Diffuse alveolar hemorrhage as the onset of ANCA associated vasculitis: Diagnostic dilemma and tactics of management
Abstract. ANCA associated vasculites (AAV) are rare and potentially fatal diseases, especially when they are beginning with diffuse alveolar hemorrhage. Current article presents a complicated case of AAV diagnosing as bilateral pneumonia, when a 38-year-old female patient had a fulminant onset of vasculitis accompanied by massive hemoptysis and respiratory failure. Ineffectiveness of antibiotics and appearance of extrapulmonary symptoms (hematuria, cutaneous vasculitis, sinusitis) prompted the physicians to change their diagnostic approach. After excluding infections, oncologic pathology and verifying microscopic polyangiitis (specific myeloperoxidase antibody levels > 187 U/ml), the patient was prescribed induction therapy with methylprednisolone and cyclophosphamide pulses, which produced a rapid positive effect. This clinical observation highlights the importance of including AAV in the differential diagnosis list in case of diffuse alveolar hemorrhage of unknown etiology. A comprehensive estimation of multisystem damage and early immunological testing are essential for the timely diagnosis of AAV, while standard therapy is highly effective even in fulminant cases of the disease.Anikin D.A., Demko I.V., Krasovskaya I.S., Stepanenko S.S.
Keywords
ANCA associated vasculitis
microscopic polyangiitis
diffuse alveolar hemorrhage
pulmonary hemorrhage
anti-myeloperoxidase antibodies (anti-MPO)
glomerulonephritis
cyclophosphamide
References
1. Анаев Э.Х., Баранова И.А., Белевский А.С. Легочные васкулиты: диагностика и лечение. Терапевтический архив. 2018;90(3):99–106. (Anaev EK, Baranova IA, Belevsky AS. Pulmonary vasculitis: Diagnosis and treatment. Terapevticheskii arkhiv = Therapeutic Archive. 2018;90(3):99–106 (In Russ.)). https://doi.org/10.26442/terarkh201890399-106
2. Зайцева А.С., Степанян И.Э., Шабалина И.Ю., Дмитриева Л.И., Шмелев Е.И. Редкий вариант АНЦА-ассоциированного гранулематоза с полиангиитом, осложненного диффузной альвеолярной геморрагией. Пульмонология. 2019;29(2):229–234. (Zaytseva AS, Stepanyan IE, Shabalina IYu, Dmitriyeva LI, Shmelev EI. A rare variant of ANCA-associated eosinophilic granulomatosis with polyangiitis complicated by diffuse alveolar hemorrage. Pulmonologiya = Russian Pulmonology Journal. 2019;29(2):229–234 (In Russ.)).
https://doi.org/10.18093/0869-0189-2019-29-2-229-234
3. Анаев Э.Х., Княжеская Н.П. Эозинофильный гранулематоз с полиангиитом: диагностические и терапевтические проблемы. Пульмонология. 2023;33(4):542–551. (Anaev EKh, Kniajeskaia NP. Eosinophilic granulomatosis with polyangiitis: Diagnosis and treatment-related issues. Pulmonologiya = Russian Pulmonology Journal. 2023;33(4):542–551 (In Russ.)).
https://doi.org/10.18093/0869-0189-2022-4118
4. Соловьянова Е.Н., Боровков Н.Н., Маслова Т.И., Линева Н.Ю., Пащенко А.Д. АНЦА-ассоциированные системные васкулиты в клинической практике. Терапия. 2016;2(6):54–61. (Solovianova YeN, Borovkov NN, Maslova TI, Lineva NYu, Pashchenko AD. ANCA-associated systemic vasculitis in clinical practice. Terapiya = Therapy. 2016;2(6):54–61 (In Russ.)).
5. Байрашевская А.В., Дегтярова Н.Д., Раденска-Лоповок С.Г. АНЦА-ассоциированные васкулиты. Архив патологии. 2022;84(1):50–58. (Bayrashevskaya AV, Degtyareva ND, Radenska-Lopovok SG. ANCA-associated small-vessel vasculitides. Arkhiv patologii = Russian Journal of Archive of Pathology. 2022;84(1):50–58 (In Russ.)).
https://doi.org/10.17116/patol20228401150
6. Molnar A, Studinger P, Ledo N. Diagnostic and therapeutic approach in ANCA-associated glomerulonephritis: A review on management strategies. Front Med (Lausanne). 2022;9:884188.
PMID: 35721093. https://doi.org/10.3389/fmed.2022.884188
7. Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016;75(9):1583–94.
PMID: 27338776. https://doi.org/10.1136/annrheumdis-2016-209133corr2
8. Самсонова М.В., Черняев А.Л. Гранулематозные заболевания легких. Пульмонология. 2017;27(2):250–261. (Samsonova MV, Chernyaev AL. Granulomatous lung diseases. Pulmonologiya = Russian Pulmonology Journal. 2017;27(2):250–261 (In Russ.)).
https://doi.org/10.18093/0869-0189-2017-27-2-250-261
9. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1–11.
PMID: 23045170. https://doi.org/10.1002/art.37715
10. Schwarz MI, Brown KK. Small vessel vasculitis of the lung. Thorax. 2000;55(6):502–10.
PMID: 10817800. https://doi.org/10.1136/thorax.55.6.502
11. Sokolowska BM, Szczeklik WK, Wludarczyk AA, Kuczia PP, Jakiela BA, Gasior JA et al. ANCA-positive and ANCA-negative phenotypes of eosinophilic granulomatosis with polyangiitis (EGPA): Outcome and long-term follow-up of 50 patients from a single Polish center. Clin Exp Rheumatol. 2014; 32(3 Suppl 82):S41–47. PMID: 24854371.
12. Kitching AR, Anders HJ, Basu N, Brouwer E, Gordon J, Jayne DR et al. ANCA-associated vasculitis. Nat Rev Dis Primers. 2020;6(1):71.
PMID: 32855422. https://doi.org/10.1038/s41572-020-0204-y
13. Sanchez Alamo B, Moi L, Bajema I, Faurschou M, Flossmann O, Hauser T et al. Long-term outcomes and prognostic factors for survival of patients with ANCA-associated vasculitis. Nephrol Dial Transplant. 2023;38(7):1655–65.
PMID: 36617233. https://doi.org/10.1093/ndt/gfac320
About the Authors
Dmitry A. Anikin, MD, assistant at the Department of hospital therapy and immunology with postgraduate education course, Professor V.F. Voyno-Yasenetsky Krasnoyarsk State Medical University of the Ministry of Healthcare of Russia; pulmonologist at the Department of pulmonology, Regional Clinical Hospital (Krasnoyarsk). Address: 660022, Krasnoyarsk, 1 Partizana Zheleznyaka St.E-mail: anikin27111994@mail.ru
ORCID: https://orcid.org/0000-0002-1598-436X. eLibrary SPIN: 3045-8493
Irina V. Demko, MD, Dr. Sci. (Medicine), professor, head of the Department of hospital therapy and immunology with postgraduate education course, Professor V.F. Voyno-Yasenetsky Krasnoyarsk State Medical University of the Ministry of Healthcare of Russia; head of the Pulmonary Allergological Center at the Regional Clinical Hospital (Krasnoyarsk). Address: 660022, Krasnoyarsk, 1 Partizana Zheleznyaka St.
E-mail: demko64@mail.ru
ORCID: https://orcid.org/0000-0001-8982-5292. eLibrary SPIN: 6520-3233
Irina S. Krasovskaya, MD, head of the Department of pulmonology, Regional Clinical Hospital (Krasnoyarsk). Address: 660022, Krasnoyarsk, 3A Partizana Zheleznyaka St.
E-mail: family-duck@mail.ru
ORCID: https://orcid.org/0009-0001-1249-0292
Sofia S. Stepanenko, student of the group 602 of the Faculty of general medicine, Professor V.F. Voyno-Yasenetsky Krasnoyarsk State Medical University of the Ministry of Healthcare of Russia. Address: 660022, Krasnoyarsk, 1 Partizana Zheleznyaka St.
E-mail: sonia.02@mail.ru
ORCID: https://orcid.org/0009-0006-2456-9498
Similar Articles
