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6Wschodnioeuropejskie Czasopismo Naukowe (East European Scientific Journal) #14, 2016 ШШ
УДК 616.211-002.7-07
Kudaibergenova S.F., Dzharkinbekova G.K., Mukanova Zh.T., Zhaysakova D. Y., Mirzabayev D.E., Hurshidov F.N.
Asfendiyarov Kazakh National Medical University
THE DIFFICULTIES OF DIAGNOSIS OF WEGENER'S GRANULOMATOSIS (A CASE FROM PRACTICE OF OTORHINOLARYNGOLOGIST)
Key words: Wegener's granulomatosis, diseases of the nose and paranasal sinuses, computed tomography, oligosymptomatic course of the disease.
Resume
There is a such case from practice of otorhinolaryngologist as an observation of the patient with a diagnosis of "Wegener's granulomatosis" in this article. This pathology is quite rare, and these patients are usually observed at the rheumatology. But often, there may be a situation where patients are treated primarily to other specialists, as it happened in the case we described.^iagnostic criteria of Wegener's granulomatosis with the defeat of the respiratory tract are necrotizing rhinitis, sinusitis, the destruction of cartilage and bones of the nose and paranasal sinuses, AT to the cytoplasm of neutrophils in the blood serum, the detection in biopsy material of the giant cell granulomatous necrotizing vasculitis, accompanied by granulomatous inflammation. But these clinical manifestations are seen at the late stage of the disease and at early stages and in oligosymptomatic course of the disease patients patients may go without diagnosis and adequate treatment.=That is why despite the fact that there is a lot of literature on this issue, we felt that our observation could be interesting for practitioners.
Introduction.
Wegener's granulomatosis is a rare disease and these patients are usually observed in Rheumatology. But in recent years, domestic and foreign literature appears increasingly publications when such situations arise, and patients were treated primarily to other specialists. One of the important pathological and clinical features of Wegener's disease is a primary lesion of the nose and paranasal sinuses. That is why in terms of diagnosis, treatment and clinical follow-up of these patients a significant role of otolaryngologist.
There are many definitions of this disease given by different authors. Here are some of them. Wegen-er's granulomatosis (F. Wegener, German pathologist, born in 1907;) is a systematic disease often fatal which affects the upper respiratory tract, including the nasal mucosa, and kidney [1, 2]. Synonims of the Wegener's granulomatosis are Wegener's disease, ne-crotizing granuloma of upper respiratory tract with the nephrithis. Wegener's granulomatosis (WG) is a disease from the group of systematic vasulitis which is characterized by granulomatous lesions, primarily the upper respiratory tract, lungs and kidneys [3]. Skin changes may occur in a variety of (haemorrhagic, viral, nodules), rash, arthralgia mainly large joints [4]. There can be eye damage due to the spread of granu-lomatous process in the eye orbit to the development of exophthalmos. Some authors identify granulomato-sis of the nose such a form of the WG but most see it as an independent disease [5, 6]. Granulomatosis of the nose (of the face) is a malignant (middle,fatal)form of the disease and characterized by defeat of the nose, soft tissues of the face region, oral cavity and palate with the development of extensive necrotizing defects [7,8]. The internal organs are usually not affected. Here is an example of the disease from our own practice
Patient X., 28 years old was observed in the center of rheumatology in Almaty (Kazakhstan) in Octo-
ber 2014 with a diagnosis of "Wegener's granulomato-sis". From the anamnesis we know that in the last few years the patient repeatedly suffered from acute respiratory viral infection, and was treated at home by himself. In February, with the strong toothache he addressed to a dentist. He was treated with a superficial form of the caries of upper fourth tooth on the right. But after treatment, pain in the tooth persisted. With suppositious odontogenic sinusitis, a man was sent to the otolaryngologist domiciliary. There were X-ray scan of the nose and paranasal sinuses, which shown a picture of the wall sinusitis, and diagnostic puncture of the right maxillary sinus. Pathological contents were not indentified. The washings were clean without pus. The pain in the projection of the right maxillary sinus kept, but less intense.
Patient periodically took analgesics. In April, he addressed to the otolaryngologist with the same kind of pain. There was made computed tomography (CT) scan of the nasal cavity and paranasal sinuses and identified parietal thickening of the mucosa of the maxillary sinus on the right (as in the case in February).
(From outpatient card) during rhinoscopy nasal breathing was satisfactory. There were determined hyperemia of the mucous, moderately severe dry mucous membranes of the nasal cavity, single crust at the right side. There were no changes in blood and urine tests and all the indicators were within normal limits. From the anamnesis it is known that the disease had frequent relapses, and toothache diagnosed as a neuralgic pain. Patient refused to repeate the puncture because for the first time at the same X-ray picture the washings from the right maxillar sinus were clean. There was conservative therapy by antibiotics based on sensitivity (cefazolinum), decongestants, treatment by movement. In a smear from the nasal mucosa sown skin staphylococcus that is resistant only to penicillin and macrolides.
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In July, after secondary treatment of the patient at the otolaryngology, he was sent to the Republican Hospital for a consultation with Professor otolaryngologist, where was a consilium with the maxillofacial surgeon.
There was made spiral computed tomography (SCT) of the nasal cavity and paranasal sinuses at the
Picture 3 -Spiral CT of the skull skull in the axial projection (slight thickening of the parietal mucous of maxillar sinus on the right)
It was recommended to take biopsy material from the mucous of the nasal cavity. There were found isolated multinucleated giant cells and inflammation in the biopsy material. No one payed attention. Because a similar pattern is found in other processes occurring in the nose, especially at an infectious inflammation, at the connective tissue damage, at Goodpasture's syndrome, at hypersensitivity vasculitis, at tuberculosis process [1]. But then all the clinical manifestations on the part of the tooth and sinus subsided. Then appeared low-grade fever, pain, swelling in the knee joints and the patient is taken to the treatment by the therapist.
The fact of that the patient works as a butcher made therapist suspect brucellosis inflammation of the
Republican Hospital. On the SCT revealed a slight parietal thickening of the mucosa of the maxillary sinus on the right (as in the case in February), and secretive-ness of the front latticed cells on the right (Figure 1, 2, 3, 4 - SCT maxillary, Stack, frontal, sphenoid sinuses in the coronary and axial projections).
Picture 4 - Spiral CT of the skull in the axial projection (normal view of the frontal sinuses)
joints. Blood analyses showed moderate leukocytosis, accelerated erythrocyte sedimentation rate (36 mm / h), and increased C-reactive protein (CRP). Blood test for brucellosis is negative, the internal organs without any pathology. Since the treatment is not carried out by the therapist the patient is directed to consult a rheumatologist in the city rheumatologic center (SRC) of Almaty with a diagnosis of rheumatoid arthritis (not excluded brucellosis etiology).
There are also more complicated, but more effective analysis to detect anti-neutrophil cytoplasmic antibody (ANCA) [9, 10, 11]. These tests are not done for every patient, but only in the case of suspicion of a systemic disease of the connective tissue and only in specialized clinics. In a study in the SRC blood test
Picture 1 - Picture 2 - Spiral CT of the skull in the axial projec-
Spiral CT of the skull in the coronal projection (a tion (secretiveness of the front latticed cells on the
slight parietal thickening of the mucous membrane right) in the right maxillary sinus)
for the presence of ANCA was positive and also C-reactive protein was positive.
The patient was hospitalized to the city reumato-logic department of Almaty, diagnosed with "Wegener's granulomatosis". And then, may be, against acute parietal sinusitis formed perforation of the nasal septum. The patient was immediately appointed as pred-nisolone 1 mg per 1 kg of body weight. Positive effects include rapid calming of inflammation from the joints. But then appeared ulceration on the skin of nose. At the end of October 2014, during the treatment of the patient by prednisolone were increased hemor-rhagic purulent discharge from the nose. Body temperature was low-grade, the sum of leukocytosis up to15,0 109/l.
Repeatedly evaluated radiographic changes in the trachea, bronchi and lungs. There were no changes in the cartilage and bone. Urinalysis showed protein in the urine, and no red blood cells. Underwent antibiotic therapy, the dose of prednisone increased to 60 mg per
day. But the treatment made necrotic changes in the nose and sinuses increase. Radiographic studies of bones of the nose and the PNS have not been conducted.
Antibacterial therapy initiated, continued predni-solone at a dose of 10 mg per day, assigned cytotoxic drug methotrexate 7.5 mg per week under the control of the blood analysis leukocytes and platelets. About a week later there was a productive cough with mucous expectoration of sputum, fever remained febrile. During the therapy in the dynamic X-ray examination of the chest, there was a softening of the cartilage of the upper trachea.
At the examination of upper respiratory tract: in the projection of the nasal bridge there was a gaping hole. Almost all of the contents of the nasal cavity was absent, no nasal septum cartilage and bone, and no inferior turbinates. Also there was a destruction of the middle turbinate to the right (see. Picture 5).
Picture 5
This is what was left of the nose. There were ne-crotic tissue with putrid odor, dirty crusts that were difficult to be removed, beneath erosion, ulcers, on the remains of the middle turbinate granulomatous tissue with uneven rough surface. Reactive swelling of orbital area at the left and right. Sight is saved. It was need to conduct hygiene procedures in the nasal cavity. Necrotic tissue was removed and at the same time tissue pieces from different foci were taken for a biopsy. Also was made a correction of antibiotic therapy based on sensitivity (appointed as ciprofloxacin).
Admission of cytostatic drug methotrexate 10 mg per week continued. After daily hygiene procedures made in the nasal cavity by antiseptic solutions and all the therapy, necrotic process in the nose suspended. Jt was recommended observation by otholaringologist and rheumatologist at the place of residence, and after stabilization of a specific process in remission - to hold the plastic outer nose [12].
After treatment the patient was discharged home for a break in the treatment, and then repeat the recommended course of antibacterial therapy, taking into the antibiotics according to sensitivity and treatment with high doses of hormones and cytotoxic drugs. But despite treatment about a month later the patient died
from developed acute renal failure. Glomerulonephritis joined to the process ,and proceeded without arterial hypertension, with the rapid development of renal failure.
Discussion.
May be, the process began in the nasal cavity and the progression of the disease provoked by such seemingly innocuous manipulation as treatment of dental caries, endonasal sinus opening on the right, and taking a biopsy of the tissue of the nose.
Where and at what stage we could think about the consistency of the symptoms and about Wegener's granulomatosis?
Perhaps, at the stage of the consultation when the giant cell clusters were found, although they are found in many specific diseases, especially when tuberculosis.
Wegener's granuloma taken from the nasal tissue in October were not confirmed. Apparently, the presence of recurrent parietal sinusitis (not pus) should be considered a diagnostic criterion of WG.
From the literature it is known that a definitive diagnosis can be established only by biopsy, which should be taken from the organ affected by granulomatosis, which in our case is not confirmed primary
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diagnosis [13, 14]. Perhaps the reason is that in We-gener's granulomatosis nonspecific bacterial pockets develop often and we took pieces of tissue from the normal range of inflammatory and granulomatous region remained intact. According to the literature, 50% of patients have rheumatoid factor [15, 16]. To date, the literature describes a number of observations of the disease; in most cases it was diagnosed after death
[17]. According to many authors, patients with Wegener's granulomatosis do not live more than 5 years
[18].
Conclusions:
1. Despite the absence of pathological changes in the blood count and total urine analysis, during persistently proceeding nonsuppurative Parietal-hyperplastic sinusitis with the aim to early diagnosis of Wegener's granulomatosis is necessary to make an analysis to identify the anti-neutrophil cytoplasmic antibody (ANCA).
2. In the primary lesion of the nose and paranasal sinuses in Wegener's granulomatosis is expedient to carry out the repeated study of the urinary system, because in 50% of cases, death occurs as a result of glo-merulonephritis, with the rapid development of renal failure.
3. Considering that the first manifestations of the disease arise from the upper respiratory tract, the continued uncertain etiology of pain combined with hy-perplasia of the mucosa of the paranasal sinuses may be the only manifestation of Wegener's granulomato-sis in the early stages of the disease, and in spite of the rarity of Wegener's granulomatosis, we must pay special attention to this state and should alert the otolar-yngologist.
Literature:
1. Rheumathology : clinical recommendations / ed. Acad. EL RAMS Nasonova. - 2nd ed., Rev. and add. - M .: GEOTAR Media, 2010. - 752 p.
2. Popova N.A. Difficulties in Whegener's granulomotosis' treatment (clinical observation)
www.medconfer.com ; © Bulletin of Medical Internet Conferences, 2012 (Journal Bulletin of Medical Internet conferences .- № 2 / Volume 2/2012 S.62-63.)
3. S. J. Spalding, M. Cambria, Th. Arkachaisri. Distinguishing Wegener's Granulomatosis From Necrotizing Community Acquired Pneumonia: A Case Report and Comparison of Radiographic Findings. Pediatr. Pulmonol. 2009; 44(2): 195-197.
4. S. Madhira, Q. A. Hamid, S. Moorthy, N. Prayaga, Srikanth. Kolloju Limited Wegener's Granulomatosis with Predominant Otological Presentation. Indian J. Otolaryngol. Head Neck Surg. 2011 July; 63(Suppl 1): 4-5.
5. G.I.Markov. Diagnosis and treatment of We-gener's granulomatosis // Herald otorinolaringology.-№1.- 2010.-S. 19-20.
6. B. Makay, T.Tuncel, N. Uzuner, E. Unsal. Juvenile idiopathic arthritis and Wegener granulo-matosis: causal or casual relationship? Pediatr. Rheumatol. Online J. 2011; 9(Suppl 1): P175.
7. S. Swain, R. Ray. Wegener's Granulomatosis of Nose: A Case Report. Indian J. Otolaryngol. Head Neck Surg. 2011 October; 63(4): 402-404.
8. Yakimenko EA Zakatova LV Tikhonchuk NS Vasilets VV Antipov NN Tbileli VV Differential diagnosis of T-lymphoma nasal type, takes place under the guise of Wegener's granulomatosis (clinical observation) // Journal Bulletin problems of biological medicine. -№1.-2010.
9. Paul F. Milner. Nasal Granuloma and Periarteritis Nodosa. Br. Med. J. 1955 December 31; 2(4956): 1594-3, 1597-1599.
10. A. E. Read, C. S. Treip .Wegener's Granu-lomatosis. Granuloma of the nose with systemic poly-arteritis nodosa. Postgrad. Med. J. 1957 April; 33(378): 199-204.
11. José A. Gómez-Puerta, Xavier Bosch. Anti-Neutrophil Cytoplasmic Antibody Pathogenesis in Small-Vessel Vasculitis : An Update. Am J. Pathol. 2009 November; 175(5): 1790-1798.
12. S.Hamour, A. D. Salama, Ch. D. Pusey. Management of ANCA-associated vasculitis: Current trends and future prospects. Ther. Clin. Risk Manag. 2010; 6: 253-264.
13. Ch. Lacoste, N. Mansencal, M. Ben m'rad,
C. Goulon-Goeau, P.Cohen, L. Guillevin, T. Hanslik. Valvular involvement in ANCA-associated systemic vasculitis: a case report and literature review. BMC Musculoskelet Disord. 2011; 12: 50.
14. F. Sachse, W. Stoll. Nasal surgery in patients with systemic disorders. Curr Top Otorhinolaryngol. Head Neck Surg. 2010; 9:
15. Klimenko SV Wegener's granulomatosis: clinical features of the modern trend, prognostic factors, and outcomes. Author. Dis. ... Cand. med. M., 2006.- 119 p.
16. Sinenko A.A., Y. Kulakov E.B., Abramo-chkin N.M., Lupach, O.I. Syusina, E.M. Fayzenger, E.J. Yevdokimov, L.M. Moldovanova. The experience of the patient with Wegener's granulomatosis: the difficulty of diagnosis and treatment // Herald of modern clinical medicine in 2011, №02, Volume 4.
17. Yakimenko E.A., Zakatova L.V., Tikhonchuk N.S., Vasilets V.V., Antipov N.N., Tbileli V.V. Differential diagnosis of T-lymphoma nasal type, takes place under the guise of Wegener's granulomatosis (clinical observation) // Journal Bulletin problems of biological medicine. -№1.-2010.
18. Sigitova O.N. , Bikmuhammetova E.I., Bog-danov A.R. Wegener's granulomatosis (clinical case study) // Bulletin of modern clinical meditsiny.- Issue number 3/2010-that 3.- S.75-76.
Summary. Difficulties in the diagnosis of Wegener's granulomatosis.
(Case study of ENT doctor)
Kudaibergenova S.F., Dzharkinbekova G.K., Mukanova Zh.T., Zhaissakova D. Ye., Mirzabaeyev
D. E., Khurshidov F.N.
у
Key words: Wegener's granulomatosis, a disease of the nose and paranasal sinuses, computed tomography, asymptomatic course.
This article describes a clinical case study of the doctor-ENT: observation of a patient with a diagnosis of "Wegener's granulomatosis". The Wegener's granulomatosis is an uncommon disease and usually the patients with this type of diagnosis observed by specialists of rheumatology.But, often, there may be situations when patients are treated primarily by other specialists as it happened in the our case. The symptoms of Wegener's granulomatosis with desordes of the upper respiratory tract are the presence of necrotizing rhinitis, sinusitis, destruction of cartilage and nasal bones and paranasal sinuses, antibodies to the cytoplasm of neutrophils in the blood serum, the detection in wa biopsy specimen of giant cell granulomatous necrotizing vasculitis accompanied by granulomatous inflammation. But these clinical
manifestations arise at the late stage of the disease and so at the early, oligosymptomatic stages the diagnosis may be not be evident in some patients. We believe our observation will be interesting for doctors.
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Information about authors :
Saule Fazylovna Kudaibergenova
PhD., MD., Professor,
Head of the department of Otorhinolaryngology Asfendiyarov Kazakh National Medical University.
The highest qualificational category of othol-aringologist
mob: +77054614095; office: +7(727)2646844; +7(727)2647292.
E-mail: medlors@mail. ru
Саипова Дурдона Салиджановна
магистр 3-года,
Ташкентский педиатрический медицинский институт Рузметова Ирода Арслановна к.м.н., доцент
Ташкентский педиатрический медицинский институт Эгамбердиева Дано Абдисаматовна
к.м.н., доцент
Ташкентский педиатрический медицинский институт
ОЦЕНКА ГЕМОДИНАМИЧЕСКИХ ПАРАМЕТРОВ У БОЛЬНЫХ ХРОНИЧЕСКОЙ БОЛЕЗНЬЮ ПОЧЕК
ASSESSMENT OF HEMODYNAMIC PARAMETERS IN PATIENTS WITH
CHRONIC KIDNEY DISEASE
Аннотация: Данная статья освящает проблемы кардиоренального синдрома у больных хронической болезнью почек (ХБП) почечной этиологии. В статье отражено функциональное состояние миокарда в зависимости от стадии ХБП. У пациентов с ХБП индекс массы миокарда левого желудочка (ИММЛЖ) был выше нормы у 48,2% больных ХБП. При ХБП II-IV ст. ИММЛЖ превышал нормальные значения 140±5 г/м, 142±6 г/м, 146±7 г/м, соответственно (р<0,001) и был наиболее высоким при ХБП IV ст. по сравнению с ХБП I ст., соответственно 146±7 г/м и 124±6 г/м (р<0,001). При ХБП I ст. диастолическая дисфункция встречалась у 23%, при ХБП II ст. у 30%, при ХБП III ст. у 51% пациентов и при ХБП IV ст. - у 62% пациентов. Следует отметить, что при ХБП I ст. диастолическая дисфункция встречалась почти у четверти больных, и ее частота увеличивалась по мере прогрессирования ХБП, достигая максимальных значений при ХБП IV ст. Так, впервые показано, что у больных ХБП на ранних стадиях, в том числе и у нормотензивных больных выявляется диастолическая дисфункция левого желудочка.
Ключевые слова: хроническая болезнь почек, кардиоренальный синдром, диастолическая дисфункция левого желудочка, гипертрофия левого желудочка, индекс массы миокарда.
Актуальность проблемы. Хроническая болезнь почек (ХБП) занимает одно из ведущих мест в общей структуре заболеваемости и смертности населения. Кардиоренальные взаимоотношения определяются общностью механизмов формирования и прогрессирования поражения сердечно-сосудистой системы и почек при таких распространенных в общей популяции заболеваниях, как артериальная гипертензия, сахарный диабет, распространенный атеросклероз [6,9,12]. В настоящее время снижение функции почек расценивается как
фактор ускоренного развития патологии сердечнососудистой системы [3,10]. Наличие умеренной почечной дисфункции ассоциировано с увеличением распространенности ИБС и цереброваску-лярной патологии [6,8]. Взаимозависимость патологических процессов сердечно-сосудистой системы и почек определяет кардиоренальные взаимоотношения, как непрерывную цепь событий, составляющих порочный круг - кардиоре-нальный континуум [3,7]. Однако, ряд аспектов этой проблемы остаются малоизученными.
