Systemic ANCA-vasculitis-associated granulomatosis with polyangiitisina 16-year-old teenage girl

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Introduction: ANCA (antineutrophil cytoplasmic antibodies) associated vasculitis is a group of relatively rare autoimmune diseases of unknown etiology characterized by immune inflammation and vascular wall necrosis [1]. Vessels of predominantly small caliber are affected, and the lungs and kidneys are often involved in the pathological process. Due to the rarity of ANCA-associated systemic vasculitis, as a rule, are diagnosed at later stages of the disease, which leads to rapid disability of patients and high mortality due to aggressive damage to the respiratory tract, lungs and the development of renal failure [2]. Pathogenetic therapy is based on the use of various combinations of immunosuppressive drugs to suppress the pathological immune response. There are traditionally two phases in treatment: induction of remission, aimed at rapidly suppressing the activity of systemic inflammation and clinical manifestations of the disease, and maintenance of remission, the purpose of which is to prevent exacerbations in patients who have achieved remission [3].
The purpose of the work: to demonstrate the difficulties of diagnosis and treatment of a 16-year-old teenager with systemic ANCA-associated vasculitis by the example of a clinical case.
Materials and methods of the study: the analysis of the medical documentation of a patient who was undergoing examination and treatment in the cardiorheumatology department of the Medical school "VODKB No. 1" in Voronezh was carried out
The results of the study: In October 2023, a 16-year-old girl was admitted to the VODKB No. 1 Medical School with complaints of fever, chills, nasal congestion, difficulty in nasal breathing, persistent cough, fatigue, and slight hearing loss.
A child from the first pregnancy, which proceeded without peculiarities. Body weight at birth 3200 g, length 52 cm. She grew and developed according to her age. Preventive vaccinations were carried out according to the calendar. Previous illnesses: SARS is rare, she did not suffer from childhood infections. The allergic history is not burdened. Hereditary diseases are denied.
Anamnesis of the disease: episodes of nasal congestion up to several days were noted during the year. An unpleasant smell from the nose appeared periodically. She was observed by an otorhinolaryngologist in a polyclinic at her place of residence with a diagnosis of rhinosinusitis, and symptomatic therapy was performed. At the end of September 2023, the girl's condition deteriorated sharply. The temperature rose to febrile figures, a dry cough appeared, difficulty in nasal breathing. She was being treated in an infectious diseases hospital. An X-ray of the chest revealed changes of an infiltrative nature, Consulted by a phthisiologist - there is no data for the tuberculosis process. She was consulted by an otorhinolaryngologist-left-sided acute rhinosinusitis, catarrhal exudative otitis media on the left; ulceration of the nasal mucosa. She received antibacterial therapy (ceftriaxone, cefopyrazone-sulbactam, linezolid, meronem) without positive dynamics. Infectious diseases were excluded. Procalcitonin is less than 0.5 ng/ml. With suspicion of ANCA-associated vasculitis granulomatosis with polyangiitis, she was transferred to the VODKB Nº l Medical Center.
Objectively: The condition is serious. Fever, intoxication, damage to the nasal mucosa — sucreous crusts, bleeding, difficulty in nasal breathing, hearing loss, obsessive cough.
The skin is clean and pale. The mucous membranes are clean and pink. There are hemorrhagic crusts in the nasal passages. The pharynx is not hyperemic. Peripheral lymph nodes of all groups are small and painless. There is no swelling. Nasal breathing is difficult. Breathing is weakened in the lungs. The boundaries of the heart are within the normal range. The tones are clear, rhythmic. Heart rate – 84 beats / min; blood pressure 115/70 mmHg. The abdomen is soft, painless. The liver and spleen are not enlarged. Urination is not impaired. The chair is decorated.
Survey results:
total blood count (UAC) - leukocytes 20.1x109/l, neutrophils- 83.8%, lymphocytes- 7.2%, monocytes- 8.6%, eosinophils- 0%, basophils- 0.4%, erythrocytes- 3.89x1012/L, hemoglobin- 94 g/L, platelets- 530x103/L, ESR- 54 mm/h;
biochemical blood analysis: total protein- 64.5, LDH total- 291.6 units / l, urea-2.6 mmol/ L, creatinine- 38 mmol/ L, glucose- 4.81 mmol/L, AlAT-524.1 units / l, AsAT- 851.7 units /l, total bilirubin- 4.6 mmol /l, CRP-103.7 mg/l, RF-41.8 IU/ml, ASL-O- 329.8 IU/ml;
general urinalysis: transparent, pH 7.0, relative density 1015, protein —no, erythrocytes — no, leukocytes — no;
coagulogram- ACTV- 21.8 sec, PV- 12.6 sec, PTI- 78.7%, INR-1.07, fibrinogen-511.7 mg%.
Immune status: Complement – From 3 to 1.44 units, From 4 to 0.25 units, Ig A – 2.29 g/l; Ig G -13.51 g/l; Ig M – 1.42 g/l; Ig E – 1020 IU/ml;
ANCAcombi: Anti-PR3 reaction is questionable, Anti-MPO negative, Anti-BPI negative, Anti-Elastase positive, Anti-Catheepsin G negative, Anti-Lysozym negative, Anti-Lactoferin positive; Antibodies: pANCA ≤ 2.0 (N-20), cANCA 68.8 (N-20).
Radiography of the paranasal sinuses: X-ray-signs of impaired airiness of the left maxillary sinus, changes in the density of the nasal concha.
Chest X-ray: X-ray-signs of bilateral multiple globular lung lesion with signs of disintegration, (Figure 1).
CT scan of the chest organs: CT scans show signs of multiple infiltrates of both lungs, most of which have decay zones. signs of an indistinct hydropericardium.
Ultrasound of the liver and kidneys: ULTRASOUND- signs of diffuse changes in the liver, moderate diffuse changes in the kidneys, impoverishment of blood flow with CDK in the cortical layer of both kidneys.
Taking into account the following criteria: damage to the paranasal sinuses with ulceration of the mucous membrane and the formation of saddle deformity of the nose, hearing organ (otitis media), lungs, increased titers of cANCA antibodies, the diagnosis was established: Systemic ANCA associated vasculitis – granulomatosis with polyangiitis. Damage to the upper respiratory tract, lungs. Liver damage. A high degree of activity.
The girl started immunosuppressive therapy: pulse therapy with methylprednisolone No. 3 followed by oral administration of prednisolone, pulse therapy with cyclophosphane. After 2 weeks from the start of therapy, the child's well-being improved, body temperature returned to normal, cough decreased, but then the condition worsened again due to the appearance of respiratory failure of 2 art., an increase in cough, hemoptysis, an increase in body temperature to 39.0. There was a need for oxygen subsidies through nasal cannulas (SO2 = 92-94%).
According to chest X-ray data, negative dynamics was noted due to the progression of lung tissue damage specific to the underlying disease, which threatened the development of massive pulmonary bleeding due to possible erosion of the pulmonary vessels. The patient was transferred to a surgical hospital, where she underwent bronchoscopy and pulmonary bleeding was confirmed. After stabilization of her condition, she continued treatment in a somatic hospital. Mycophenolate mofetil 1.5 g per day is prescribed instead of cyclophosphane. The patient underwent a telemedicine consultation with a rheumatologist at the V.A. Nasonova Federal State Medical Research Institute in Moscow, and agrees with the interpretation of the diagnosis and the therapy. However, taking into account the extremely severe course of the disease and the rapidly progressing immunopathological process, it is recommended to strengthen immunosuppressive therapy with the inclusion of a genetically engineered biological product - rituximab - 375 mg/m2 according to the standard scheme. In addition, taking into account the high risk of infectious complications in this variant of the course of the disease, against the background of immunosuppressive therapy, the patient was intravenously injected with immunoglobulin at a low immunosuppressive dose. She tolerated the infusions of immunoglobulin and rituximab well.

Chest X-ray (January 2024): positive dynamics is noted due to the reduction of decay cavities. At the site of the reduced decay cavities, vascular interstitial changes are noted - developing fibrosis. No new foci have been identified (Figure 2).
Against the background of the ongoing treatment, the child's condition improved, the temperature returned to normal, the effects of intoxication disappeared, the cough decreased, the voice increased, and laboratory indicators of disease activity decreased.
The child needs long-term immunosuppressive therapy and prevention of possible complications.
Conclusion: systemic ANCA-associated vasculitis is a rare disease among children. The analysis of the clinical picture in combination with data from instrumental and immunological studies is the basis for timely diagnosis. The course of the disease was complicated by pulmonary bleeding, which required additional endoscopic intervention. Standard immunosuppressive therapy in combination with rituximab significantly improved the patient's condition.

About the authors

Vasilisa Alekseevna Gritsunova

Voronezh State Medical University named after N.N. Burdenko

Email: lissa.vasilisa37@gmail.com
ORCID iD: 0009-0001-3192-0729

6th year student of the Pediatric Faculty

Russian Federation, 10 Studentskaya str., Voronezh, 394036, Russia

Anastasia Sergeevna Kovaleva

Voronezh State Medical University named after N.N. Burdenko

Email: nastkrot2000@gmail.com
ORCID iD: 0009-0005-4508-9090

6th year student of the Pediatric Faculty

Russian Federation, 10 Studentskaya str., Voronezh, 394036, Russia

Marat Magdamovich Zakirov

Voronezh Regional Children's Clinical Hospital No. 1

Email: maratzak3@mail.ru
ORCID iD: 0000-0003-2769-6928

Head of the department, pediatric cardiologist, rheumatologist

Russian Federation, 1 Burdenko str., Voronezh, 394024, Russia

Egor Lvovich Kondrykinsky

Voronezh Regional Children's Clinical Hospital No. 1

Email: egor_lvk@mail.ru
ORCID iD: 0000-0001-8886-2091

PhD, Pediatric cardiologist, rheumatologist

Russian Federation, 1 Burdenko str., Voronezh, 394024, Russia

Elena Vladimirovna Homarova

Voronezh Regional Children's Clinical Hospital No. 1

Email: khomarovael@mail.ru
ORCID iD: 0000-0002-8339-4417

pediatric cardiologist, rheumatologist

1 Burdenko str., Voronezh, 394024, Russia

Inna Vladislavovna Kondratieva

Voronezh State Medical University named after N.N. Burdenko

Author for correspondence.
Email: Innakondrateva6121@yandex.ru
ORCID iD: 0000-0002-7564-0382

Candidate of Medical Sciences, Associate Professor of the Department of Hospital Pediatrics

Russian Federation, 10 Studentskaya str., Voronezh, 394036, Russia

References

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