Songul Cildag

Hajdu-Cheney syndrome with ventricular septal defect.

Hajdu-Cheney syndrome (HCS) is a rare osteolysis syndrome characterized by generalized, progressive osteoporosis and acroosteolysis [1]. Cardiovascular abnormalities, including patent ductus arteriosus or atrial or ventricular septal defect (VSD) might be rarely accompanied by HCS as case presentations [2]. We present here a case of HCS with cardiovascular defect that has not been reported previously in the literature. A 31-year-old man presented to our clinic with a nonhealing wound on the plantar surface of his left foot. He had a history of right below-knee amputation, surgical closure of VSD, and spontaneous autoamputation of the right metatarsophalangeal joint and the first, third, and fourth distal phalanges of the left foot. His general condition was stable with blood pressure of 120/80 mmHg, pulse

Rituximab therapy in rheumatoid arthritis and primary biliary cholangitis

The prevalence of rheumatoid arthritis (RA) has been reported to be 1.8% in patients with primary biliary cholangitis (PBC), and mitochondrial antibodies (AMA) have been found in 10% of RA patients.1,2 It was reported that T and B cells play an important role in the inflammatory process of both RA and PBC.3,4 The potential therapeutic target is determined by the pathogenetic mechanism. We report a case series of patients with RA and PBC with good clinical response to rituximab. The first case was a 61-year-old female with the diagnosis of RA and PBC. The serological tests were negative, except for rheumatoid factor (RF), anti-cyclic citrullinated peptide antibodies (anti-CCP) and AMA-M2. Serum lipids, glucose, renal function tests, parathyroid-thyroid hormones, viral markers and protein electrophoresis pattern were normal. Abdominal ultrasonography showed no pathology. She had a medical history of smoking and receiving ursodeoxycholic acid (15 mg/kg/day), methotrexate (MTX, 15 mg/week), hydroxychloroquine (200 mg/day) and methylprednisolone (4–8 mg/day). Due to high disease activity (DAS-28: 6.24) rituximab (1000 mg IV every 2 weeks) was administered. The second case was a 68-year-old female with the diagnosis of RA and PBC. She had medical history of using ursodeoxycholic acid (10 mg/kg/day), leflunomide (20 mg/day), hydroxychloroquine (200 mg/day) and prednisone (10 mg/day). She had a RF of 34.2 U/mL (3–18 IU/mL) and anti-CCP of 8.7 U/mL (0–4.99 U/mL). AMA-M2 was positive and viral serology was negative. Abdominal ultrasonography showed no pathology. Due to exacerbation of

ABO blood groups and rheumatic diseases

Objective Various genetic and environmental risk factors have been shown to be associated with the incidence of rheumatic diseases. However, the pathogenesis of rheumatic diseases poorly understood. Several studies have shown associations of ABO blood groups with various diseases. Our study aimed to determine whether there is an association between the types of rheumatic diseases and ABO and Rh blood groups. Material and Methods The study included the patients, followed up at the Immunology-Rheumatology clinic between January 2016 and December 2016 for diagnosis of rheumatic disease, who had an ABO Rh blood data. Age, gender, type of rheumatic disease, ABO Rh blood groups were recorded. Results When 823 patients were assessed for blood types, 42.5% patients had A type, 33.2% had O type, 15.4% had B type, and 8.9% had AB type. There was significant difference in the distribution of blood types in rheumatic diseases. While SpA, vasculitis, UCTD, Behçets and RA were more common in the patients with A blood type; FMF, SLE, SSc and SjS were more common in the patients with O blood type. In addition, the blood type where all the diseases are observed the least commonly was AB. There was significant difference in the distribution of Rh factor in rheumatic diseases. 92.2% patients were Rh positive and 7.8% patients were Rh negative. Conclusion In our study, we thought that the higher incidence of different rheumatic diseases in different blood types was associated with different genetic predisposition.

Tst, Quantiferon-tb Gold test and t-spot.Tb test for detecting latent tuberculosis infection in patients with rheumatic disease prior to anti-tnf therapy

Introduction Influenza subtypes vary by clinical, radiological, and prognostic courses and may go along with viral pneumonia. We aimed to identify clinical, radiological, and prognostic aspects of influenza epidemic during years 2016-2017. Materials and Methods Influenza cases reported to the Public Health Directorate in our city was assessed retrospectively. Clinical, radiological, and prognostic parameters were compared based on influenza subtypes. Result We analyzed samples from 197 cases with suspected influenza. Mean age of the subjects was 51.17 ± 26.74. We found influenza A/H1N1, influenza A/H3N2, and influenza B in 59 (30.0%), 29 (14.7%), and 3 (1.5%) cases, respectively. Comorbidity was present in 48 (24.4%) cases. Most common radiological finding was interstitial pattern. Seventy-one and 79 per cent of H1N1 and H3N2 cases were influenza pneumonia, respectively. The prevalence of overall mortality was 5.5% with a predominance in H1N1 over H3N2. Influenza vaccination had been performed in 6.8% and 3.4% of H1N1 and H3N2 cases, respectively. We detected no mortality in any vaccinated patient. We identified 6 pregnant women, 2 of which ended up with preterm birth, and another one with abortion. Conclusions Often manifested as lower respiratory tract infection, influenza may cause epidemics with increased mortality rate. Influenza should be suspected when interstitial pattern was seen on radiological images. H1N1 cases course worse. Since the prognosis is better in vaccinated patients, seasonal influenza vaccination among the community needs to be elevated. In addition, protective measures like vaccination should be taken in pregnancy to avoid preterm delivery or abortion.

Pustulotic Arthro-Osteitis (Sonozaki Syndrome): A Case Report and Review of Literature

Pustulotic arthro-osteitis (PAO) is a rare chronic inflammatory disease, which has now been classified as a seronegative spondyloarthritis. The sternoclavicular and sternocostal joints, pelvis, vertebra, hip, and long bones are affected. Skin findings of the disease are accepted as a variant of pustular psoriasis, but some authors have suggested that palmoplantar pustulosis (PPP) is a different entity. The synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome should be considered in the differential diagnosis. PAO differs from SAPHO by the absence of hyperostosis and the difference in skin manifestations. Here, we aimed to present a 34-year-old female patient with a diagnosis of PAO with typical skin findings and joint involvement.

Comparison of clinical and laboratory findings in patients with systemic lupus erythematosus with regard to age at onset

Objective Systemic lupus erythematosus (SLE) rarely has a late onset. Late-onset SLE (LSLE) has a milder course and less organ involvement. The purpose of the present study was to compare the clinical and laboratory (lab) findings of SLE regarding age at onset. Materials and Methods Seventy-two patients with SLE were included in the study. The age at onset was considered adult-onset SLE (ASLE) if it was <50 years and LSLE if it was ≥50 years. Lab parameters and clinical findings were compared accordingly. Results Overall, 41 (56.9%) patients had ASLE, and 31 (43.05%) patients had LSLE based on the age at onset. The ratio of female-to-male patients was higher in ASLE, and no significant difference was found with regard to gender distribution (12.6:1 and 5.2:1 for ASLE and LSLE, respectively; p=0.239). While malar rash and fever were more common in ASLE, no difference was found regarding the other clinical findings. Only IgG anti-cardiolipin was more common in LSE between the lab parameters. Conclusion Although it is known that LSLE has a milder course and less organ involvement, there are differences in clinical and lab findings and organ involvement in various studies. The results of our study showed no significant difference in organ involvement between ASLE and LSLE.

Rhupus syndrome and Chiari's network

A 69-year-old female patient was admitted to our clinic with photosensitivity, symmetric erosive polyarthritis, and cutaneous vasculitis of lower extremities. Rhupus syndrome was diagnosed, and Chiaris network in the right atrium and interatrial septum patent foramen ovale was achieved on transthoracic and transesophageal echocardiography. If it is thought that increased prevalence of antiphospholipid antibodies in patients with rhupus, this congenital remnant is important for the thrombosis risk, cardiac event, and stroke. The association of both diseases may lead to more serious events and cause worse prognosis. Here, our aim is to present a 69-year-old female patient with rhupus syndrome presenting with cutaneous vasculitis and Chiaris network in the right atrium.

The effect of omalizumab treatment on IgE and other immunoglobulin levels in patients with chronic spontaneous urticaria and its association with treatment response

Introduction Suppression of free immunoglobulin E (IgE) levels and an increase in total IgE levels are observed during omalizumab treatment. However, whether omalizumab has any effect on other immunoglobulins is unknown. Aim To investigate the effect of omalizumab treatment on serum IgE and other immunoglobulins, and demonstrate any association with response to treatment in patients with chronic spontaneous urticaria (CSU). Material and methods The study included 41 patients diagnosed with CSU. Baseline and post-12-week-treatment total IgE, IgA, IgM, and IgG levels and blood eosinophil, neutrophil, lymphocyte and platelet levels were compared. Patients were grouped based on weekly urticaria activity score (UAS-7) responses and these parameters were compared. Results There was a significant increase in baseline and post-12-week-treatment total IgE levels, while there was no significant difference in other immunoglobulin levels. A significant reduction was found in neutrophil counts after the treatment, whereas there was no significant difference in eosinophil, lymphocyte and platelet levels. There was no difference in these parameters between groups with complete response and without complete response. Conclusions Omalizumab treatment can also be used in patients with immunoglobulin deficiency. Due to the observed reduction in neutrophil counts after the treatment, patients must be closely followed for whole blood parameters.

Can Allergen Specific Immunotherapy Induce Familial Mediterranean Fever Attacks

Meandros Med Dent J Allergen-specific immunotherapy is a treatment aimed to reduce allergic symptoms and need for medication use after exposure to specific allergens. Allergen-specific immunotherapy is the only curative treatment that can change the natural course of allergic diseases. Although, allergen-specific immunotherapy is quite effective in the treatment of atopic dermatitis, allergic asthma, allergic rhinitis, and insect bites, serious treatment-related side effects may still occur. Since allergen-specific immunotherapy can cause changes in the immune system, it is theoretically possible that it can also affect immune system mediated inflammatory diseases such as autoimmune diseases. This is the first case report of an occurrence of Familial Mediterranean fever attacks during allergen-specific immunotherapy.

Angiographic assessment of atherosclerotic load at the lower extremity in patients with diabetic foot and charcot neuro-arthropathy

Background: The aim of this study was to investigate atherosclerotic load at the lower extremity in patients with diabetic foot and charcot neuro‐arthropathy and compare them with patients with diabetic foot without charcot neuro‐arthropathy. Methods: This retrospective study consists of 78 patients with diabetic foot who had lower extremity angiography with antegrade approach. All patients were classified into two groups; neuro ischemic wounds with charcot neuro‐arthropathy (30/78) and without charcot neuro‐arthropathy (48/78).Atherosclerotic load at the side of diabetic foot was determined by using the Bollinger angiogram scoring method. Comparison of atherosclerotic load between the two groups was performed. Results: The mean of total and infrapopliteal level angiogram scoring of all patients was 33.3 (standard deviation, sd:±17.2) and 29.3 (sd:±15.6), respectively. The mean of total and infrapopliteal level angiogram scoring of neuroischemic wounds with charcot neuro‐arthropathy group was 18.1 (sd:±11.6) and 15.7 (sd:±10.4), respectively. The mean of total and infrapopliteal level angiogram scoring of neuroischemic wounds without charcot neuro‐arthropathy group was 42.8 (sd:±12.7) and 37.7 (sd:±12.0), respectively. There was a statistically significant difference between the two groups of mean total and infrapopliteal angiogram scoring (p < 0.01). Conclusion: This angiographic study confirms that the atherosclerotic load in patients with diabetic foot and chronic charcot neuro‐arthropathy is significantly less than in patients with neuroischemic diabetic foot wounds without chronic charcot neuro‐arthropathy.