Ömer Nuri Pamuk

Cutaneous involvement in sarcoidosis: analysis of the features in 170 patients

In our study, we retrospectively evaluated the clinical features of patients diagnosed as sarcoidosis at our center within the last 36 years and who had skin involvement. Cutaneous involvement was observed in 170 patients (32.9%, 136 females, 34 males). The most frequent skin lesion was erythema nodosum (EN) (106 subjects, 20.5%). In addition, skin plaques and subcutaneous nodules were observed in 22 cases (4.3%), maculopapular eruptions in 19 cases (3.7%), scar lesions in 15 cases (2.9%), lupus pernio (LP) in 14 cases (2.7%) and psoriasiform plaques in five cases (0.9%). Among patients with LP (64.3%) and scar lesions (40%), pulmonary parenchymal involvement was more frequent than patients with other skin lesions. Parenchymal involvement present in 10.4% of patients with EN was significantly less than in patients with LP and scar lesions (P values, respectively, <0.001, 0.002). When patients with skin involvement were compared to other sarcoidosis patients, it was seen that the frequency of females among those with skin involvement was significantly higher than the frequency among other sarcoidosis patients (P<0.001). Parenchymal involvement in sarcoidosis patients without skin involvement was less frequent than in patients with LP; however, more frequent than in patients with EN (both P values=0.002). As a conclusion, skin involvement was diagnosed in approximately one-third of our sarcoidosis patients with a generally female predominance. EN was the most frequent skin lesion encountered. Parenchymal involvement was more frequent in patients with LP and scar lesions and less frequent in patients with EN.

Overview of 321 patients with idiopathic thrombocytopenic purpura: Retrospective analysis of the clinical features and response to therapy

In this retrospective study, we evaluated the clinical features and the effects of various treatment modalities on the clinical course in patients diagnosed with idiopathic thrombocytopenic purpura (ITP) at our center between 1984–2000. We retrospectively examined the medical records of 321 (229 females, 92 males) ITP patients. One hundred and seventy-one (53.3%) patients were lost to follow-up. When evaluating the clinical features, all 321 patients were included; however, when the response to treatment modalities was evaluated only 150 patients followed up regularly were considered. The median age of the patients on initial diagnosis was 34xa0years (range: 14–78). At initial diagnosis, 235 (73.2%) patients had signs of bleeding. Of patients diagnosed with ITP initially, six later turned out to have systemic lupus erythematosus (SLE) and two myelodysplastic syndrome (MDS). The median follow-up of 150 patients followed up regularly was 30xa0months (range: 4–396). One hundred and thirty-seven of these subjects had an indication for treatment and 94.2% of them were administered either standard or high-dose steroids as the first-line therapy. Complete remission (CR) was defined as any platelet count >100,000/mm3 lasting for 3xa0months or longer without treatment. CR was achieved in 51.9% of the patients given steroids as the initial therapy. During a median follow-up of 33xa0months, relapse occurred in 58.2% of these patients, and after a median follow-up of 11xa0months the rest of them were still in remission. Ninety-eight patients followed up regularly were administered second-line therapies. CR was obtained in 44.4% of the patients who used steroids as second-line therapy. Within a median follow-up of 15xa0months, 20.8% of these patients relapsed. Splenectomy was performed in 76 patients and CR was obtained in 68.4% of the regularly followed up patients. Relapse occurred within a median of 96xa0months in 15.4% of the patients who had CR. Kaplan-Meier curves showed that the duration of CR obtained by splenectomy was significantly higher than that obtained by steroids (p<0.001). The 10-year disease-free survivals in patients who used steroids and who underwent splenectomy were, respectively, 13% and 58%. In our adult ITP patients, steroids induced nearly similar rates of CR both as first-and second-line therapies. Splenectomy seems to be effective in patients unresponsive to steroids. The duration of CR obtained by splenectomy is significantly longer when compared with the duration of CR obtained by steroid therapy.

The frequency of thyroid antibodies in fibromyalgia patients and their relationship with symptoms.

We determined the frequency of thyroid autoantibodies in fibromyalgia (FM) patients and the relationship between FM symptoms and these antibodies. Euthyroid 128 FM patients, 64 rheumatoid arthritis (RA) patients, and 64 healthy control subjects were included in the study. The sociodemographic features and the clinical features of FM patients were determined. By using a visual analog scale, patients were questioned about the severity of FM-related symptoms. All patients were administered with Duke-Anxiety Depression (Duke-AD) scale, the physical function items of the fibromyalgia impact questionnaire scale. Thyroid autoimmunity was defined as the presence of detectable antithyroglobulin (TgAb) and/or antithyroid peroxidase (TPOAb) antibodies by the immunometric methods. Patients with a connective tissue disorder, hypo- or hyperthyroidism, and patients who had psychiatric treatment within the last 6xa0months were not included into the study. The frequencies of thyroid autoimmunity in FM (34.4%) and RA (29.7%) patients were significantly higher than controls (18.8%) (p<0.05). Twenty-six (20.3%) FM patients had positive TgAb and 31 (24.2%) had positive TPOAb. When patients with thyroid autoimmunity were compared to others, it was seen that the mean age, the percentage of postmenopausal patients, the frequency of dryness of the mouth, and the percentage of patients with a previous psychiatric treatment were higher in this group (p<0.05). FM patients had thyroid autoimmunity similar to the frequency in RA and higher than controls. Age and postmenopausal status seemed to be associated with thyroid autoimmunity in FM patients. The presence of thyroid autoimmunity had no relationship with the depression scores of FM patients.

Diarrhoea following renal transplantation

In this study, we retrospectively evaluated all attacks of diarrhoea in our renal transplant recipients that came to our medical attention between 1985 and 2000. Also, the clinical features of patients with diarrhoea were compared with the features of recipients without diarrhoea. We diagnosed 41 attacks of diarrhoea in 39 (12.6%) of 308 renal transplant recipients during this time period. An aetiology was detected in 33 (80.5%) of all diarrhoeal episodes and in seven (17.1%) of those the specific agent was diagnosed with the help of stool microscopy. The most frequent causes of diarrhoeal attacks were infectious agents (41.5%) and drugs (34%). Six (14.6%) episodes of diarrhoea were chronic and six were nosocomial. About two‐thirds of diarrhoea developed within the late post‐transplant period (>6u2003months). When recipients with diarrhoea were compared with those without diarrhoea, it was seen that diarrhoeal patients had significantly higher creatinine and significantly lower albumin levels when compared with the latter group (pu2003<u20030.05). Also, the frequency of antibiotic usage was significantly higher in diarrhoeal patients than in the control group (pu2003<u20030.05). Four (10.2%) patients with diarrhoea died despite institution of the appropriate therapy. Two of these deaths were primarily related to diarrhoea and the aetiological agent was Clostridium difficile in both these cases. During the 15‐yr study period, 3.6% of all deaths and 5.1% of infection‐related deaths in transplant recipients were secondary to diarrhoea. As a result, we observed that infections and drugs were the most frequent causes for diarrhoea in our series of renal transplant recipients. Also, diarrhoea was an important cause of mortality in this patient population.

A primary Sjögren's syndrome patient with distal renal tubular acidosis, who presented with symptoms of hypokalemic periodic paralysis: Report of a case study and review of the literature.

Although renal tubular acidosis (RTA), secondary to autoimmune interstitial nephritis, develops in a large proportion of patients with Sjögren’s syndrome (SS), most of the subjects are asymptomatic. Here, we shall present a 39-year-old female patient who came to us with hypokalemic periodic paralysis (HPP), and who was later diagnosed with distal RTA. The patient, who had xerostomia and xerophthalmia for a long period of time, was diagnosed with primary SS from serologic and histologic findings. The patient recovered by being prescribed potassium replacement therapy. Although renal biopsy was not performed, corticosteroids were administered because HPP indicated severe interstitial nephritis. HPP did not reoccur during a 2-year follow-up period. We also review cases with SS-related distal RTA and HPP.

Increased circulating platelet-leucocyte complexes in patients with primary Raynaud's phenomenon and Raynaud's phenomenon secondary to systemic sclerosis : a comparative study

Platelet activation and circulating platelet–leucocyte complexes increase in vascular ischemic events and autoimmune inflammatory diseases. Platelet activation markers and platelet–leucocyte complexes were evaluated in primary Raynauds phenomenon (RP) and in RP secondary to systemic sclerosis (SSc). Whole-blood flow cytometry was utilized to quantify CD62P, platelet microparticles (PMP), platelet–monocyte complexes (PMC) and platelet–neutrophil complexes (PNC) in primary RP and in SSc patients with secondary RP. SSc patients with secondary RP had significantly higher platelet CD62P expression than primary RP patients and controls (P = 0.017 and 0.004, respectively). Primary and secondary RP patients had higher mean PMC and PNC levels than controls (all P ≤ 0.001). PMP level in SSc patients with pulmonary hypertension was significantly higher than in others (P = 0.048). All parameters were similar in SSc patients with and without digital ulcers, aspirin-users and nonusers (P > 0.05). CD62P level decreased significantly after iloprost administration in four patients with digital ulcers (16.1 ± 17.4 vs 7.4 ± 3.8%, P = 0.03). Our results suggest there is platelet–leucocyte complex formation in RP, and, despite antithrombotic therapy, platelet activation and platelet–leucocyte interaction are ongoing in SSc. This is important as it might have potential therapeutic implications with respect to using antiplatelet drugs in SSc.

Incidence of gallstones in chronic renal failure patients undergoing hemodialysis: experience of a center in Turkey.

OBJECTIVES:In this case–control study, we sought to determine whether the incidence of gallbladder stones (GBS) was increased in chronic renal failure (CRF) patients on a hemodialysis (HD) program. We also evaluated factors, such as lipid profiles and gallbladder motility, that could affect the formation of GBS. In addition, we reviewed other available studies on this subject and compared the factors that might have some influence on the development of GBS.METHODS:A total of 182 CRF patients (135 male, 47 female, mean age 32.1 yr) undergoing chronic HD and who were referred to our transplantation center in the last 10 yr and 194 healthy controls (137 male, 57 female, mean age 33.3 yr) were included in the study. Abdominal ultrasound was performed on all patients, and ALT, AST, and lipid profiles were determined. In addition, 19 patients with CRF (12 male, 7 female, mean age 33.5 yr) and 22 controls (14 male, 8 female, mean age 33.2 yr) who were age and sex matched were randomly chosen for gallbladder emptying, monitored by ultrasound at 30-min intervals for 2 h after a mixed meal. Fasting volume, minimal residual volume, and ejection fraction of the gallbladder were assessed. For statistical analysis, χ2, t test, and logistic regression analysis were used.RESULTS:GBS were detected in seven patients with CRF (3.85%, 5 male, 2 female) and three controls (1.55%, one male, two female) (p > 0.05). The mean follow-up time of CRF patients after diagnosis was 39.3 months (range: 2–168), the mean duration of HD was 21.8 months (range: 1–120). The analysis of seven stones in the CRF group revealed that five were cholesterol-rich stones, and two were mixed (cholesterol and bilirubin) stones. Cholesterol levels were higher in the control group, and triglycerides were higher in the CRF group, but these findings were nonsignificant (p > 0.05). Other biochemical values were not significantly different between the groups. CRF patients with and without GBS were similar in their duration of CRF and HD, age, and other biochemical parameters (p > 0.05). When gallbladder emptying was considered, there was no difference between the two groups in fasting volume, residual volume, and ejection fraction (CRF: 89.7%; controls: 92.3%) of the gallbladders (p > 0.05).CONCLUSIONS:We detected similar incidences of GBS in CRF patients undergoing HD and healthy controls, and this was comparable to the results of most of the previous studies. Young male CRF patients had a nonsignificantly higher incidence of GBS than control males. Although cholesterol-rich GBS were predominant, we could not find any significant difference between the groups when factors that could affect GBS formation, such as lipid profiles and gallbladder motility, were taken into account.

Lupus pernio in sarcoidosis: clinical features and treatment outcomes of 14 patients.

Lupus pernio (LP) is the most characteristic skin lesion of sarcoidosis. In this study, we retrospectively evaluated the clinical features of sarcoidosis patients with diagnosed LP at our center. Of 516 sarcoidosis patients diagnosed within a 36-year period, 14 (2.7%) had skin lesions that were clinically and histologically diagnosed as LP. Thirteen of our LP patients were females, and one was a male (mean age: 46.3, range: 24-67). In 2 sarcoidosis patients, the initial presentation of the disease was LP. When the LP patients were compared with other sarcoidosis patients, there were more females, and the frequency of extrapulmonary involvement and the number of patients with advanced stage disease were higher (P <0.001). u2003Oral and/or intralesional steroid therapy was the preferred treatment modality in all our patients and led to either recovery or regression in most patients with LP. As our study was an uncontrolled, retrospective one with few patients, it is difficult to say whether steroids are effective. LP runs a chronic course and spontaneous remission of lesions of more than 2 years’ duration is quite rare. Because of the unwanted side effects of steroids, the efficacy of new treatment modalities should be tested especially in sarcoidosis patients with only skin involvement.

Brucella Glomerulonephritis: Review of the Literature and Report on the First Patient with Brucellosis and Mesangiocapillary Glomerulonephritis

Although mild proteinuria is commonly observed during the course of brucellosis, biopsy-proven glomerulonephritis (GN) is quite rare. We present the first case of mesangiocapillary glomerulonephritis (MCGN) associated with brucellosis and summarize all cases of Brucella GN published to date. Our patient, who had a congenital bicuspid aortic valve, also had heart failure, fever, urinary abnormalities and proteinuria. Renal biopsy revealed MCGN. Although the clinical features raised the possibility of GN associated with endocarditis, transesophageal echocardiography did not show any vegetations.

Secondary amyloidosis in Castleman's disease: review of the literature and report of a case

Abstract. It is quite rare to diagnose secondary amyloidosis during the course of Castlemans disease (CD). A 51-year-old female who complained of fatigue, weight loss, and fever was diagnosed with CD – plasma cell type – in our hospital in 1993. One year after diagnosis, she developed nephrotic syndrome, the etiology of which was found to be secondary amyloidosis based on renal biopsy. As the patient rejected therapy, she was discharged after only symptomatic treatment. At her last follow-up in March 2001, she had no complaints; physical examination, blood chemistries, and urinalysis were normal. Abdominopelvic tomography revealed no lymphadenopathy in the abdomen, which had been previously present. We could identify 17 other cases of CD with secondary amyloidosis in the literature. Ours is the 18th such case and the 2nd case of multicentric CD leading to amyloidosis. This case also shows that CD might sometimes run a relatively benign course being cured with no therapy, whereas it might have a rapidly fatal downhill course – even with therapy – in others. Still, effective treatment strategies need to be developed.