Eosinophilic granulomatosis with polyangiitis in comorbidpatient: is the immunological diagnostics always unambiguous

Eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg—Strauss syndrome, represents a rare form of ANCA-associated necrotising vasculitis which affects small vessels. This disease is characterized by typical combination of immunological disturbances, hypereosinophilia, severe...

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Autores principales:	V. N. Marchenko, E. A. Bruchkus, A. A. Lebedeva, D. A. Davydov, N. L. Shaporova, O. V. Dudina, O. A. Smul’skaya, M. K. Zinakova
Formato:	article
Lenguaje:	RU
Publicado:	SPb RAACI 2020
Materias:	еosinophilic granulomatosis with polyangiitis churg-strauss syndrome immunological diagnostics anca ancaassociated Immunologic diseases. Allergy RC581-607
Acceso en línea:	https://doaj.org/article/1c89ad6d0c4f462da77115e0347432ce
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spelling	oai:doaj.org-article:1c89ad6d0c4f462da77115e0347432ce2021-11-18T08:03:49ZEosinophilic granulomatosis with polyangiitis in comorbidpatient: is the immunological diagnostics always unambiguous1563-06252313-741X10.15789/1563-0625-EGW-1925https://doaj.org/article/1c89ad6d0c4f462da77115e0347432ce2020-04-01T00:00:00Zhttps://www.mimmun.ru/mimmun/article/view/1925https://doaj.org/toc/1563-0625https://doaj.org/toc/2313-741XEosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg—Strauss syndrome, represents a rare form of ANCA-associated necrotising vasculitis which affects small vessels. This disease is characterized by typical combination of immunological disturbances, hypereosinophilia, severe bronchial asthma, transient pulmonary infiltrates, and kidney injury which is less frequent than in other forms of necrotising vasculitis.Verification of the diagnosis is often hampered by comorbidities, such as chronic obstructive pulmonary disease (COPD) in the patients with long-term smoking history and occupational hazards.In this article, we report a clinical case of EGPA in elderly patient with preceding COPD which caused diagnostic difficulties for this eosinophilic syndrome. Clinical pattern at the beginning of disease was presented by moderate inspiratory dyspnea and cough with small amounts of mucus sputum, which appeared after longterm exposure to chlorine-containing substances. COPD diagnosis in this patient was based on clinical pattern, long smoking experience, and occupational hazards. However, persistence of the symptoms during the ongoing therapy, as well as multidirectional dynamics of transient pulmonary infiltrates found on repeated CT-scans, prompted us to intensify diagnostic search for a systemic disease. Clinical, laboratory and instrumental signs of bronchial asthma were revealed, as well as hypereosinophilia and sensory polyneuropathy, which, if combined with CT-scan data, allowed us to prove the EGPA diagnosis.This case shows that, despite great value of immune diagnostics, with negative blood tests for ANCA, it is necessary to detect mutually complicating comorbid pathology. EGPA was considered the basic diagnosis, and COPD as accompanying disorder, taking into account such reasons as an unfavorable prognosis for EGPA and the need for long-term chemotherapy with systemic corticosteroids and monoclonal antibodies. ANCA-negative testing in the patient, absence of severe kidney and skin lesions allows to suggest better clinical prognosis in this patient.V. N. MarchenkoE. A. BruchkusA. A. LebedevaD. A. DavydovN. L. ShaporovaO. V. DudinaO. A. Smul’skayaM. K. ZinakovaSPb RAACIarticleеosinophilic granulomatosis with polyangiitischurg-strauss syndromeimmunological diagnosticsancaancaassociatedImmunologic diseases. AllergyRC581-607RUMedicinskaâ Immunologiâ, Vol 22, Iss 2, Pp 383-392 (2020)
institution	DOAJ
collection	DOAJ
language	RU
topic	еosinophilic granulomatosis with polyangiitis churg-strauss syndrome immunological diagnostics anca ancaassociated Immunologic diseases. Allergy RC581-607
spellingShingle	еosinophilic granulomatosis with polyangiitis churg-strauss syndrome immunological diagnostics anca ancaassociated Immunologic diseases. Allergy RC581-607 V. N. Marchenko E. A. Bruchkus A. A. Lebedeva D. A. Davydov N. L. Shaporova O. V. Dudina O. A. Smul’skaya M. K. Zinakova Eosinophilic granulomatosis with polyangiitis in comorbidpatient: is the immunological diagnostics always unambiguous
description	Eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg—Strauss syndrome, represents a rare form of ANCA-associated necrotising vasculitis which affects small vessels. This disease is characterized by typical combination of immunological disturbances, hypereosinophilia, severe bronchial asthma, transient pulmonary infiltrates, and kidney injury which is less frequent than in other forms of necrotising vasculitis.Verification of the diagnosis is often hampered by comorbidities, such as chronic obstructive pulmonary disease (COPD) in the patients with long-term smoking history and occupational hazards.In this article, we report a clinical case of EGPA in elderly patient with preceding COPD which caused diagnostic difficulties for this eosinophilic syndrome. Clinical pattern at the beginning of disease was presented by moderate inspiratory dyspnea and cough with small amounts of mucus sputum, which appeared after longterm exposure to chlorine-containing substances. COPD diagnosis in this patient was based on clinical pattern, long smoking experience, and occupational hazards. However, persistence of the symptoms during the ongoing therapy, as well as multidirectional dynamics of transient pulmonary infiltrates found on repeated CT-scans, prompted us to intensify diagnostic search for a systemic disease. Clinical, laboratory and instrumental signs of bronchial asthma were revealed, as well as hypereosinophilia and sensory polyneuropathy, which, if combined with CT-scan data, allowed us to prove the EGPA diagnosis.This case shows that, despite great value of immune diagnostics, with negative blood tests for ANCA, it is necessary to detect mutually complicating comorbid pathology. EGPA was considered the basic diagnosis, and COPD as accompanying disorder, taking into account such reasons as an unfavorable prognosis for EGPA and the need for long-term chemotherapy with systemic corticosteroids and monoclonal antibodies. ANCA-negative testing in the patient, absence of severe kidney and skin lesions allows to suggest better clinical prognosis in this patient.
format	article
author	V. N. Marchenko E. A. Bruchkus A. A. Lebedeva D. A. Davydov N. L. Shaporova O. V. Dudina O. A. Smul’skaya M. K. Zinakova
author_facet	V. N. Marchenko E. A. Bruchkus A. A. Lebedeva D. A. Davydov N. L. Shaporova O. V. Dudina O. A. Smul’skaya M. K. Zinakova
author_sort	V. N. Marchenko
title	Eosinophilic granulomatosis with polyangiitis in comorbidpatient: is the immunological diagnostics always unambiguous
title_short	Eosinophilic granulomatosis with polyangiitis in comorbidpatient: is the immunological diagnostics always unambiguous
title_full	Eosinophilic granulomatosis with polyangiitis in comorbidpatient: is the immunological diagnostics always unambiguous
title_fullStr	Eosinophilic granulomatosis with polyangiitis in comorbidpatient: is the immunological diagnostics always unambiguous
title_full_unstemmed	Eosinophilic granulomatosis with polyangiitis in comorbidpatient: is the immunological diagnostics always unambiguous
title_sort	eosinophilic granulomatosis with polyangiitis in comorbidpatient: is the immunological diagnostics always unambiguous
publisher	SPb RAACI
publishDate	2020
url	https://doaj.org/article/1c89ad6d0c4f462da77115e0347432ce
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_version_	1718422336409960448

Eosinophilic granulomatosis with polyangiitis in comorbidpatient: is the immunological diagnostics always unambiguous

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