. Besprochen wird hier die Frage, ob es sich um eine Koexistenz einer IgG4-assoziierten Lungenerkrankung mit einer Sarkoidose handelt oder um das Spektrum einer Erkrankung.

Keywords: Fibroinflammatory changes; Granulomatosis; IgG4; Obliterative endothelialitis; Sarcoidosis.

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References

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