Artigo Acesso aberto Revisado por pares

Behcet's Disease in Saudi Arabia

1989; King Faisal Specialist Hospital and Research Centre; Volume: 9; Issue: 4 Linguagem: Inglês

10.5144/0256-4947.1989.353

ISSN

0975-4466

Autores

Abdullah Al-Dalaan, S. Al-Balaa, Kamal M. El-Ramahi, C. N. A. Rajapakse,

Tópico(s)

Ocular Diseases and Behçet’s Syndrome

Resumo

Original ArticlesBehcet's Disease in Saudi Arabia Abdullah Al-Dalaan, MD Suliman Al-Balaa, MD Kamal El-Ramahi, and MD Chula N. A. RajapakseMD Abdullah Al-Dalaan Address reprint requests and correspondence to Dr. Al-Dalaan: Department of Medicine, King Faisal Specialist Hospital and Research Centre, P. O. Box 3354, Riyadh 11211, Saudi Arabia. From the Department of Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh Search for more papers by this author , Suliman Al-Balaa From the Department of Medicine, King Khalid University Hospital, Riyadh Search for more papers by this author , Kamal El-Ramahi From the Department of Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh Search for more papers by this author , and Chula N. A. Rajapakse From the Department of Medicine, King Khalid University Hospital, Riyadh Search for more papers by this author Published Online:1 Jul 1989https://doi.org/10.5144/0256-4947.1989.353SectionsPDF ToolsAdd to favoritesDownload citationTrack citations ShareShare onFacebookTwitterLinked InRedditEmail AboutAbstractForty-six patients with Behcet's disease were studied. Of those, 37 were Saudi and 9 were non-Saudi Arabs. Male-to-female ratio was 4.1:1. One hundred percent had mouth ulcers, 91% genital ulcers, 65% ocular involvement, 61% skin lesions, 59% joint involvement (nonerosive), 35% central nervous system manifestations, 26% gastrointestinal involvement, 26% vascular lesions, 11% psychiatric problems, and 11% pulmonary involvement. Only one patient had renal involvement which was documented by biopsy. In 72% of the patients, HLA-B5(51) was positive.A Al-Dalaan, S Al-Balaa, K El-Ramahi, CNA Rajapakse, Behcet's Disease in Saudi Arabia. 1989; 9(4): 353-359IntroductionBehcet's disease was first described in 1937 by a Turkish dermatologist, Hulusi Behcet,1 as a triad of oral and genital ulcers and uveitis. Later the scope of the disease widened to include other manifestations, such as cutaneous vasculitis, meningoencephalitis, synovitis, and alimentary tract involvement. There are various diagnostic criteria for the disease. O’Duffy postulated the major criteria.2,3 In 1969, Mason and Barnes suggested a series of major and minor criteria.4 The disease is most common in the Mediterranean Basin, Middle East, and Japan, with incidence varying from 1/10,000 population at Hokkadim in Japan to 2.1/100,000 in Kuwait. The disease is much less common in Europe and the United States with incidence of 1/300,000 in Minnesota.Not much attention has been paid to Behcet's disease in Saudi Arabia, and we are describing 46 patients who attended various clinics in King Faisal Specialist Hospital and Research Centre (KFSH&RC) and King Khalid University Hospital (KKUH).PATIENTS AND METHODSForty-six patients with Behcet's disease were seen in KFSH&RC and KKUH in Riyadh during the period from 1979 through 1987. They presented to various clinics depending on the manifestations of the disease.5 Twenty patients were followed prospectively in the clinics and underwent full clinical examinations by us, as well as by an ophthalmologist, a dermatologist, and other physicians. The data for the rest of the patients were taken from hospital charts.The following determinations were made in most of the patients: complete blood cell count, erythrocyte sedimentation rate (ESR), C-reactive protein, immunoglobulin, alpha-naphthoflavone antinuclear factor, rheumatoid factor (RF), serologic test for syphilis, prothrombin time (PT) and partial prothrombin time (PTT), urinalysis, hepatic and renal profiles, complement (C3, C4, CH50), electrocardiogram (ECG), and various radiologic examinations. Thirty patients had HLA typing. Every effort was made to rule out conditions similar to Behcet's disease, especially for those patients who had vascular manifestations of the central nervous system or with gastrointestinal symptoms.RESULTSOf the 46 patients, 37 were male and 9 female, giving a male-to-female ratio of 4.1:1. Their ages ranged from 16 to 51 years, with an average age of 31.5. The duration of illness varied from 4 months to 13 years, with a mean of 6.4 years. All the patients in our study fulfilled the Mason and Barnes criteria,4 and patients with “incomplete” disease were excluded. Table 1 summarizes details of the patients.Table 1. Clinical data on patients in Saudi Arabia with Behcet's disease.Table 1. Clinical data on patients in Saudi Arabia with Behcet's disease.Mouth UlcersMouth ulcers were present in all of our patients (Figure 1). The ulcers varied in size and shape from small ulcers in the buccal mucosa to extensive ulcers from the lips down to the fauces. The lesions were quite painful. No esophageal ulcers were seen. These ulcers were indistinguishable from common recurrent aphthous ulcers that occurred in 20% of the population.6,7 The duration of these lesions varied between 10 and 20 days, and the frequency of attacks varied between three and seven per year. No scarring was seen in our patients following oral ulcers.Figure 1. Patient 29, showing extensive oral ulceration.Download FigureGenital UlcersGenital ulcers were present in 42 (91%) of our patients. They were multiple and painful, with a recurrence rate of one to three per year. Most ulcers left scars after healing. In most patients the genital ulcer was by history only, and scars were documented in the hospital charts. Constitutional symptoms were not present in most of the patients with active orogenital ulcers. Epididymo-orchitis was not documented in any patient.Ocular LesionsOcular symptoms and signs were present in 30 (65%) of the patients. Symptoms varied from itching and redness to severe pain and blindness. Visual problems caused the patients to seek treatment early. Exacerbation of ocular symptoms was not associated with increased disease activity in other organs, such as oral or genital ulceration. Table 2 summarizes the types of ocular involvement.Table 2. Ocular lesions in 30 patients with Behcet's disease.Table 2. Ocular lesions in 30 patients with Behcet's disease.Skin ManifestationsSkin lesions were present in 28 (61%) of our patients. A skin lesion was not the presenting feature in any patient and was always preceded by orogenital ulcer or other manifestations. Unfortunately, a pathergy test was done in only a few patients who returned for follow-up, and it would be difficult to assess its significance as a diagnostic parameter. Some of the patients had more than one lesion.8Table 3 reflects the scope of skin lesions.Table 3. Skin manifestations in 28 patients with Behcet's disease.Table 3. Skin manifestations in 28 patients with Behcet's disease.Joint InvolvementTwenty-seven patients (59%) had joint involvement varying from single arthralgia to florid arthritis resembling rheumatoid arthritis.4,9 Arthralgia without arthritis was present in 16 patients and was generalized in only four patients. Joint involvement was associated with other manifestations of the disease, especially orogenital ulcers, skin lesions, and systemic symptoms. Arthritis was present in 12 patients, and the most commonly involved joints were knees, ankles, and metacarpophalangeal joints. There was knee joint effusion in five patients, with exudate aspirated. Morning stiffness was present, but unlike rheumatoid arthritis, the symptom did not improve as the day progressed. The ESR was elevated in all patients with active disease, and RF was negative in all the patients. The temporomandibular joint was involved in one patient (no. 19, Table 1). Many patients had multiple joint involvement; however, the sacroiliac joint was not affected in any patient. Table 4 summarizes joint involvement.Table 4. Joint involvement in 27 patients with Behcet's disease.*Table 4. Joint involvement in 27 patients with Behcet's disease.*Neurologic ManifestationsNeurologic symptoms were present in 16 patients (35%) and varied from simple headache to quadriparesis. Two patients presented with headache, blurred vision, nausea and vomiting, as well as papilledema. Both were diagnosed as having intracranial hypertension based on evidence obtained by computed tomographic (CT) scan and cerebrospinal fluid (CSF) opening pressures of 320 mm Hg in patient 12 and 345 mm Hg in patient 22. Neither had factors predisposing to benign intracranial hypertension, and both responded well to dexamethasone treatment.Patient 23 presented with acute ascending paralysis with intercostal involvement and required assisted ventilation. The CSF showed increase in protein. Lymphocytosis responded well to management and was diagnosed as Guil-lain-Barre syndrome. Patients 5 and 13 had paraplegia with sensory level at T-10 and T-2, respectively. In patient 5, the CT myelogram showed a syringomyelia-like cavity at T5-6, compatible with infarction. However, in patient 13 results of all investigations were normal. Three patients had paraplegia, and one had quadriplegia without a sensory level. The CT myelogram, CSF examination, evoked responses, and electroencephalogram were normal. Thus, these manifestations were attributed to brain stem involvement by Behcet's disease.Vascular InvolvementVascular symptoms occurred in 12 patients (26%). Migratory thrombophlebitis was seen in four patients. These patients presented with painful erythematous cordlike lesions which lasted for a few days, leaving behind skin discoloration which later faded. During follow-up, these patients developed deep vein thrombosis and inferior vena caval obstruction. Three patients had inferior vena caval obstruction with prominent collateral vein over the abdomen with the other collaterals patent on venogram. One patient had hepatic vein thrombosis and presented a picture compatible with Budd-Chiari syndrome. Four patients had thrombosis limited to the leg veins.Patient 2 had a mass on chest x-ray film, hemoptysis, cough, and pleuritic chest pain, and was later diagnosed as having pulmonary artery aneurysm (Figure 2).Figure 2. Chest x-ray film for patient 2 demonstrating right hilar mass, proved later to be pulmonary artery aneurysm.Download FigurePatient 24 had acute chest pain of a pleuritic nature and a shadow on chest x-ray film. An aneurysm was suspected, and aortogram showed aneurysm of descending thoracic artery with clot filling (Figure 3). The descending thoracic artery was resected, but 3 years later the aneurysm recurred.Figure 3. Aortogram for patient 24 showing aortic aneurysm.Download FigurePatient 38, who was only 24 years old, presented with acute central chest pain radiating to his left arm and associated with sweating, nausea, and vomiting. Acute myocardial infarction was proved by ECG and cardiac enzyme levels. No underlying predisposing factor was present, and this was attributed to vasculitis of Behcet's disease.Laboratory InvestigationsFive patients (two male and three female) had anemia. One female had iron-deficiency anemia, and the others had anemia of chronic illness. The ESR was elevated during exacerbation of the disease, and the highest value was 130 mm/h. Levels of platelets and white blood cells were normal in all patients. The antinuclear antibodies, rapid plasma reagin, and RF were always negative.Urinalysis was abnormal in only one patient (no. 31) who showed proteinuria and hematuria. Renal biopsy revealed mesangial proliferative glomerulonephritis.The PT and PTT were prolonged in three patients who were on anticoagulant therapy at the time they presented to us. Complement level C3, C4, and CH50 were normal in all 46 patients. Immunoglobulins IgG6, IgM1, and IgM5 were elevated in 12 patients.Thirty-two patients had HLA typing (Table 5). Twenty-three patients (72%) had HLA-B5(51), and out of these 15 (66%) had uveitis and 13 (60%) had arthritis. There is, therefore, a strong correlation between uveitis, arthritis, and HLA-B5(51).Table 5. HLA typing of 32 patients with Behcet's disease.Table 5. HLA typing of 32 patients with Behcet's disease.DISCUSSIONOur study bears an interesting similarity to two other studies from the region.7,10 Like the others, our study confirms that Behcet's disease is not an uncommon disease in this region,9 and the clinical manifestations also appear to be similar.7,9–12However, interesting differences are also present. Neurologic manifestations were greater in severity and frequency in our study than in the others.3,11,13–15 On the other hand, there appear to have been more psychiatric manifestations in Kuwait14 than either Riyadh or Iraq.15 These differences may reflect an interest in the respective specialties in the different institutions. However, since all three studies have come from referral hospitals,13–15 the differences could to some extent be real.Many of the neurologic manifestations in our study, as well as in the other studies, were attributable to pyramidal tract lesions, possibly caused by impairment of arterial supply.16 The two patients (nos. 12 and 22) with raised intracranial pressure and no focal lesions probably had intracranial venous sinus thrombophlebitis. To our knowledge, the syringomyelia-like lesion seen in patient 5 has not been documented before and could also have followed chronic ischemia. The ascending paralysis similar to Guillain-Barre syndrome seen in patient 23 also has not been reported previously with Behcet's disease. The nature of its pathogenesis is not easily determined.Though no patients with inferior vena caval thrombosis were observed in the Kuwait study,14 we encountered four such patients, including three who presented with it and one who developed it subsequently. Arterial involvement was seen in three of our patients in contrast to two from Kuwait14 and none from Iraq.15 The arterial aneurysm seen in two of these, though rare, is well documented.12,17The acute myocardial infarction seen in patient 38, however, does not appear to have been documented before. The etiology of vascular occlusions in Behcet's disease is probably multifactorial.16,18,19 Inflammation of the vessel wall is a major contributor. A hypercoagulable state and decreased fibrinolysis are other possible factors promoting occlusive vascular events in Behcet's disease.We recorded a 70% incidence of HLA-B5(51), which was very similar to that in Iraq,15 Turkey,20 and Japan,21 though it was not found in Britain.11 This figure contrasts with the 26% seen in normal persons in this population. However, information on the pathergy test in this study was too inadequate to look for a correlation between it and the presence of HLA-B5(51) as others have done.In contrast to the study by Rosenthal et al,10 this study showed that microscopic hematuria and/or proteinuria are rare in patients with Behcet's disease. Only one patient had proteinuria and hematuria, and renal biopsy showed moderate mesangial proliferative glomerulonephritis. Both Hamza et al22 and Oshima and Shimuzi12 reported renal biopsies with normal appearance or only minor pathologic changes, fibrosis around arterioles, and glomerular hypercellularity.ARTICLE REFERENCES:1. Behcet H. "Geschwure am Mund" . Dermatol Wochenschr. 1937; 105: 1152–7. Google Scholar2. Jorizzo JL, Hudson RD, Schmalstieg FC, et al. "Behcet's syndrome: immune regulation, circulating immune complexes, neutrophil migration, and colchicine therapy" . J Am Acad Dermatol. 1984; 10: 205–14. Google Scholar3. O'Duffy JD, Goldstein NP. "Neurologic involvement in seven patients with Behcet's disease" . Am J Med. 1976; 61: 170–8. Google Scholar4. Mason RM, Barnes CG. "Behcet's syndrome with arthritis" . Ann Rheum Dis. 1969; 25: 95–103. Google Scholar5. Berlin C. "Behcet's disease as a multiple symptom complex: report often cases" . Arch Rheumatol. 1960; 82: 127-73-9. Google Scholar6. Lehner T. "Oral ulceration and Behcet's syndrome: progress report" . Gut. 1977; 18: 491–511. Google Scholar7. Rogers RS. "Recurrent aphthous stomatitis: clinical characteristics and evidence for an immunopathogenesis" . J Invest Dermatol. 1977; 69: 499–509. Google Scholar8. Nazzaro PO. "Cutaneous manifestation of Behcet's disease: clinical and histological findings" . Proceedings of 15th International Symposium on Behcet's Disease, Rome, 1965. New York: Karger, 1966: 15–41. Google Scholar9. Madkour M, Kudwah F. "Behcet's disease (letter)" . Br Med J. 1978; 2: 1786. Google Scholar10. Rosenthal T, Weiss D, Gafni J. "Renal involvement in Behcet's syndrome" . Arch Intern Med. 1978; 138: 1122–4. Google Scholar11. Chamberlain MA. "Behcet's syndrome in 32 patients in Yorkshire" . Ann Rheum Dis. 1977; 36: 491–9. Google Scholar12. Oshima Y, Shimizu T. "Clinical studies on Behcet's syndrome" . Ann Rheum Dis. 1963; 22: 36–45. Google Scholar13. Wolf S, Schotland DL, Philip LL. "Involvement of the nervous system in Behcet's disease" . Arch Neurol. 1965; 12: 315–25. Google Scholar14. Mousa AM, Marafie AA, Rifai KM, et al. "Behcet disease in Kuwait and Saudi Arabia" . Scand J Rheumatol. 1986; 15: 310–32. Google Scholar15. Al-Rawi ZS, Sharquie KE, Khalifa SJ, Al-Hadithi FM. "Behcet disease in Iraqi patients" . Ann Rheum Dis. 1986; 45: 987–90. Google Scholar16. Schotland DL, Wolf SM, White HH, Dubin HV. "Neurological aspects of Behcet disease" . Am J Med. 1963; 34: 544. Google Scholar17. Mowat AG, Hothersall TE. "Gangrene in Behcet's syndrome" . Br Med J. 1969; 2: 636. Google Scholar18. Davies JD. "Behcet's syndrome with haemoptysis and pulmonary lesions" . J Pathol. 1973; 109: 351–6. Google Scholar19. Slavin RE, de Groot WJ. "Pathology of the lung in Behcet's disease" . Am J Surg Pathol. 1981; 5: 779–84. Google Scholar20. Takano M, Miyajima T, Kiuchi M, et al. "Behcet's disease and the HLA system" . Tissue Antigen. 1976; 8: 95–9. Google Scholar21. O'Duffy JD, Lehner T, Barnes CG. Close association HLABW51, Mt2 and Behcet's disease: summary of the international conference on Behcet's disease, Tokyo, Japan, 1981. J Rheumatol. 1983: 154–8. Google Scholar22. Hamza M, Zribi A, Chadli A, Benayed H. "La maladie de Behcet: etude de 22 cas" . Nouv Presse Med. 1975; 4: 563–6. Google Scholar Previous article Next article FiguresReferencesRelatedDetailsCited byRamani S (2019) Behcet's Disease in Saudi Arabia, Annals of Saudi Medicine , 10:3, (336-336), Online publication date: 1-May-1990.Amr S and Nasrallah N (2019) Renal Involvement in Behcet's Disease, Annals of Saudi Medicine , 10:3, (336-337), Online publication date: 1-May-1990.Al-Dalaan A, Al-Balla S, El-Ramahi K, Sieck J and Al-Arfaj H (2019) Pulmonary Involvement in Behcet's Disease, Annals of Saudi Medicine , 10:4, (414-419), Online publication date: 1-Jul-1990. Volume 9, Issue 4July 1989 Metrics History Accepted12 November 1988Published online1 July 1989 InformationCopyright © 1989, Annals of Saudi MedicinePDF download

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