The relationship between nailfold capillaroscopic assessment and telangiectasia score with severity of peripheral vascular involvement in systemic sclerosis.

OBJECTIVES: To determine the association of nailfold video-capillaroscopy (NVC) findings and telangiectasia score with digital ulcer (DU) history and severity of peripheral vascular involvement (PVI) in systemic sclerosis (SSc). METHODS: Fifty-nine SSc patients fulfilling Leroy & Medsger criteria were evaluated including telangiectasia score, disease activity and severity scores. NVC was performed according to qualitative (early, active and late patterns) and semi-quantitative assessments. RESULTS: When DU+ and DU- groups were compared; the mean score of capillary number (CN) was 2.0±0.5 vs. 1.4±0.7 (p<0.001), irregularly enlarged capillaries (IEC) was 1.8±0.6 vs. 1.4±0.7 (p<0.05), microangiopathy evolution score (MES) was 2.5±1.5 vs. 1.8±1.0 (p<0.05) and 'early' pattern was significantly less frequent in DU+ patients (1 vs. 9, p=0.016). The frequency of severe-PVI (Medsger severity score of 2-4) was 22% in females (12/54) and 80% in males (4/5). When severe and non-severe groups were compared; the mean score of CN was 2.1±0.4 vs. 1.5±0.7 (p<0.001), MES was 2.8±1.6 vs. 1.8±1.1 (p<0.05) and 'early' pattern was significantly less frequent in patients with severe PVI (0 vs. 9, p=0.049). The mean values of telangiectasia score were similar between groups. CONCLUSIONS: DU history and severe PVI in SSc were associated with capillary loss and microangiopathy. 'Early' NVC pattern was very rare in patients with DU history and was not found in severe PVI. Severe PVI in males was more frequent than females. Telangiectasia scores were not found to be related to PVI. NVC may be a helpful method in the assessment of SSc patients for PVI prognosis, warranting prospective studies.

The Relationship between P-Selectin Polymorphisms and Thrombosis in Antiphospholipid Syndrome: A Pilot Case-Control Study.

OBJECTIVE: The selectins are cell adhesion molecules that mediate the interactions among leukocytes, activated platelets, and endothelial cells. We aimed to investigate whether P-selectin polymorphisms are associated with thrombosis in patients with antiphospholipid syndrome (APS). MATERIALS AND METHODS: The diagnosis and classification of APS were based on the report of an international workshop. Genomic DNA was extracted from citrated blood samples of all subjects. Three single nucleotide polymorphisms associated with the P-selectin coding region (S290N, c.1087G>A; N562D, c.1902G>A; T715P, c.2363A>C) were assessed. RESULTS: There were 26 APS (65%) patients with thrombosis. The number of patients without thrombosis was 14 (35%). The frequency of the N562D-DN genotype was significantly higher in patients with APS than in healthy controls (p=0.003). The frequency of this genotype was significantly higher in patients with APS with thrombosis compared with patients with no thrombosis (p=0.03). The N562D-NN genotype was found at a higher frequency in patients with APS than in healthy controls (p=0.004). CONCLUSION: Our results suggest that the N562D polymorphism of the DN genotype of P-selectin is associated with an increased risk of thrombosis in patients with APS.

Efficacy of anakinra treatment in a patient with colchicine-resistant familial Mediterranean fever.

Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by self-limited recurrent attacks of fever and serositis. The serious complication of FMF is AA-type amyloidosis, which can result in end-stage renal disease. Although colchicine is effective in the majority of patients, there is no established treatment for those who are resistant or intolerant to colchicine. We herein report the efficacy of anakinra in a 52-year-old Turkish patient with FMF, secondary amyloidosis and renal transplant, who was resistant to colchicine treatment.

Improvement of coronary flow velocity reserve with telmisartan in patients with autosomal-dominant polycystic kidney disease.

OBJECTIVES: Endothelial dysfunction (ED) has been reported in patients with autosomal-dominant polycystic kidney disease (ADPKD). Coronary flow velocity reserve (CFVR) is a noninvasive test showing endothelial function of epicardial coronary arteries and coronary microcirculatory function. The aim of this study was to investigate the effect of the angiotensin receptor blocker, telmisartan, on CFVR in patients with ADPKD. METHODS: Thirteen patients with ADPKD and well-preserved renal function and 22 healthy controls were included in the study. CFVR was measured at baseline and after dipyridamole infusion by echocardiography. CFVR was calculated as the ratio of hyperemic to baseline average peak diastolic velocities. After the baseline evaluation of CFVR, patients started telmisartan at a dose of 80 mg/day and were followed for 12 months. CFVR was remeasured after 6 and 12 months of therapy. RESULTS: Patients with ADPKD had significantly lower CFVR compared to healthy subjects. CFVR increased significantly after 6 months and 12 months of telmisartan therapy (P = 0.001) in patients with ADPKD. CONCLUSION: One year of telmisartan therapy significantly improved CFVR in patients with ADPKD. This finding suggests that the stimulation of the renin-angiotensin-aldosterone system contributes to the ED in these patients.

Thrombotic thrombocytopenic purpura associated with multiple myeloma.

Thrombotic thrombocytopenic purpura is a thrombotic microangiopathic disease. Associations with collagen vascular diseases, pregnancy, some drugs, surgical intervention, and infections are documented (known). Malignancy is also one of the known factors associated with thrombotic thrombocytopenic purpura. These malignancies are usually the disseminated solid organ tumors. Hematological malignancies constitute a rare association. Here, we present a patient with thrombotic thrombocytopenic purpura associated with multiple myeloma and discuss the pathogenesis. To our knowledge, this is the first case report of thrombotic thrombocytopenic purpura associated with multiple myeloma.

Leukocytoclastic vasculitis due to thalidomide in multiple myeloma.

Thalidomide is successfully used in the treatment of multiple myeloma, leprosy and various autoimmune diseases due to its anti-angiogenic, immunomodulatory and anti-inflammatory effects. Thalidomide's most common side effects are constipation, neuropathy, fatigue, sedation, rash, tremor and peripheral edema. We achieved complete response with a 400 mg/day dose thalidomide therapy in a 58-year-old male patient diagnosed with relapsing refractory multiple myeloma. While continuing thalidomide for sustainable response, the therapy was terminated at the ninth month due to development of leukocytoclastic vasculitis. We describe the case and discuss the place of thalidomide in the treatment of multiple myeloma and the rare occurrence of leukocytoclastic vasculitis during thalidomide therapy in multiple myeloma, since only one such case has been reported in the literature thus far.

Hemophagocytic Syndrome Associated with Immune Reconstitution Inflammatory Syndrome in a Patient with AIDS Related Burkitt's Leukemia/Lymphoma.

Highly active antiretroviral therapy (HAART) has markedly decreased human immunodeficiency virus- (HIV-) related mortality and the incidence of opportunistic infections. The dramatic reduction in HIV-1 RNA and increase in CD4 lymphocyte count mean a recovery in immune function. This restoration in immune function may be associated with paradoxical deterioration in subclinical opportunistic infections in some patients, a condition called immune reconstitution inflammatory syndrome (IRIS). IRIS, a "paradoxical" inflammatory response to either previously treated or subclinical infections or noninfectious diseases, can manifest during the restoration phase of immunity hemophagocytic syndrome (HS) which is a very rare complication in patients with acquired immune deficiency syndrome (AIDS). We describe a case of hemophagocytic syndrome associated with IRIS in a patient with AIDS related Burkitt's leukemia/lymphoma (BL). IRIS was probably the cause of hemophagocytosis for our patient. Zoster infection may facilitate to IRIS. With the increasing number of people with HIV infection and the accompanying use of HAART, much more clinical manifestations of IRIS will be experienced especially in patients given high dose chemotherapy, just like in our case.

The causes of acute Fever requiring hospitalization in geriatric patients: comparison of infectious and noninfectious etiology.

Introduction. Infectious diseases may present with atypical presentations in the geriatric patients. While fever is an important finding of infections, it may also be a sign of noninfectious etiology. Methods. Geriatric patients who were hospitalized for acute fever in our infectious diseases unit were included. Acute fever was defined as presentation within the first week of fever above 37.3°C. Results. 185 patients were included (82 males and 103 females). Mean age was 69.7 ± 7.5 years. The cause of fever was an infectious disease in 135 and noninfectious disease in 32 and unknown in 18 of the patients. The most common infectious etiologies were respiratory tract infections (n = 46), urinary tract infections (n = 26), and skin and soft tissue infections (n = 23). Noninfectious causes of fever were rheumatic diseases (n = 8), solid tumors (n = 7), hematological diseases (n = 10), and vasculitis (n = 7). A noninfectious cause of fever was present in one patient with no underlying diseases and in 31 of 130 patients with underlying diseases. Conclusion. Geriatric patients with no underlying diseases generally had infectious causes of fever while noninfectious causes were responsible from fever in an important proportion of patients with underlying diseases.

Amyloid arthropathy mimicking seronegative rheumatoid arthritis in multiple myeloma: case reports and review of the literature.

We report two patients who suffered from symmetrical polyarthritis simulating rheumatoid arthritis. Acute phase response was almost within normal limits, and autoantibodies including rheumatoid factor were negative. Both of them were diagnosed as having amyloid arthropathy (AmyA) secondary to kappa multiple myeloma based on deposition of kappa-light chain-immunoreactive amyloid in biopsied tissue and Bence Jones protein in urine. Systemic AL amyloidosis may be important in the differential diagnosis of chronic polyarthralgia.

Amphotericin B-induced cutaneous leucocytoclastic vasculitis: case report.

A 37-year-old female patient was diagnosed with ulcerative colitis 8 months ago and medical treatment with oral azathioprine, low-dose corticosteroids and 5-ASA was started. Following 3 months without any symptoms, the patient had total colectomy and ileostomy. After this period, liposomal amphotericin B (3 mg kg(-1) day(-1)) was given with the diagnosis of probable fungal infection. Palpable purpuric skin lesions on the anterior surface of both legs appeared on the 55th day of amphotericin B treatment. Histological examination of a skin biopsy was consistent with leucocytoclastic vasculitis. We present a case of cutaneous leucocytoclastic vasculitis in which amphotericin B might presumably be the aetiological factor.