Current Rheumatology Reviews - Volume 9, Issue 1, 2013

The prevalence of psoriasis is 2-3% in European Countries, therefore psoriasis is one of the most frequently occurring inflammatory skin diseases. Psoriasis results from an interaction of genetic factors and environmental conditions such as infections, smoking or intake of certain drugs. Psoriatic arthritis is diagnosed in about 20% of patients with psoriasis. Pustular forms are much more rarely seen and have a genetic background distinct from plaque psoriasis. Psoriasis is associated with a multitude of comorbidities such as rheumatoid arthritis, inflammatory bowel diseases, diabetes mellitus, obesity, hypertension and dysfunctions in lipid metabolism. Quality of life is markedly reduced in psoriasis patients and many of them suffer from depression and anxiety. An interdisciplinary treatment of psoriasis and its comorbidities is therefore essential. Today, adequate therapy according to medical guidelines is able to heal skin lesions and to improve quality of life.

SAPHO and its relative CMRO are uncommon but not rare chronic conditions with unknown etiology. Environmental factors, perhaps related to microorganisms, may be important triggers, but there is no support for a septic nature. The monogenic animal models called cmo and Lupo with autosomal recessive transmission have not been replicated in human diease. Interesting but unconfirmed studies indicate impaired p53 formation, increased IL-10 production and decreased capacity to mount ROS responses in different patients whith SAPHO. There is more evidence supporting an autoinflammatory than an autoimmune pathogenesis of SAPHO. Susceptibility genes on chromosomes 1 and 18 need to be confirmed. More studies in larger numbers of patients are needed to confirm the often anecdotal observations reviewed here. It is hoped that this review may stimulate such work.

The pathophysiology of SAPHO syndrome still remains to be determined. However, like in other forms of spondylarthritides, this rare condition seems to result from the combination of genetic, environmental and immunological factors. Surely, SAPHO syndrome cannot be simply regarded as the adult form of the ‘caricatural’ DIRA (deficiency in interleukin-1 receptor antagonist) syndrome, although this purely genetic disease also causes multiple osteomyelitis and pustular rashes. An initial bacterial trigger, mainly represented by the cutaneous saprophyte Propionibacterium acnes, could take advantage of a selective deficiency of the innate immunity, implicating neutrophils. This could elicit thereafter a ‘hyperimmune’ reaction, as in other chronic inflammatory conditions like reactive arthritis, Crohn’s disease or hidradenitis suppurativa. The reported efficacy of either longterm antibiotic regimens (especially with azithromycin) or immunomodulatory biologic agents targetting TNF-α or IL-1 supports the concept of a post- or para-infectious hyperresponsiveness disorder, with a convincing rationale for ‘hybrid’ therapies.

In the early seventies isolated Sternocostoclavicular Hyperostosis (SCCH) was described as a persistent painful swelling of the sternum, clavicles and upper ribs. A few years later associations with skin lesions were reported and SCCH was suggested as a part of psoriatic spondylarthritis. Then, different terms such as pustulotic arthro-osteitis and others were used. In 1987 a French national survey proposed the term SAPHO syndrome, standing for synovitis, acne, pustulosis, hyperostosis and osteitis. This term has proven to be useful till now to describe these osteoarticular disorders with associated skin lesions, showing a chronic relapsing course, heterogenic clinical features and different etiologies.

The clinical picture of childhood chronic recurrent multifocal osteomyelitis (CRMO) is characterized by an aseptic chronic osteomyelitis, most often affecting the metaphyses of the long bones. Skin inflammation (palmoplantar pustulosis, psoriatic lesions, acne) and inflammatory bowel disease may be associated with CRMO and therefore subsume this disease into the entity of SAPHO syndrome. Deregulated cytokine/chemokine expression in myeloid cells seems to drive chronic inflammation of the disease. NSAIDs may reduce pain, but additional second-line treatments (DMARDs, biologicals) are needed to treat persisting or progressing inflammation in a significant number of patients. The use of bisphosphonates may be a promising option in treating NSAID-refractory osteomyelitis in CRMO.

The peculiar bone involvement, represented by osteitis, is the common denominator of SAPHO syndrome. Hyperostosis and osteitis are chronic inflammatory reactions involving the cortical and trabecular bone respectively; both are characterised by increased sclerosis. Hyperostosis appears radiologically as chronic endosteal and periosteal thickening with narrowing of the medullary canal, but areas of ostelysis may also be present. Conversely, osteitis appears as increased osteosclerosis involving the trabecular infrastructure of cancellous bone. The occurrence of hyperostosis with little or no osteitis is not uncommon. SAPHO syndrome may have a prolonged course with phases of reacutization and remission; the long-term prognosis is usually fairly good, but sometimes a disabling course may occur. Our experience demonstrated that the majority of patients suffering from SAPHO syndrome experienced a chronic course, requiring continous treatment, whilst in a third of the cases the patients reported multiple remission and exacerbations of the disease with flares lasting till to 8 months. Only in a minority of cases the bone inflammation faded and never recurred. Female sex, peripheral arthritis, ACW involvement, the coexistence of more than one cutaneous symptoms, and high inflammatory indices are correlated with a chronic disease course and involvement of new osteoarticular sites.

The interventional studies with bone biopsy of the SAPHO lesions and microbiological investigation are a significant addition to a long range of publications showing an association of SAPHO with Propionibacterium acnes. Infectious agents isolated from SAPHO patients have merited special attention for many years. Their possible etiological role is supported by the pathogen isolation from different sites: anterior chest wall, spine, synovial fluid, bone tissue, and skin pustules. A range of pathogens have been found, including Staphylococcus aureus, Hemophilusparainfluenzae, actinomyces, and even Treponemapallidum. P. acnes is a much more frequent pathogen and plays a particular role. The article focus is to emphasize limited use of interventional method to verify infection ethology of SAPHO in publications last two years. Successful therapy may be really effective and eradicative on a basis of the pathogen identification.Randomized control studies are needed to confirm the effects of antibiotic therapy.

SLE is characterized by overproduction of various types of autoantibodies. Under certain circumstances, antibodies targeting some of the neoepitopes of the complement system can be seen. The most studied among antibodies directed against a component of the complement system is anti-C1q. Anti-C1q antibodies are present in approximately one third of the patients with lupus, who often have high clinical disease activity and in particular renal involvement. In the presence of high titers of anti-C1q antibodies also the levels of C1q and C3 and C4 components of the complement system are also usually low. The presence of the anti-C1q antibodies is not limited or specific just for SLE or lupus nephritis. For the first time, they were described in HUVS (Hypocomplementemic Urticarial Vasculitis Sydrome), later in Felty´s syndrome, rheumatoid vasculitis, hepatitis C, poststreptococcal glomerulonephritis or aging population.

Chloroquine and hydroxychloroquine are 4-aminoquinoline compounds commonly employed as anti-malarial drugs. Chloroquine and its synthetic analogue, hydroxychloroquine also belong to the disease-modifying anti-rheumatic drug class because these drugs are immunosuppressive. The immunosuppressive activity of chloroquine and hydroxychloroquine is likely to account for their capacity to reduce T-cell and B-cell hyperactivity as well as pro-inflammatory cytokine gene expression. This review evaluated experimental and clinical trials results as well as clinical response data relative to the use of chloroquine and/or hydroxychloroquine as first-line medical therapies in systemic lupus erythematosus, rheumatoid arthritis, primary Sjogren’s syndrome, the anti-phospholipid syndrome and in the treatment of sarcoidosis. A primary outcomes measure in these clinical trials was the extent to which chloroquine and/or hydroxychloroquine reduced disease progression or exacerbations and/or the use and dosage of corticosteroids. The relative efficacy of chloroquine and hydroxychloroquine in modifying the clinical course of these autoimmune disorders is balanced against evidence that these drugs induce adverse effects which may reduce their use and effectiveness in the therapy of autoimmune disorders.

Objective: To examine whether vitamin D deficiency is a determinant risk factor of chronic low back pain (LBP) in Moroccan postmenopausal women. Methods: A biochemical assay of serum calcium, phosphate, 25(OH)D, and parathormone (PTH) was performed for 105 patients complaining from a chronic LBP with no obvious causes and compared to those of 45 healthy patients. All participants were postmenopausal. Patients were matched with controls for age and body mass index (BMI). Vitamin D deficiency was defined as a circulating level of 25(OH)D below 20 ng/ml. Results: Vitamin D deficiency was significantly more common in patients suffering from chronic LBP than in controls (79 % vs 61.4 %; P= 0.02). Falls antecedent was also associated with chronic LBP (37.1 % in patients vs 20.5% in controls; P< 0.01). There was no significant association between chronic LBP and age, BMI, smoking status, nor with number of pregnancies. In multiple logistic regression, after adjusting for potential confounders factors potentially influencing chronic LBP (age, BMI, smoking status, number of pregnancies), the main determinants of chronic LBP were vitamin D deficiency [OR 2.5 (95% IC, 1.1-5.8; P = 0.04)] and falls antecedent [OR 3 (95% IC, 1.2-7.2; P = 0.01)]. Conclusion: Our study shows a significant association between vitamin D deficiency and chronic LBP in Moroccan postmenopausal women. Further studies are clearly warranted to determine the effectiveness and the mechanism(s) of this links between vitamin D deficiency and chronic LBP.

Background: A number of children with Juvenile Idiopathic Arthritis (JIA) got admitted in the pediatrics ward of a medical college hospital. However, burden and clinical pattern of JIA have not been studied in our country. Objectives: To estimate the burden of JIA admitted in the pediatric department of medical college hospital of Bangladesh and to observe the status of active JIA in respect to magnitude, clinical profile and hematological changes. Methods: A prospective study was carried out among the patients admitted in pediatric ward of a medical college hospital, Bangladesh from 2008 to 2010. All active JIA patients admitted during this period were included in this study. JIA was diagnosed according to the ILAR criteria. Male/Female ratio, age, Total Leukocyte Count (TLC), Differential count (D/C), Platelet count, Erythrocyte Sedimentation Rate (ESR), C Reactive Protein (CRP), Rheumatoid Factor (RF) of JIA patients were recorded. These were statistically analyzed with the help of SPSS programme using chi-square test. Results: Twenty five cases of active JIA were diagnosed during this period. Magnitude was 3.75 per 1000 admitted cases. Male/Female ratio was 1.08:1. 10 (40%) cases were between the ages of 8 to 11 years. Polyarticular seronegative JIA was found in 10 (40%) cases and oligoarticular JIA in the next order 6 (24%). High ESR, TLC, platelet count was observed in 21 (84%) [p <.001], 19 (76%) [p <.009], 3 (12%) [p <.000] cases respectively. Conclusion: Burden of JIA was similar to all the areas of the world. Polyarticular seronegative JIA was commonly observed in our study. High ESR, increased TLC and Platelet count was the reliable predictor of active JIA.