Volume 04 Issue 02-2024
15
International Journal of Medical Sciences And Clinical Research
(ISSN
–
2771-2265)
VOLUME
04
ISSUE
02
P
AGES
:
15-19
SJIF
I
MPACT
FACTOR
(2021:
5.
694
)
(2022:
5.
893
)
(2023:
6.
184
)
OCLC
–
1121105677
Publisher:
Oscar Publishing Services
Servi
ABSTRACT
Wegener's granulomatosis (granulomatosis with polyangiitis) is a rare autoimmune disease characterized by vasculitis
of small and medium-sized vessels and the formation of granulomas. Most often, Wegener's granulomatosis (WG)
affects the upper respiratory tract, lungs, kidneys, and eyes. Without treatment, the disease progresses rapidly and
leads to death. GV was first described at the end of the 19th century. The characteristic clinical picture of hepatitis B
was described by the German pathologist Friedrich Wegener. After the reports of F. Wegener in 1936 and 1939. In
cases of necrotizing vasculitis with granulomatous inflammation as a special nosological form of vasculitis, this disease
became known as Wegener's granulomatosis. In 2006, F. Wegener’s conne
ction with Nazism was discovered, and it
was proposed to abandon the term “Wegener’s granulomatosis” in favor of the term “granulomatosis with
polyangiitis,” which, in general, more reflects the modern view of the essence of this disease. The term "Wegener
's
granulomatosis", however, is today used along with the term "granulomatosis with polyangiitis".
KEYWORDS
Wegener's granulomatosis, vasculitis, polyangiitis.
Research Article
GRANULOMATOSIS WITH POLYANGIITIS: LITERATURE REVIEW
Submission Date:
February 01, 2024,
Accepted Date:
February 06, 2024,
Published Date:
February 11, 2024
Crossref doi:
https://doi.org/10.37547/ijmscr/Volume04Issue02-03
Boymuradov Sh.A.
Tashkent Medical Academy, Uzbekistan
Khaitmurodov D.E.
Tashkent Medical Academy, Uzbekistan
Kurbonov Y.Kh.
Tashkent Medical Academy, Uzbekistan
Journal
Website:
https://theusajournals.
com/index.php/ijmscr
Copyright:
Original
content from this work
may be used under the
terms of the creative
commons
attributes
4.0 licence.
Volume 04 Issue 02-2024
16
International Journal of Medical Sciences And Clinical Research
(ISSN
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2771-2265)
VOLUME
04
ISSUE
02
P
AGES
:
15-19
SJIF
I
MPACT
FACTOR
(2021:
5.
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)
(2022:
5.
893
)
(2023:
6.
184
)
OCLC
–
1121105677
Publisher:
Oscar Publishing Services
Servi
INTRODUCTION
Granulomatosis with polyangiitis (GPA), formerly
known as Wegener's granulomatosis (WG), is a rare
disease from the group of systemic ANCA-associated
vasculitides (systemic vasculitis associated with the
presence of antineutrophil cytoplasmic antibodies),
characterized by granulomatous inflammation and
necrotizing vasculitis of small and medium-sized
vessels (capillaries, venules, arterioles, arteries),
affecting mainly the upper and lower respiratory tract,
as well as the kidneys [1-4]. The prevalence of hepatitis
B in the population is 25-60 cases per 1 million
population, the incidence is 3-12 cases per 1 million.
However, the incidence of hepatitis B in European
countries, according to some data, has increased 4
times over the past 30 years. An increase in new cases
of the disease has been noted in the autumn-winter
period [3].
The German pathologist F. Wegener described this
disease as a separate nosological unit, noting the
characteristic clinical and morphological features, and
for the first time identified this disease as a special
form of vasculitis [5-8]. In 1954, G. Gudman and J. Churg
formulated clinical and morphological criteria for
hepatitis B, including a triad of pathological signs:
systemic
necrotizing
vasculitis,
necrotizing
granulomatous inflammation of the respiratory tract
and
necrotizing
glomerulonephritis.
The
first
description of GW in Russian literature belongs to A.I.
Strukov and V.V. Badmaeva [ 9].
The reasons for the development of this malignant
necrotizing vasculitis are still unknown. The
morphological substrate of the disease is granulomas,
accompanied by necrosis [10-12]. Therefore, the gold
standard for confirming the diagnosis today remains
histological examination of the affected tissue (nasal
mucosa, lung tissue, skin or kidney, granulation tissue
of the orbit of the eye), obtained during surgery or by
biopsy. Two types of changes are detected in the
tissues studied: necrotizing granuloma and necrotizing
vasculitis. The disease is characterized by a widespread
necrotizing process in blood vessels of all levels with
predominant damage to muscular arteries and the
microvasculature. Panarteritis with the spread of
inflammatory phenomena to all three membranes of
the vessel is typical for hepatitis B. As a result of
destructive-productive vasculitis, giant cell granulomas
are formed, followed by their destruction or necrosis
[1, 3-15].
GV is divided into local, limited and generalized forms.
The local form is an isolated lesion of the upper
respiratory tract, organ of hearing and organ of vision.
The limited form implies damage to the lungs with the
presence or absence of local lesions of the upper
respiratory tract, hearing and vision. The generalized
Volume 04 Issue 02-2024
17
International Journal of Medical Sciences And Clinical Research
(ISSN
–
2771-2265)
VOLUME
04
ISSUE
02
P
AGES
:
15-19
SJIF
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MPACT
FACTOR
(2021:
5.
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(2022:
5.
893
)
(2023:
6.
184
)
OCLC
–
1121105677
Publisher:
Oscar Publishing Services
Servi
form is characterized by the development of
glomerulonephritis, regardless of the presence or
absence of damage to other organs. During the GW
there are three periods. The onset of the disease is
characterized by local changes in the upper respiratory
tract, middle ear or eyes [16]. During the period of
generalization, internal organs, primarily the lungs and
kidneys, are involved in the pathological process. In the
terminal period, the development of renal and/or
pulmonary heart failure progresses. The clinical picture
of hepatitis B is very diverse. Common symptoms at the
onset of the disease are fever, general weakness,
weight loss, myalgia/arthralgia [17]. The onset of the
disease can be subacute (with the development of
clinical symptoms over several weeks) or primary
chronic. One of the leading manifestations is damage
to the ENT organs, which occurs in the advanced stage
of the disease in 90-94% of patients.
Early diagnosis of hepatitis B is a difficult clinical task
and requires a thorough examination of the patient
using modern research methods to identify
pathognomonic symptoms. A targeted search for
damage to the respiratory tract is necessary, including
rhinoscopy, laryngoscopy, computed tomography of
the paranasal sinuses and lungs, since for a long time
the disease can be asymptomatic or accompanied by
scanty clinical symptoms. Only in 50% of patients the
diagnosis is verified in the first 3-6 months from the
onset of the disease, and in 7% of patients, HB remains
undifferentiated for 5-16 years from the onset of the
first symptoms [18].
In 1990, the American College of Rheumatology
developed classification criteria for GPA [3]:
1. Inflammation of the nose and mouth, ulcers in the
mouth, purulent or bloody discharge from the nose.
2. Changes in the lungs during X-ray examination
(nodules, infiltrates or cavities).
3. Microhematuria (>5 red blood cells per field of view)
or accumulations of red blood cells in the urine
sediment.
4. Biopsy: granulomatous inflammation in the arterial
wall or in the perivascular and extravascular space.
The presence of two or more criteria allows a diagnosis
to be made with a sensitivity of 88% and a specificity of
92%. In the absence of biopsy results, it is proposed to
include an additional symptom
–
hemoptysis
–
into the
diagnostic criteria.
CONCLUSION
Early diagnosis of hepatitis B is a difficult clinical task
and requires a thorough examination of the patient
using modern research methods to identify
pathognomonic symptoms. A targeted search for
damage to the respiratory tract is necessary, including
rhinoscopy, laryngoscopy, computed tomography of
the paranasal sinuses and lungs, since for a long time
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Publisher:
Oscar Publishing Services
Servi
the disease can be asymptomatic or accompanied by
scant clinical symptoms.
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