features of the course of wegener's...

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Generalization of Scientific ResultsUSA, Michigan

www.journalofresearch.us¹ 9-10, September-October 2018 [email protected] Journal of Research

http://dx.doi.org/10.26739/2573-5616-2018-10-2-9

Abstract: Wegener's granulomatosis (WG) - a rare systemic disease of unknown etiology.Histopathological characterized by granulomatous necrotizing systemic vasculitis, mostoften involving the upper and lower airways with further lung damage. WG debut inmost cases differs no specificity of clinical picture and the complexity of timely diagnosis,which leads to a delay of treatment and poor prognosis. Presented 1 case of WG causedconsiderable difficulties in diagnosis at the early stage of the disease.

Keywords: systemicvasculitis, Wegener's granulematosis

FEATURES OF THE COURSE OF WEGENER'SGRANULOMATOSIS IN COMBINATION WITH

COMORBID PATHOLOGY

Rizamukhamedova Mashhura Zokirovna,Djuraeva Elnora Rustamovna, Berdieva Dilfuza Umurzakovna,

Tashpulatova Maktuba Mukhamedalievna,Tashkent Medical Academy

HEALTH SCIENCE

Manuscript info:Received September 17, 2018., Accepted October 16, 2018., Published October 30, 2018.

Recommended citation: Rizamukhamedova Mashhura Zokirovna, Djuraeva Elnora Rustamovna,Berdieva Dilfuza Umurzakovna, Tashpulatova Maktuba Mukhamedalievna. FEATURES OF THECOURSE OF WEGENER'S GRANULOMATOSIS IN COMBINATION WITH COMORBIDPATHOLOGY. 9-10 American Journal of Research P. 86-90 (2018).

Wegener's granulomatosis (WG)is a systemic vasculitis of the arteriesand veins of the middle caliber,characterized by the development ofnecrotizing granulomatousinflammation with a predominantlesion of the upper respiratory tract,lungs and kidneys [1]. WG was firstdescribed in 1931 by H. Klinger(1936) and F. Wegener (1939)[5].Both men and women are sick oftenin the age of about 40 years. [4, 9].

The etiology of the disease isunknown. It is assumed that the onsetof the disease is related to the effect of

infectious agent. Some researchers notea link between WG with a previouspurulent infection or tuberculosisof the respiratory tract. A moresevere course of WG in patients withS. staphylococcus aureus(Staphylococcus aureus) carriage inthe nasal cavity is shown, which ischaracterized by more frequentexacerbations associated with exposureto exotoxins of staphylococcuspossessing the properties of superantigens. The potential etiological roleof colloidal silicon compounds is alsodiscussed. [5].

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Pathogenesis of WG is associatedwith the development of widespreadinflammation of small vessels andthe simultaneous formation ofperivascular and extravasalgranulomas of macrophage type withLanghans cells in the affected organsand tissues. The serologic marker ofthe disease is antibodies to thecytoplasm of neutrophils that bindto antigens expressed by neutrophils(proteinase-3, myeloperoxidase,etc.), and can cause degranulationof these cells with the release ofproteolytic enzymes. Otherpathogenetic mechanisms arediscussed: immune complex lesionof the vascular wall, lymphocyticcytotoxic reactions, etc. [3].

The clinical picture of WG [1] ischaracterized by multi ple organdysfunction. With WG, more than90% of the cases show signs of upperrespiratory tract, lung and middleear injuries. Sinusitis is observed in50% of patients, in 36% - rhinitis,in 10% pleurisy, in 25% media otitis.Upper respiratory tract and ear areaffected in 92%, lungs - in 85% ofpatients. [7].

1. Glomerulonephritis develops in75-80% of patients, but it is rarelythe first manifestation of WG.

2. The defeat of themusculoskeletal system is detectedin about 70% of patients.

3. In 52% of cases with WG, eyedamage is observed - from mildconjunctivitis to dacryocystitis,iridocyclitis, granulomas of retrobulbarfiber and exophthalmos. [8].

4. Skin lesion is noted in 46% ofpatients and includes papules,

vesicles, palpable purpura, ulcers,subcutaneous nodules. Vasculitis,granulomas or both are revealed inbiopsy.

5. The defeat of the heart isobserved in 8% of cases and leadsto pericarditis, coronary vasculitis,myocardial infarction, damage ofmitral and aortic valves, andatrioventricular blockade.

6. The defeat of the nervous systemis noted in 23% of patients andincludes polyneuropathy, cerebralvasculitis and cerebral granulomas.[1,6].

"Gold standard" - histologicalexamination of affected tissue (nasalmucosa, lung tissue, skin or kidney,granulation tissue of the orbit of theeye) which obtained by biopsy, withthe detection of fibrinoid necrosisand inflammation of the vessel wallin combination with perivascularand extravasal granulomas [2,11].Characteristic changes in laboratoryindicators: normochromic anemia,thrombocytosis, neutrophilicleukocytosis, acceleration of ESR,increase of the level of CRP. ANCAis identified in 50-70% of patients,which considered as a factor ofunfavorable prognosis of the disease.RF is detected approximately inevery second patient, which is anonspecific marker of activity ofWG. The level of Ig in serum, as arule, within the limits of the norm,an increase of the content of theCIC, hypocomplexemia, theappearance of AT to the glomerulusmembrane is not characteristic.

According to the literature data,the mortality rate of WG is still high.

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The most common causes of deathare intercurrent infections,respiratory and renal failure,cardiovascular disorders, malignantneoplasms (bladder cancer). Thiscauses the need for timely diagnosisof the disease with the subsequentearly appointment of aggressivetherapy before the development ofirreversible damage to vital organs.WG must be suspected in allpatients with fever, weight loss andsigns of multi-organ damage (upperrespiratory tract, lungs, urinarysyndrome, vascular purpura,multiple mononeurotic). The five-year survival rate is more than 65%with adequate therapy of WG [7].

We present the clinical case ofWG in combination with 1 type ofdiabetes mellitus, which causeddifficulties in diagnosing the disease.

Patient D., male, 27 years old,has been suffering from illness sinceMarch 2018, he began to noticepain and swelling in the left side ofthe face, mucous discharge from thenose after hypothermia. A few dayslater, there was a sub febrilecondition, the visual acuity on theright eye decreased. Since the patientsuffered 1 type of diabetes duringthe 1st year, he turned toendocrinologist and he washospitalized in the department ofendocrinology with a diagnosis ofdiabetic retinopathy. Monitoring oflaboratory parameters in the bloodtest indicated an increase of ESRup to 72 mm / h, leukocytosis(20*109/L), glucose hemoglobinlevel-9.2 mmol / L, glycemichemoglobin-9.4%, in general urine

analysis proteinuria, cylindruria,when determining acute phasesamples: CRP-24 mg/l. Radiographyof the lungs: the roots of the lungsare expanded and compacted, thepulmonary fields are clean. MRI ofthe brain: pathological changes werenot detected. The patient has receivedhypoglycemic, metabolic drugs andagents that improve peri pheralcirculation. However, the therapy didnot yield any significant results.Ulcerative necrotic eruptions withmerging character in the region ofthe right half of the back of the nose,the right eyelid and on the mucosaof the hard palate appeared duringthe stay in the hospital. There weremucopurulent discharge from thenose with the formation of bloodycrusts, which made it difficult forthe nasal breathing, worried aboutaching pains in the region of bothknee joints that were notaccompanied with swelling.Otolaryngologist andophthalmologist examined thepatient, eventually, consultation ofthe maxillofacial surgeon wasrecommended. The diagnose WGwas put to patient and recommendedtreatment in the rheumatologydepartment. At admission, thecondition of the patient was mediumseverity, the consciousness is clear.Speech hoarseness is noted. Bodytemperature was 37.40C. Saddle-shaped deformation of the nose wasindicated. The skin was pale, in theregion of the right half of the backof the nose, the right eyelid, thereare ulcerative necrotic eruptions withindistinct boundaries, in the oral

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cavity on hard palate-ulceration,shaped like oval with pronouncedred borders and grayish yellowcoating in the center.

min, AP-110/70 mmHg. Theabdomen is painless in palpation,the liver + 1cm from under the rightcostal arch. The spleen is notenlarged. Stool and diuresis are notviolated. A clinical diagnosis:Systemic vasculit is. Wegener'sgranulomatosis, generalized form,acute course, high activity, withdamaging of upper respiratory tract(destruction of the cartilaginous andbony tissue of the nasal septum, hardpalate), eyes, kidneys (secondaryglomerulonephritis with nephroticsyndrome), heart. Concomitantdiagnosis: 1 type of diabetes mellitus,decompensated stage.

In consideration of the highclinical and laboratory activity of thedisease, a combined pulse therapywith 500 mg of solumedrol and1000 mg of cyclophosphamide ¹2was performed. Methylprednisolonewas administered intravenously in adose of 40 mg per day,cyclophosphamide intravenouslydripped in a dose of 200 mg perweek, azathioprine intravenously ina dose of 100 mg per day. Thetolerability of the therapy wassatisfactory. Positive dynamics wasnoted. Completely docked signs ofrhinitis, the boundaries of necroticfoci became clearly delineated. In thefuture, the patient wasrecommended a programmed pulsetherapy.

Thus, this clinical casedemonstrates the multifacetedmanifestations of WG, the tendencyof the disease to develop ageneralized form with the defeat ofvital organs, which significantly

Moderately enlarged submandi-bular lymph nodes were palpable.Edema of the lower leg on both sides.Palpation of knee joints is painful.In the lungs, breathing is weakenedvesicular breathing, wheezing is notheard. BR-20 in min. Heart areawithout features. Heart sounds arerhythmic, muffled. HRR-114 in

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worsens the prognosis, as well as thecomplexity of diagnosing the diseasein the presence of a comorbidpathology, in particular diabetes

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8. Kubal A.A., Perez V.L. Ocular manifestations of ANCA-associated vasculitis //Rheum. Dis. Clin. North. Am. - 2010. - Vol. 36, ¹ 3. - Ð. 573-586.

9. Mahr A.D., Neogi T., Merkel P.A. Epidemiology of Wegener's granulomatosis:Lessons from descriptive studies and analyses of genetic and environmental studies andanalyses of environmental risk determinants //Clin.Exp.Rheumatology. - 2006; - 24 (Suppl.41): P 82-91.

10. Pagnoux C., Wolter N.E. Vasculitis of the upper airways // Swiss. Med. Wkly. - 2012.-Vol. 142. - P. 135-141.

mellitus. Timely active treatment ofWG has a decisive influence on thecourse of the disease and can preventdisability of the patient.

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