Development of primary central nervous system lymphoma in a systemic lupus erythematosus patient after treatment with mycophenolate mofetil and review of the literature.

Primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin lymphoma and four cases of PCNSL have previously been described in association with mycophenolate mofetil. We report the fifth case of PCNSL in a patient with lupus nephropathy while on mycophenolate mofetil treatment.

The incidence and prevalence of systemic lupus erythematosus in Thrace, 2003-2014: A 12-year epidemiological study.

BACKGROUND: We estimated the prevalence and incidence, clinical features, treatment, and prognosis of systemic lupus erythematosus (SLE) patients in the Thrace region of Turkey. METHODS: We retrospectively evaluated 331 patients (307 female, 24 male, mean age 38.5 years) diagnosed with SLE between 2003 and 2014. Clinical features, treatments, and response to various treatment modalities were recorded. Our hospital has been the only tertiary referral center for rheumatological diseases for a mixed rural and urban population of 620,477 people (306,036 females, 314,411 males) for more than 16 years. RESULTS: The mean annual incidence of SLE was 4.44/100,000 (females, 8.4/100,000; males, 0.6/100,000). The overall prevalence of SLE was 51.7/100,000 (females, 97.7/100,000; males, 7/100,000). Major organ involvement was present in the following percentages: neurologic involvement: 20.1%; renal involvement: 28.2%; autoimmune hemolytic anemia: 9.6%; thrombocytopenia: 14.7%. Seventeen SLE patients (13 females, four males) died at a median follow-up of 48 months. The five-year survival was 94.5%, and the ten-year survival was 89.9%. According to Kaplan-Meier survival analysis, poor prognostic factors were: male gender (p = 0.015); smoking (p = 0.02); pleural involvement (p = 0.011); thrombocytopenia (p = 0.021); myocarditis (p = 0.028); renal involvement (p = 0.037); treatment with cyclophosphamide (p = 0.011); and an initial high SLEDAI score (>4) (p = 0.02). Lymphopenia at the time of diagnosis appeared as a favorable prognostic factor (p = 0.008). Cox regression analysis revealed myocarditis (OR: 20.4, p = 0.018) and age at diagnosis (OR: 1.11, p = 0.035) to be poor, and lymphopenia at the time of diagnosis to be good prognostic factors (OR:0.13, p = 0.031). CONCLUSIONS: The annual incidence and prevalence of SLE in the Thrace region of Turkey is lower than those reported in North America, however they are similar to those reported for European countries. Clinical manifestations appear to be milder, whereas survival was similar to those recorded in Western countries.

Validity and reliability of the Lupus QoL index in Turkish systemic lupus erythematosus patients.

BACKGROUND: Systemic lupus erythematosus (SLE) patients have seriously impaired quality of life (QoL). In addition to activity and damage indices used in the past, tools to evaluate QoL in SLE have been developed in recent years. In this study, we test the validity of the Turkish version of the Lupus-QoL (LupusQoL-TR) score, and investigate its association with clinical findings and activity indices. METHODS: A total of 132 patients diagnosed with SLE according to ACR 1997 criteria were included. The clinical and demographic features, and biochemical data were retrieved from hospital records. SLE Disease Activity Index (SLEDAI) and damage score (SLICC-ACR) were determined at the time of administration of Lupus-QoL questionnaire. The Lupus-QoL includes 34 questions divided into eight domains. We reevaluated the LupusQoL-TR and pretested its understandability. SLE patients were concomitantly administered the LupusQoL-TR and generic SF-36. Internal consistency, test-retest reliability, convergent and discriminant validity were calculated. RESULTS: The mean age of our SLE patients was 37.9 ± 12.8 years. Internal consistency reliability ranged from 0.88 to 0.93, and test-retest reliability from 0.84 to 0.94. LupusQoL-related domains in SF-36 were correlated (from 0.66 to 0.74). Most LupusQoL-TR domains, except planning, were able to discriminate between active and inactive SLE groups. Scores in all domains of the LupusQoL-TR were found to be discriminative for patients with and without damage according to SLICC-ACR score. CONCLUSION: The LupusQoL-TR was found to be a valid patient-reported outcome measure method when evaluating QoL in Turkish SLE patients.

Evaluation of early cardiac dysfunction in patients with systemic lupus erythematosus with or without anticardiolipin antibodies.

The aim of this study was to use transthoracic Doppler echocardiographic (TTE) imaging methods to identify cardiac dysfunction, an indicator of subclinical atherosclerosis in asymptomatic systemic lupus erythematosus (SLE) patients in terms of cardiac effects. This study involved 80 patients: a study group (n = 50) and control group (n = 30). They were categorized into four subgroups: anticardiolipin antibodies (aCL) (+) (n = 14) and aCL (-) (n = 36); systemic lupus erythematosus disease activity index (SLEDAI) ≥ 6 (n = 15) and SLEDAI < 6 (n = 35); disease period ≥ 5 years (n = 21) and disease period < 5 years (n = 29); major organ involvement (+) (n = 19), major organ involvement (-) (n = 31). The ratio of mitral peak velocity of early filling to early diastolic mitral annular velocity (E/E') for the study group was found to be higher than the control (p < 0.01). Systolic septal motion velocity (Ssm) was lower in the study group compared with the control (p < 0.01). Left atrium (LA) dimension was greater in the study group than the control (p < 0.01). Ssm was found to be lower in the aCL (+) patients compared with the control and aCL (-) groups (p < 0.01, p < 0.05, respectively). LA dimension was greater in the aCL (+) and (-) groups compared with the control, (p < 0.01, p < 0.05, respectively) and aCL groups compared with each other (p < 0.05). The E/E' ratio for the aCL (+) and (-) groups was found to be greater than the control (p < 0.05). In the study, both the Ssm and the late diastolic septal velocity (sA') was found to be lower in the SLEDAI ≥ 6 group compared with SLEDAI<6 group, (p < 0.001, p < 0.05, respectively). LA dimension was statistically greater in the SLEDAI ≥ 6 group compared with the SLEDAI <6 group (p < 0.001). E' and early diastolic septal velocity (sE') were statistically lower in the disease period >5 years group compared with the disease period <5 years group (p < 0.01, p < 0.05, respectively). Carrying out regular scans with TTE image of SLE patients is important in order to identify early cardiac involvement during monitoring and treatment. Identifying early cardiac involvement in SLE may lead to a reduction in mortality and morbidity rates.

Epidemiology and outcome of adult-onset Still's disease in Northwestern Thrace region in Turkey.

OBJECTIVES: Adult-onset Still's disease (AOSD) is a rare disease that is classified among the multifactorial autoinflammatory disorders. It is characterised by fever, arthritis and, a typical salmon-coloured rash, and is accompanied by fever at nights. Currently, there is limited data on the prevalence of AOSD. METHODS: Patients diagnosed with AOSD at the Department of Rheumatology of Trakya University Medical Faculty, between 2003 to 2014 were reviewed retrospectively. Patients' clinical features, laboratory measurements, demographics, treatments, follow-up durations, disease courses, outcomes and complications were evaluated. RESULTS: Our study included 42 patients with AOSD of whom, 32 (76.2%) were females and 10 (23.8%) were males (female to male ratio: 3.2). Over the course of the study, the annual incidence of AOSD was 0.62/100,000; and the overall prevalence was 6.77/100,000. The most common findings were fever (97.6%), arthralgia (95.2%), arthritis (76.2%), rash (73.8%) and sore throat (40.5%). CONCLUSIONS: In our hospital-based study on AOSD which is a disease with very limited epidemiological data, the frequency of AOSD was found to be significantly higher than in other series. Female gender was more common in our series; and polycyclic pattern was more common in patients with longer follow-ups.

The clinical manifestations and survival of systemic lupus erythematosus patients in Turkey: report from two centers.

BACKGROUND: Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with a variety of clinical features. Survival has become longer as a result of better treatment modalities and better supportive care. There is no information on survival of SLE patients in Turkey. We evaluated clinical features and survival in SLE patients in two rheumatology departments. METHODS: All SLE patients being followed up by the Department of Rheumatology, Trakya University Medical Faculty, and the Department of Rheumatology, Marmara University Medical Faculty, over the 1996-2012 period were included. Patients were diagnosed with SLE if they fulfilled at least four American College of Rheumatology (ACR) criteria. The clinical and laboratory features, mortality data were obtained from medical charts. RESULTS: We had 428 SLE patients, and women (399 patients, 93.2%) far outnumbered men (29 patients, 6.8%). The mean age at the time of SLE diagnosis was 40.3 ± 12.4 years. The most frequent clinical manifestations were arthritis (76.9%) and photosensitivity (70.1%). Renal disease was present in 32.9% of patients and neurological involvement in 12.9% of patients. After a median follow-up of 60 months, 19 patients died. The most frequent causes of death were ischemic heart disease, chronic renal failure and sepsis. The rate of five-year survival was 96%; 10-year survival, 92%; and 15-year survival, 88.8%. Multivariate Cox analysis showed that serositis at the time of diagnosis, SLE disease activity index (SLEDAI) score 6, and autoimmune hemolytic anemia were independent prognostic factors. CONCLUSIONS: Data from two centers in Northwestern Turkey show that the mortality rate for SLE is similar to the rate in Western countries.

Effect of fibromyalgia on bone mineral density in patients with fibromylagia and rheumatoid arthritis.

OBJECTIVES: Fibromyalgia (FM) may t cause a decrease in bone mineral density (BMD) because of decreased mobility. The condition is relatively frequent in rheumatoid arthritis (RA) and RA patients with FM have more disability than those without FM. We evaluated the effect of FM on BMD and investigated the effect of FM on BMD in RA patients. MATERIALS AND METHODS: We included age-matched 56 FM, 52 RA patients, and 37 healthy females as controls. Twenty three of all RA subjects met 1990 ACR FM criteria. Patients using the antiresorptive drugs, those on hormone replacement therapy, patients with thyroid or parathyroid dysfunction were excluded. Self-reported pain and fatigue severity, functional items of FM impact questionnaire were questioned in FM and RA patients. In all subjects, BMD of the lumbar spine and femur neck were determined by dual X-ray absorptiometry, and T-scores were recorded. RESULTS: Self-reported pain and fatigue scores in FM subjects were significantly higher than in RA patients (P<0.001). The mean lumbar spine and femur neck BMD and their T-scores in RA patients were significantly lower than in FM and control groups (P values<0.01). There was no difference in BMD between FM subjects and the control group. BMD in RA patients with and without FM were similar (P>0.05). There was a significant negative correlation between self-reported pain score and lumbar spine BMD in FM subjects (r=-0.41, P=0.006). CONCLUSIONS: In spite of functional disability, FM does not cause a decrease in BMD. The presence of FM in RA patients does not result in a change in BMD.

Are clinical features in Löfgren's syndrome-related erythema nodosum different from idiopathic erythema nodosum?

BACKGROUND AND OBJECTIVES: We retrospectively evaluated acute sarcoidosis (Löfgren's syndrome) patients diagnosed at 2 centers and compared the clinical features of Löfgren's syndrome (LS) related erythema nodosum (EN) to patients with idiopathic IEN who were diagnosed within the same time frame. METHODS: Thirty patients (10 males, 20 females) who were diagnosed with LS and were being followed up for the last 8 years at 2 centers were included. Thirty patients (4 males, 26 females) who were admitted to the rheumatology outpatient clinics for IEN during that time period were taken as controls. The clinical and laboratory features at the initial admission, treatment modalities and response were recorded. RESULTS: Twentyfour (80%) patients with LS related EN had arthritis and/or arthralgia. Fifteen of them had only findings of periarticular ankle inflammation and 4 had polyarthritis. When LS related EN patients were compared to IEN patients, the former group had more arthritis and/or arthralgia (p < 0.001), leucocytosis (p = 0.02), lymphopenia (p = 0.005) and thrombocytosis (p = 0.05), and higher ESR (p = 0.02). Twentyfive (83.3%) patients with LS related EN were administered oral corticosteroids. In 21 patients, hilar lymphadenopathy disappeared on control chest x-ray and CT; in 3 patients, minimal residual lymph node enlargement was persistent. During a median follow-up of 54 months (range: 10-84 months), none of the LS related EN patients had clinical relapse. CONCLUSIONS: Apart from BHL, arthritis and/or arthralgia especially periarticular ankle inflammation is the feature which could be used to differentiate LS related EN from IEN. There is more need for steroids in LS patients and the symptoms quickly resolve with steroids.

The development of trigeminal neuralgia related to auricular chondritis in a patient with rheumatoid arthritis-relapsing polychondritis and its treatment with etanercept. Description of the first case.

Cranial neuropathy is an uncommon manifestation of relapsing polychondritis (RPC). Optic neuropathy is the most common type of cranial nerve involvement in RPC. Until now, trigeminal neuralgia (TN) has been reported with different rheumatic diseases, however, there is no reported case of TN associated with RPC. We here present a case of RPC with TN. A 57 year-old female patient previously diagnosed with rheumatoid arthritis (RA) and RPC presented us with polyarthritis, auricular and nasal chondritis, and TN. Cranial MRI and MRI angiography of the brain did not show any pathology. The patient partially responded to RA therapy; and carbamazepine and etanercept were administered. RA-related joint findings, her chondritis and TN symptoms improved completely with etanercept. We presume that the TN was caused by compression of the trigeminal nerve from inflammation or ischemia secondary to vasculitis.

Giant cell arteritis and polymyalgia rheumatica in northwestern Turkey: Clinical features and epidemiological data.

OBJECTIVE: In this study, we evaluated clinical and epidemiologic features of our giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) patients. METHODS: We retrospectively recorded down the general features of patients with GCA and PMR diagnosed at our center within the last 6 years. The incidence rates per 100000 aged > or =50 were calculated. In addition, we reported the frequencies of GCA/PMR in our previous epidemiologic study. RESULTS: Nineteen patients were diagnosed with GCA (10F, 9M) and 53 with isolated PMR (39F, 14M). The annual incidence for GCA in subjects > or =50 years old was 1.13/100000, and for PMR it was 3.15/100000. The incidence of GCA and PMR in females were, respectively, 1.14/100000 and 4.48/100000. In males, the incidences of GCA and PMR were, respectively, calculated as 1.1/100000 and 1.72/100000. In our population-based study, the prevalences of GCA and PMR (> or =50 ages) were estimated as 20/100000. Fourteen (73.7%) GCA patients had symptoms of PMR. Two patients had developed unilateral and one patient bilateral permanent visual loss. Initial ESR was lower than 40 mm/hr in one GCA patient (5.3%) and in 6 PMR patients (11.3%). The median duration of follow-up was 16 months in GCA; and 8 months in PMR patients. One patient with PMR and another patient with GCA had lung cancer. One PMR patient had myelodysplastic syndrome. During follow-up, 4 patients with GCA died. CONCLUSION: We detected a lower frequency of GCA/PMR in our center in northwestern Turkey than in Scandinavian and southern European countries.

A low prevalance of purified protein derivative test positivity in Turkish patients with rheumatoid arthritis. Association with clinical features and HRCT findings.

OBJECTIVES: In this study, we aimed to evaluate the frequency of purified protein derivative (PPD) skin test positivity and associated clinical features in RA patients. MATERIALS AND METHODS: We included 94 (80 F, 14 M, mean age: 55.8) consecutive RA patients with a disease duration of 8.7 years. PPD test was performed in all RA patients; clinical features were recorded down; chest x-ray, pulmonary function tests and HRCT were available in all cases. As the control group, we included data of 21 SLE, 44 AS, 27 OA, 16 gouty arthritis and 18 vasculitis patients. RESULTS: The frequencies of PPD positivity in RA (29.8%) and SLE (19%) patients were lower than in patients with AS (65.9%), gouty arthritis (68.8%) and OA (63%) (all p values < 0.01). PPD-positive RA patients were more frequently smokers (p = 0.005) and had a higher rate of RF seropositivity (p = 0.04) than PPD-negatives. PPD was less frequently positive in erosive RA disease (p = 0.033). Chest x-rays and HRCT abnormalities were detected in 41.8% and 62.7% of RA patients, respectively. Frequencies of chest x-ray and HRCT abnormalities in PPD-positive and PPD-negative patients were not different from each other (p > 0.05). CONCLUSION: In our country in which tuberculosis is relatively frequent -contrary to the situation in AS patients- we observed a lower frequency of PPD positivity in RA and SLE patients compared to patients with other rheumatic diseases. We did not find any relationship between PPD positivity and the frequency of chest x-ray, HRCT abnormalities.

An increased frequency of gallbladder stones in rheumatoid arthritis patients. Factors related to gallbladder stone formation.

OBJECTIVE: In this study, we determined the frequency of gallbladder stone (GBS) in rheumatoid arthritis (RA) patients and evaluated factors which could affect the formation of GBS--such as lipids and the GB motilities of the patients. METHODS: One hundred and thirteen RA patients (92F, 21M, mean disease duration: 8.9 years) and 117 healthy controls (94F, 23M) were included. In all RA patients, the clinical findings were recorded down; biochemical parameters and body mass index (BMI) were determined; and, abdominal ultrasonography was performed. In addition, 16 RA patients and 20 controls who were age-matched were randomly chosen for GB emptying monitored by ultrasound at 30-minute intervals for 2 hours after a mixed meal. Fasting volume (FV), residual volume (RV) and ejection fraction (EF) for all GBs were assessed. RESULTS: There was a tendency towards a higher frequency of GBS including cholecystectomy (11 GBS, 11 cholecystectomy, 19.5%) in RA patients when compared to controls (8 GBS, 5 cholecystectomy, 11.1%) (p = 0.08). The frequency of GBS plus cholecyctectomy in female RA patients (22.8%) was significantly higher than the control group (11.7%, p = 0.044). Logistic regression analysis showed that only older age was significantly associated with the presence of GBS in RA (OR:1.05, p = 0.048). There was no difference between the 2 groups in FV (p > 0.05). RV, PRV and EF were significantly higher in RA patients than in the control group (p < 0.05). CONCLUSION: We diagnosed a higher frequency of GBS in female RA patients when compared to controls. Impaired GB motility in RA patients might contribute to an increased incidence of GBS development.

The variation in chronic widespread pain and other symptoms in fibromyalgia patients. The effects of menses and menopause.

OBJECTIVES: We determined the relationship between the menstrual cycle and fibromyalgia (FM) symptoms in premenopausal women. In addition, we compared the clinical features of FM patients diagnosed pre-and postmenopausally. METHODS: We included 80 premenopausal, and 72 postmenopausal patients with FM. All patients were questioned about the severity of their pain and symptoms of FM by using a visual analog scale (VAS). In addition, the patients were asked questions about symptoms of somatization, depression and anxiety. Postmenopausal subjects were asked about the change in their FM symptoms with the onset of menopause; and premenopausal subjects were asked whether their FM symptoms changed during the menses. In addition, 40 premenopausal patients were requested to fill in a diary about their FM symptoms using VAS throughout one menstrual cycle. RESULTS: Postmenopausal patients had more severe pain on VAS (p = 0.048). Of all the postmenopausal females, 25% said that their FM symptoms started with the onset of menopause and 26.4% said that the severity of their previous symptoms increased after menopause. Of all the premenopausal females, 45% admitted to higher pain severity and 57.5% to a higher fatigue severity during the menses. The patients who defined an increase in their symptoms during the menses were the ones with higher sleep disturbance scores, more somatization symptoms and more tender points (p values < 0.05). The results of the diaries revealed that the mean pain and fatigue scores in the menstrual and luteal phases were higher than the scores in the follicular and premenstrual phases (p values < 0.05). CONCLUSIONS: The menstrual cycle and the onset of menopause affect pain and the severity of other FM-related symptoms in approximately one half of the subjects.

Henoch-Schönlein purpura in two brothers imprisoned in the same jail: presentation two months apart.

We present two brothers who came to us with similar complaints within a two-month interval and who were diagnosed as having Henoch-Schönlein purpura. Interestingly, the two brothers were prisoners in the same jail. In addition, we shall review the small number of familial Henoch-Schönlein purpura cases that have been reported in the literature to date. Our two patients exhibited arthritis and/or arthralgia, purpuric skin lesions, abdominal pain and hemiaturia, and were treated with steroids. We did not detect the presence of any inciting agent and hypothesize that an undefined factor present in the shared environment might have triggered the disease in two subjects with a similar genetic background.

Colchicine neuromyopathy: a report of six cases.

Colchicine has been in use for therapeutic purposes for many years. It can, however, cause subacute onset muscle and peripheral nerve toxicity in patients with chronic renal failure. In this report we describe 6 patients who developed neuromyopathy after the administration of colchicine. All patients presented with proximal muscle weakness, elevated serum creatine kinase (CK) levels, and neuropathy and/or myopathy on electromyography (EMG). The diagnosis of colchicine toxicity was confirmed in all cases by the normalization of CK levels and EMG after discontinuation of the drug. Toxicity developed in 4 renal failure patients on therapeutic doses of the drug, while one patient took a massive dose for suicidal reasons, and the other was on high-dose therapy. Patients using colchicine--especially those with renal failure--should be warned about the side effects of the drug and physicians should be careful in the administration of the drug.

Prevalence of Behçet's disease in rural western Turkey: a preliminary report.

OBJECTIVES: To study the prevalence of Behçet's disease (BD) in a rural area of Western Turkey. METHODS: This study was conducted in seven villages in the rural Havsa region which has a total population of 5727. The physicians and medical students visited every household and distributed a questionnaire on the symptoms of rheumatic disorders. One of the questions was whether they had ever suffered from mouth ulcers. If the response was affirmative the proband was examined by a rheumatologist and a dermatologist for other symptoms. The individuals with true aphthous ulcerations had pathergy tests and were invited to the hospital for ophtalmic examinations. RESULTS: Of the 5203 individuals who participated in this survey, 4861 were 10 years or older. 124 out of 190 individuals who responded positively to the question regarding the presence of mouth ulcers had true aphthous ulcerations. 115/124 underwent a pathergy test and 4 showed positive reactions without any other stigmata of BD, except for folliculitis in one. Among the individuals with a negative pathergy test, 2 had acneiform skin lesions and 1 a genital ulcer. No individual exhibited any ocular symptoms or findings characteristic for BD. Only 1 person with aphthous ulceration, folliculitis and a positive pathergy test was considered to have BD. CONCLUSION: In this survey, the prevalence of BD was found to be 2: 10,000 (95% confidence interval 0 to 4.3: 10,000). This figure is lower than what has been reported in four previous studies from different regions in Turkey. This finding might be due to ethnic distribution and geographical differences.

Prevalence of serum antibodies to hepatitis B and C viruses in patients with primary glomerulonephritis.

We retrospectively analysed the medical records of patients who underwent renal biopsy in our nephrology department over the past 8 years. Our aim was to discover the frequency of hepatitis B (HBV) and hepatitis C virus (HCV) in patients with primary glomerulonephritis (GN) in Turkey, a moderately endemic country for both infections. The study included 144 male and 104 female patients aged between 12-70 years. The frequency of HBsAg and anti-HCV were found to be similar to healthy controls, respectively 6.5% and 1.6% (p>0.05). As is true for low endemic countries, HBV and HCV infections might be rarely associated with GN in Turkey.

Amyloid goitre in familial Mediterranean fever: report on three patients and review of the literature.

Familial Mediterranean fever (FMF) is a hereditary disease, the most threatening complication of which is systemic amyloidosis. The thyroid gland may be asymptomatically involved in most patients with systemic amyloidosis secondary to FMF. However, clinically detectable thyroid goitre is quite rare, and until now only nine cases of thyroid goitre secondary to amyloid deposition in FMF have been reported. Of 1,100 FMF patients regularly followed up at our centre, thyroid goitre due to the accumulation of amyloid substance could be detected in only three (0.27%). In this report, we summarise the clinical and laboratory features of these patients. All three patients were euthyroid. Total thyroidectomy was performed for compressive symptoms in one patient and for aesthetic purposes in the other two. In countries with a high prevalence of FMF, such as Turkey, secondary amyloidosis of the thyroid gland should be borne in mind in long-standing FMF patients.

Glomerulonephritis in Behçet's disease: report of seven cases and review of the literature.

Despite being recognised much more frequently than in the past, renal involvement has not previously been regarded as a feature of Behcet's disease (BD). In this study we aimed to assess the frequency of renal involvement in BD by performing urinalyses of 674 consecutive BD patients; we also retrospectively evaluated the charts of 4212 BD patients for the incidence of glomerulonephritis (GN). Urinary abnormalities (proteinuria and/or haematuria) were present in 10.8%; and during a period of 23 years GN was detected by renal biopsy in seven (0.16%) BD patients. Two patients with GN were lost to follow-up; end-stage renal failure developed in only one patient, and she underwent renal transplantation. We were unable to determine any pathognomonic feature that was predictive of renal involvement. Although males tend to have a more serious clinical course of BD the incidences of urinary abnormalities and GN were similar in both sexes in our series. According to our results, we can conclude that urinary abnormalities are more frequent in BD; however, serious renal lesions develop in only very few of these patients.

Secondary amyloidosis in progressive systemic sclerosis.

Progressive systemic sclerosis (PSS) is a connective tissue disease that may affect many organs, including the kidneys. It is quite rare to see secondary amyloidosis due to PSS. We present a patient with a 9-year history of PSS who developed nephrotic syndrome, and whose renal biopsy was compatible with secondary amyloidosis. He died from massive upper gastrointestinal bleeding caused by oesophageal telangiectasia.