Whipple’s disease is a rare multisystemic disease caused by the gram-positive bacillus Tropheryma whipplei. The paper characterizes the etiology and pathogenesis of the disease, gives a broad spectrum of its clinical manifestations, and outlines the basic principles of diagnosis and therapy.

The paper considers different risk factors for osteonecrosis (ON) and some aspects of its pathogenesis: impairments in the differentiation of stromal cells, the vascular provision of intraand extravasal genesis, the quality of proper bone tissue due to generalized or local osteoporosis, intravascular coagulation factors contributing to microthrombogenesis. The basic types of ON are identified.

The paper deals with the most common classical autoinflammatory disease familial Mediterra-nean fever (FMF)/periodic disease. This is a monogenic hereditary disease caused by mutations with an autosomal recessive pattern of inheritance. The most common types of mutations are given. Hyperactivation of innate (antigen-specific) immunity is a basic pathogenic mechanism of the disease and IL-1ß is a leading mediator. FMF prominently occurs in certain ethnic groups (Sephardic Jews, Armenians, Turks, and Arabs). In spite of the fact that there may be multiple organ failure, 12-72-hour febrile fever episodes accompanied by the symptoms of peritonitis and/or pleuropericarditis. AA amyloidosis is the most serious complication of FMF. Colchicine therapy is a basic treatment for preventing this complication. In case of colchicine inef-fi-cacy/intolerance, other agents, including genetically engineered biological drugs (IL-1ß inhibitors, etc.), may be used.

Giant cell arteritis (GCA) is a well-known vasculitis sensitive to glucocorticoid (GC) immuno-suppression. However, during long-term treatment there may be many adverse reactions that remain a serious problem so far. Since GCA encompasses a broad spectrum of clinical subtypes, ranging from severe visual loss and neurological deficits to isolated systemic signs, its treatment must be adjusted specially to each case. The literature contains contradicting recommendations for the therapy for GCA. The paper considers different treatment options for GCA, including that with neuro-ophthalmic and neurological complications, as well as the evidence for their possible adjuvant therapies. Although there is no randomized controlled clinical trial in GCA with ocular and neurological complications, the data available in the literature suggest that these patients are recommended to be admitted for high-dose intravenous methylprednisolone, monitoring, and prevention of GC-induced complications. It is expedient to use aspirin in these cases. The evidence supporting the use of methotrexate, as well as genetically engineered agents (GEAs), infliximab, etanercept) as steroid-sparing agents is discussed. Cases of using individual GEAs (adalimumab, tocilizumab and rituximab) as an alternative to GC monotherapy are described. It is concluded that there is a need for extended clinical trials evaluating the most effective and safe GC-sparing drugs.

Up to now, it is difficult to determine systemic scleroderma (SSD) activity because of the lack of validated tools to estimate changes in the pathological process. Attempts have been made to develop unified activity assessing methods for many years. The indices proposed by the European SSD Group are most popular today. This paper gives the results of using this index in a cohort of Russian patients.

In patients with rheumatoid arthritis (RA), the signs of left ventricular diastolic dysfunction and hypertrophy are detectable just at the preclinical stage of myocardial damage. RA activity, rheumatoid factor seropositivity, and systemic manifestations of the disease should be considered to be risk factors of early cardiac involvement.

The paper characterizes the specific features of the anatomy and physiology of the hip joint, the clinical presentation of coxarthrosis and presents current methods for the diagnosis and treatment of the disease. It gives the results of a trial evaluating the impact of long-term (one-year) theraflex therapy on the symptoms of hip osteoarthrosis.

Objective: to study the incidence rate of osteoporotic proximal femoral fractures in old persons from Kemerovo after 6, 12, and 24 months. Subjects and methods. The incidence of femur fractures was analyzed in people of 50 years or older who had been followed up in the Kemerovo units from 1 January 2004 to 31 December 2008. Results. In the examined period, the incidence of femur fractures by referrals for both sexes was 277.75 per 100,000 population 50 years of age and older: 179.59 for males and 335.96 for females. Among the Kemerovo dwellers, the least prevalence of femur fractures was noted in the old age group in 2004: 26.77 and 49.17 per 100,000 for males and females, respectively (p<0.05). The highest incidence of fractures was recorded in 2005 and was 40.59 and 79.64 per 100,000 for males and females, respectively (p<0.05). Femur fractures were found to be more common in women aged 50—79 years than in males of the same age. There were statistically significant group differences for the age groups of 50—54, 55—59, 65—69, 70—74, and 75—79 years (p<0.05). Among the persons aged 80 years or older, femur fractures were more frequently seen in the males than in the females (p>0.05). Conclusion. In the old women living in Kemerovo, the incidence of osteoporotic femur fractures was higher than that in the men within all years of the follow-up. The largest number of fractures was in the age group of 75 years or older in both male and female populations.

Buerger’s disease, or thromboangiitis obliterans, is a severe disabling systemic disease of vessels. The paper describes a case of thromboangiitis obliterans in a patient with three extremities amputated during vascular therapy. The course intravenous administration of the stable prostacyclin analogue iloprost (Ilomedin®) allows the only extremity to be preserved.

The paper considers problems in the treatment of acute and chronic pain in patients with rheumatic diseases and shows the role of inflammation and muscle spasm in the pathogenesis of acute and chronic pain. It gives the results of clinical trials and the clinical use of nimesulide (Nise ®) and tizanidine (Sirdalud ®) in the treatment of acute and chronic pain.

Glucocorticosteroids (GCs) that provide a good and rapid clinical effect are the drug of choice to treat rheumatic polymyalgia (RP). A review of English language publications on the treatment of RP is given. Thirty (13 randomized and 17 observational) studies of 20 and more patients with RP have been analyzed. Particular emphasis is laid on initial therapy with GCs, evaluation of their different daily doses, schemes for their dosage reduction and treatment termination, and on the frequency of recurrences. Studies dealing with the treatment with prednisone, prednisolone, methylprednisolone, and injectable sustained-release GC formulations are considered. The data of clinical trials of glucocorticoid-sparing agents (methotrexate, azathioprine) during early and maintenance therapy are analyzed. The genetically engineered agents (infliximab, etanercept) investigated in clinical trials are considered to be as alternatives; a case of using rituximab is described. The role of nonsteroidal anti-inflammatory drugs in the treatment of RP is also evaluated. An algorithm is proposed for the management of a patient with RP.

Based on the data available in the literature, the author analyzes the association of chronic pain, psychological status, and behavioral reactions, which appear as an unjustified reduction in social activity, in patients with juvenile chronic arthritis. The possible causes of pain behavior in these patients are indicated.

To prevent disease progression in patients with osteoarthosis (OA) remains a challenging problem. Despite the proposed drug, non-drug, and surgical treatments for OA, there is a clinical need for medications that have a structure-modifying effect and are able to delay or prevent cartilage degradation and to alleviate the clinical manifestations of the disease. A 3-year international randomized clinical trial has demonstrated strong evidence for the symptomand structure-modifying effect of strontium ranelate in female and male patients with clinical primary knee OA. The clinical use of the drug opens up new prospects for preventing the progression of the disease in patients with knee and hip OA.

Nonsteroidal anti-inflammatory drugs (NSAIDs) are the most important tool to control pain in rheumatic diseases; however, their application is limited by the risk of serious complications in the cardiovascular system and gastrointestinal tract (GIT). The advent of a Russian new drug that is a naproxen and esomeprazole combination (Vimovo™) extends the possibilities of NSAIDS use. This review considers the benefits of both NEC components. The former is naproxen, a traditional NSAIDS that is in common use as an effective analgesic worldwide. Its chief merit is the least cardiovascular risk among all NSAIDs (aside from aspirin). Esomeprazole is a representative of the group of proton pump inhibitors, a potent antisecretory drug that has passed major tests as an agent for the prevention of NSAID-related GIT complications. This drug combination allows patient incompliance to gastroprotective therapy to be eliminated. Large-scale clinical trials have confirmed a considerable reduction in the frequency of GIT complications with NSAIDS use as compared to the standard enteric-coated naproxen, including in patients receiving low-dose aspirin. Comparison of NEC with celecoxib has indicated that the new medication is as effective as a selective COX-2 inhibitor in both efficacy and GIT safety.

In 1998 the First Congress of the European Ankylosing Spondylitis (AS) Working Group was held and attended by 9 leading experts from Germany and the Netherlands. Much scientific and organizational work has been done for 14 years, causing all spondyloarthritis (SA) studies to be coordinated and discussed by the working group experts thrice a year. A regular SА congress is held at Ghent, Belgium, every 2 years. In 2009, the working group was renamed the Assessment of SpondyloArthritis international Society (ASAS) having about 90 members from more than 30 countries. The results of the society’s activity were new classification criteria for axial (2009) and peripheral (2011) SA, criteria for authentic sacroiliitis and spondylitis according to MRI data; guidelines for AS treatment, those for the use TNF inhibitors in SA, and the Ankylosing Spondylitis Disease Activity Score.