Didem Arslan Tas

Impact of Helicobacter pylori on the clinical course of recurrent aphthous stomatitis

BACKGROUND Recurrent aphthous stomatitis is one of the most common lesions of oral mucosa. Helicobacter pylori is suggested as one of the etiological agents of recurrent aphthous stomatitis. Here, we conduct a study for evaluating the impact of H. pylori eradication on clinical course of recurrent aphthous stomatitis. METHODS Forty-six patients with minor aphthous lesions were enrolled. The number of RAS lesions at last 6 months and vitamin B(12) levels were recorded. All patients were detected for H. pylori with endoscopic biopsy. H. pylori was positive in 30 patients and negative in 16 patients. H. pylori-positive 30 patients received eradication therapy. Three months after therapy, patients were re-evaluated with urea breath test; 18 patients were negative (eradicated), and the remainders (12 patients) were positive (non-eradicated) for H. pylori. 6 months after eradication, vitamin B(12) levels and number of aphthous lesions at 6 months were recorded. RESULTS Vitamin B(12) levels were significantly increased in H. pylori-eradicated group (P = 0.001), whereas no significant change was found in non-eradicated group (P = 0.638). Mean number of aphthous lesions (per 6 months) of H. pylori-eradicated group was significantly decreased after eradication (P = 0.0001); in the non-eradicated group, no significant change was found (P = 0.677). In Hp-positive group, number of RAS lesions and vitamin B(12) levels were negatively correlated when evaluated both before and after eradication. CONCLUSIONS This study provides evidence to support the beneficial effect of H. pylori eradication in patients with recurrent aphthous stomatitis. The underlying mechanism might be the increase in vitamin B(12) levels after eradication.

Evaluating hand in systemic sclerosis

Articular symptoms are common in SSc and joint pain is a frequent presenting feature of this disease. Hand involvement is often the first clinical manifestation of SSc and could be resulted from fibrosis or synovitis or an overlap syndrome with rheumatoid arthritis (RA); though, the latter is a controversy in practice. To define the clues when identifying the nature of the hand arthropathy in SSc. In order to determine the hand arthropathy, serological tests, hand radiography, finger-to-palm (FTP) distance and other clinical features, disease activity and functional scoring parameters were assessed. Twenty-eight consecutive SSc patients and 43 controls (21 rheumatoid arthritis and 22 healthy controls) were included. Radiographic findings in SSc patients were: Erosions 25%, joint space narrowing 17.9%, arthritis 10.7%, radiological demineralisation 42.9%, acro-osteolysis 25%, flexion contracture 28.6% and calcinosis 17.9%. Anti-CCP antibody and RF positivity were as follows: In SSc group: 3 (11%) and 7 patients (25%); In RA group: 13 (62%) and 19 patients (90.5%); In healthy control group: 1 (4%) and 3 persons (13.6%), respectively. Two patients (7.14%) were regarded as RA overlap, whom both had positive RF and positive anti-CCP results and their radiographs revealed arthritis. Seventeen patients (61%) were regarded as SSc arthropathy; all were negative for RF and anti-CCP but revealed nonarthritic radiological findings. (Among them, only one patient had positive anti-CCP result). The remainder (9 patients) had no radiological or serological finding positive for arthropathy. Arthritis was found to have correlation with heart involvement and FTP was correlated with lung involvement. Hand involvement in SSc is a challenge in rheumatology practice; Radiographic testing when evaluated with RF and anti-CCP will be a helpful tool to discriminate SSc arthropathy from RA-SSc overlap. Hand arthropathy should increase the interest in the serious internal organ involvements of SSc.

Different clinical presentation of the hyperimmunoglobulin D syndrome (HIDS) (four cases from Turkey)

Hyperimmunoglobulin D syndrome (HIDS) is one of the autoinflammatory syndromes which are characterized by febrile attacks. Duration and frequency of the febrile attacks, as well as typical organ involvements vary greatly. Recently, it is possible to reach more reliable data by the possibilities that are opened up by molecular genetics in order to highlight the aetiopathogenesis of this group of diseases. Typical patients with HIDS have an onset of disease in the first year of life. Here, we report four Turkish HIDS cases; three of whom, the symptoms started at a later age. The diagnoses were made by relevant clinical symptoms along with MVK mutations detected by DNA sequencing method. As summarised in this article, HIDS could be presented with a broad spectrum of symptoms. Although most of the HIDS patients are reported from Europe and especially Dutch ancestry, case reports are presented from all over the world. For this reason, HIDS should be kept in mind for the differential diagnosis of periodic fever syndromes or before accepting an FMF patient as colchichine resistant. We suppose that the phenomenon of “later-onset HIDS” should shed light into unresolved clinical problems of patients with periodic fever. Especially in countries that FMF is more frequent such as Turkey, even though the symptoms start later than classic cases, HIDS should be kept in mind for differential diagnosis of periodic fever syndromes.

Periodic fever and hyperimmunoglobulin D syndrome in a boy with pediatric autoimmune neuropsychiatric disorders associated with group A β-Hemolytic streptococcus.

The importance of the immune system in pediatric psychiatric disorders has been known since the 1990s. Swedo et al. (1998) have reported that obsessive-compulsive disorder (OCD), tics, and other neuropsychiatric symptoms, such as separation anxiety, irritability, hyperactivity, and attention and concentration deficits, are usually triggered by infections, and have reported a phenomenon known as pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS). The diagnostic criteria for PANDAS are as follows: 1) Presence of a tic disorder or OCD; 2) onset by puberty (usually at 3–12 years of age); 3) abrupt symptom onset or episodic course of symptom severity; 4) temporal association between symptom exacerbation and streptococcal infections; and 5) presence of neurologic abnormalities during periods of symptom exacerbation (Swedo et al. 1998). To the best of our knowledge, there is no reported case or controlled study describing the psychiatric signs or symptoms accompanying hyperimmunoglobulin D syndrome (HIDS). HIDS is among the periodic fever syndromes that are genetically inherited and share some common features, including recurrent fever, inflammation of serosal membranes, musculoskeletal involvement, skin rashes, and amyloidosis. HIDS was originally described in patients of Dutch ancestry by van der Meer et al. (1984). HIDS is characterized by sustained high fever, lymphadenopathy, abdominal pain, arthritis, and skin rashes; episode duration is from 4 to 8 weeks. HIDS is caused by mutations in the gene that encodes mevalonate kinase (MVK), an enzyme involved in the isoprenoid and cholesterol biosynthesis pathway. The four most prevalent mutations of MVK (V377I, I268T, H20P/N, and P167L) account for 71.5% of the known mutations (van der Hilst et al. 2008; Steichen et al. 2009). The differential diagnosis of HIDS is broad and includes familial mediterranean fever (FMF), tumor necrosis factor receptor associated periodic syndrome (TRAPS), periodic fever adenitis pharyngitis aphthous ulcer (PFAPA), adult-onset Still’s disease, juvenile idiopathic arthritis, rheumatic fever, and Behçet’s disease (Long 2005; Steichen et.al. 2009). Although MVK gene mutations have been suggested to be the genetic defect responsible for the etiopathogenesis of HIDS, they were not observed in a substantial proportion of those with the disease; therefore, the pathophysiology of the disease remains unclear. More than 66% of HIDS patients present to physicians within the first year of life. An earlier study of ours suggested later onset of the HIDS (Tas et al. 2012). Episodic attacks of fever (lasting 3–7 day) are generally accompanied by chills, cervical lymphadenopathy, abdominal pain, and vomiting or diarrhea. Patients may also present with headache, arthralgia or arthritis, aphthous ulceration, rash, and splenomegaly (van Der Hilst et al. 2008). Attacks may be precipitated by vaccination, viral infection, trauma, and stress (Drenth et al.1994). Laboratory test results generally show the presence of characteristic abnormalities such as an Immunoglobulin D (IgD) level >100 kU/L, and some patients also have an elevated immunoglobulin A level. We report a case with concurrent HIDS and OCD comorbid with attention-deficit/hyperactivity disorder (ADHD) combined type, speech disorder (stuttering), and Tourette’s disorder (TD).

Evaluating Choroidal Characteristics in Systemic Sclerosis Using Enhanced Depth Imaging Optical Coherence Tomography

ABSTRACT Purpose: To evaluate choroidal thickness (CT) and investigate the involvement of choroidal circulation in patients with systemic sclerosis (SSc). Methods: A total of 120 eyes of 60 SSc patients and 60 eyes of 30 healthy controls were evaluated. CT measurements were performed at the subfoveal region, 1000 µm nasal and 1000 µm temporal to the fovea, using enhanced depth imaging optical coherence tomography. CT measurements were compared between the patients and controls. Results: The average subfoveal, parafoveal nasal and parafoveal temporal CT were significantly thinner in SSc patients when compared with healthy controls (297.77 ± 60.8 µm vs 339.8 ± 50.4 µm; 267.32 ± 51.1 µm vs 308.65 ± 49.9 µm; 270.63 ± 46.3 µm vs 309.22 ± 42.4 µm, respectively; p<0.001 for all). Conclusions: Reduced CT in SSc patients supports the hypothesis of widespread vascular injury, including the ocular microcirculation.

Vitamin D insufficiency in a boy with obsessive-compulsive disorder

Vitamin D deficiency not only causes low bone mass but also may lead to neuropsychiatric disorders. In the present case, vitamin D supplementation reduced obsessive–compulsive disorder (OCD) symptoms associated with streptococcal infection in a 7‐year‐old boy. Sudden onset of symptoms, including excessive hand washing and fear of touching anything, had occurred 1 month before presentation. Although there are few studies on a possible causal relationship between vitamin D and neuropsychiatric disorders, the present report; together with previous data, suggest an etiological role of vitamin D‐related immune processes.

Sacroiliac joint involvement in systemic sclerosis

One of the major problems for systemic sclerosis (SSc) patients is suggested to be articular involvement. Mostly involved joints in SSc were reported as wrist, carpometacarpal‐interphalangeal, foot, knee, hip and shoulder; however, there has been little knowledge on the sacroiliac joint. Our aim was to evaluate sacroiliac joint involvement in SSc.

Diffuse soft tissue and vascular calcification in systemic lupus erythematosus with chronic kidney disease

The systemic disorder of mineral and bone metabolism which is related to chronic kidney disease (CKD) is called mineral and bone disorder (MBD). Calcifications related to CKD-MBD may occur in ophthalmic tissue, arterial walls, subcutaneous and periarticular soft tissues and organs. The vascular calcifications are the most important causes of mortality and morbidity in CKD. Here, we present a case of systemic lupus erythematosus with early and disseminated calcifications of vascular and periarticular soft tissues related to CKD-MBD.ZusammenfassungDie bei chronischer Nierenerkrankung (CKD, „chronic kidney disease“) auftretende systemische Störung des Mineral- und Knochenstoffwechsels wird als MBD („mineral and bone disorder“) bezeichnet. Verkalkungen im Zusammenhang mit CKD-MBD können in okulären Geweben, arteriellen Gefäßwänden, subkutanen und periartikulären Weichgeweben sowie in Organen entstehen. Die Gefäßverkalkungen sind die Hauptgründe für Morbidität und Mortalität bei CKD. Im Beitrag vorgestellt wird ein an systemischem Lupus erythematosus erkrankter Patient mit früher, disseminierter, CKD-MBD-bezogener Kalzifikation von Gefäßen und periartikulärem Weichgewebe.

Mevalonate kinase gene mutations and their clinical correlations in Behçet's disease

Genetics is suggested to play a role in the development of Behçets disease (BD). Shared phenotipic features requires an approach to differential diagnosis from periodic febrile syndromes. We planned to study for mevalonate kinase (MVK) as a candidate for a susceptibility gene for Behçets disease.

Charcot’s neuroarthropathy of the hand in a patient with diabetes mellitus

Charcot’s neuroarthropathy (CNA) is a destructive disease associated with a reduced proprioceptive sensation. Diabetes mellitus (DM) is one of the most common etiological factor for CNA which typically affects ankles and small joints of feet. Neuroarthropathy seen in upper extremity in patients with DM is a rare clinical condition. In this report, we presented a case of CNA with hand involvement who had type-II DM. Neuroarthropathy was rapidly progressed and destructive changes were observed in right hand joints after a minor trauma in current case. Charcot’s neuroarthropathy may rarely occur in the joints of hand in patients with DM. Early diagnosis of CNA is important in order to protect joints and avoid further disability.