Senol Kobak

Sarcoidosis: a rheumatologist’s perspective

Sarcoidosis is a systemic disorder of unknown etiology, which may involve various tissues and organs and is characterized by a noncaseating granuloma reaction. While pathogenesis is not yet clear, cellular immune system activation and nonspecific inflammatory response occur secondarily to several genetic and environmental factors. T helper 1-cells and macrophage-derived pro-inflammatory cytokines stimulate the inflammatory cascade and formation of granuloma occurs as a result of tissue permeability, cell influx, and local cell proliferation. The different prevalence, clinical results, and disease course observed in different races and ethnic groups, is an indicator of the heterogeneous nature of the disease. Sarcoidosis may mimic and/or may occur concomitantly with numerous primary rheumatic diseases. This disease most commonly presents with bilateral hilar lymphadenopathy, pulmonary infiltrations, and skin and eye lesions. Locomotor system involvement is observed at a range of 15% and 25%. Two major joint involvements have been described: acute and chronic form. The most common form, the acute form, may be the first sign of sarcoidosis and present with arthralgia, arthritis, or periarthritis. Chronic sarcoid arthritis is usually associated with pulmonary parenchymal disease or other organ involvement and occurs rarely. While asymptomatic muscular involvement is reported between 25% and 75%, symptomatic muscular involvement is very rare. Symptomatic myopathy may present as three different types: chronic myopathy, palpable nodular myositis, or acute myositis. Even if rare, 2–5% of cases may exhibit osseous involvement and it is frequently associated with lupus pernio, chronic uveitis, and multisystemic disease. Sarcoidosis was reported together with different rheumatologic diseases. There are studies showing that sarcoidosis may mimic the clinical and laboratory findings of these disorders. Nonsteroidal anti-inflammatory drugs and corticosteroids are used for treating the symptoms of rheumatologic findings. In patients who are unresponsive to corticosteroids, immunosuppressive and anti-tumor necrosis factor alpha drugs may be used. In this review, the incidence of rheumatologic symptoms, the clinical findings, and the treatment of rheumatologic manifestations of sarcoidosis are discussed.

Pulmonary Arterial Hypertension in Patients with Primary Sjögren's Syndrome

Introduction. Primary Sjögrens syndrome (pSS) is an autoimmune epithelitis. Pulmonary arterial hypertension (PAH) is an important and severe complication, which is encountered in many collagen tissue disorders. Early diagnostic strategies are required to define it at the asymptomatic stage. Doppler echocardiography is an important, noninvasive screening test for PAH diagnosis. Objective. The aim of this present study is to define the frequency of PAH in patients with pSS and to reveal correlations with laboratory and clinical findings. Material and Methods. A total of 47 patients, who were diagnosed with pSS according to American-European Study Group criteria were enrolled in the study. After all patients were evaluated clinically and by laboratory tests, Doppler echocardiography was performed in the cardiology outpatient clinic. Systolic pulmonary artery pressure (SPAP) >30 mm Hg values, which were measured at the resting state, were accepted as significant for PAH. Results. Forty-seven patients with pSS were included in the study. The mean age of patients was 48 years and the mean disease duration was 5.3 years. PAH was defined in 11 of the 47 patients (23.4%). The SPAP value was over 35 mm Hg in 5 out of 11 patients, whereas six patients had SPAP measuring 30–35 mm Hg. While pulmonary hypertension was related with earlier age and shorter duration of disease (P = 0.04), there was no statistically significant correlation between SPAP increase and clinical findings (P > 0.05). Conclusion. We have defined high PAH frequency in patients with pSS. Since there are different data in the literature, it is obvious that large scale, multicentre studies are required.

Retinopathy due to antimalarial drugs in patients with connective tissue diseases: are they so innocent? A single center retrospective study.

Introduction:  Antimalarial medications are basal active drugs used for the treatment of various rheumatological conditions. Their common side‐effects include eye damage.

Efficacy and safety of adalimumab in a patient with ankylosing spondylitis on peritoneal dialysis

The most commonly used treatments in patients with ankylosing spondylitis include nonsteroidal anti-inflammatory and disease modifying anti-rheumatic drugs (DMARDs), but most of these have nephrotoxic effects. In patients who undergo chronic hemodialysis, DMARDs are not widely preferred due to the chance of increased adverse effect incidence and the risk on patient survival, in addition to already present immunosuppression. The efficacy and safety of anti-TNF alpha drugs for the treatment of renal dysfunction that develops associated to secondary amyloidosis in inflammatory rheumatic diseases have been reported in various studies. In this report, the efficacy and safety of adalimumab was shown in patients with active ankylosing spondylitis who undergo peritoneal dialysis because of chronic renal failure.

The prevalence of sacroiliitis and spondyloarthritis in patients with sarcoidosis.

Introduction. Sarcoidosis is a chronic granulomatous disease, which can involve different organs and systems. Coexistence of sarcoidosis and spondyloarthritis has been reported in numerous case reports. Purpose. To determine the prevalence of sacroiliitis and spondyloarthritis in patients previously diagnosed with sarcoidosis and to investigate any possible relation with clinical findings. Materials and Methods. Forty-two patients with sarcoidosis were enrolled in the study. Any signs and symptoms in regard to spondyloarthritis (i.e., existence of inflammatory back pain, gluteal pain, uveitis, enthesitis, dactylitis, inflammatory bowel disease, and psoriasis) were questioned in detail and biochemical tests were evaluated. Sacroiliac joint imaging and lateral heel imaging were performed in all patients. Results. Sacroiliitis was found in 6 of the 42 (14.3%) sarcoidosis patients and all of these patients were female. Common features of the disease in these six patients were inflammatory back pain as the major clinical complaint, stage 2 sacroiliitis as revealed by radiological staging, and the negativity of HLA B-27 test. These six patients with sacroiliitis were diagnosed with spondyloarthritis according to the criteria of ASAS and of ESSG. Conclusion. We found spondyloarthritis in patients with sarcoidosis at a higher percentage rate than in the general population (1–1.9%). Controlled trials involving large series of patients are required for the confirmation of the data.

Fecal calprotectin is associated with disease activity in patients with ankylosing spondylitis

Calprotectin is one of the major antimicrobial S100 leucocyte proteins. Serum calprotectin levels are associated with certain inflammatory diseases such as rheumatoid arthritis, systemic lupus erythematosus and inflammatory bowel disease. The aim of this study was to investigate serum and fecal calprotectin levels in patients with ankylosing spondylitis (AS) and show their potential relations to the clinical findings of the disease. Fifty-one patients fulfilling the New York criteria of AS and 43 healthy age- and gender-matched volunteers were included in the study. Physical and locomotor system examinations were performed and history data were obtained for all patients. Disease activity parameters were assessed together with anthropometric parameters. Routine laboratory examinations and genetic testing (HLA-B27) were performed. Serum calprotectin levels and fecal calprotectin levels were measured by an enzyme-linked immunosorbent assay. The mean age of the patients was 41.5 years, the mean duration of the disease was 8.6 years, and the delay in diagnosis was 4.2 years. Serum calprotectin levels were similar in both AS patients and in the control group (p=0.233). Serum calprotectin level was correlated with Bath AS disease activity index (BASDAI) and Bath AS functional index (BASFI) (p=0.001, p=0.002, respectively). A higher level of fecal calprotectin was detected in AS patients when compared with the control group. A statistically significant correlation between fecal calprotectin level and BASDAI, BASFI, C-reactive protein and Erythrocyte sedimentation rate were detected (p=0.002, p=0.005, p=0.001, p=0.002, respectively). The results indicated that fecal calprotectin levels were associated with AS disease findings and activity parameters. Calprotectin is a vital disease activity biomarker for AS and may have an important role in the pathogenesis of the disease. Multi-centered prospective studies are needed in order to provide further insight.

Coexistence of Sarcoidosis and Systemic Sclerosis

Sarcoidosis is a multisystem granulomatous disease characterized by hilar lymphadenopathy, involvement of internal organs, and diverse skin lesions. Systemic sclerosis is an autoimmune disease characterized by skin hardening and different internal organ fibrosis, including vascular abnormality. Immune response associated with Th-2 has been shown in the early and active stage of the disease. In this paper, we report coexistence of systemic sclerosis with sarcoidosis in a female patient presenting with granulomatous dermatitis, interstitial lung disease, and Raynauds phenomenon complaints.

The Prevalence of Antinuclear Antibodies in Patients with Sarcoidosis

Introduction. Sarcoidosis, which is a chronic inflammatory granulomatous disease, can mimic different rheumatologic diseases including connective tissue diseases. Antinuclear antibodies are the markers used for connective tissue diseases. Aim. To determine antinuclear antibody frequency and any possible correlation with clinical and laboratory data in sarcoidosis patients. Material and Method. Forty-two sarcoidosis patients, 45 rheumatoid arthritis patients, and 45 healthy volunteers who were followed up in rheumatology outpatient clinic were included in this study. Demographic, clinical, serological, and radiological data of all patients were recorded. Antinuclear antibodies were determined with indirect immunofluorescent method and 1/100 titration was accepted as positive. The cases that were ANA positive were evaluated with immunoblot method. Results. Average age of the 42 patients (10 males) with sarcoidosis was 45.2 (20–70 years), and average disease duration was 3.5 years. ANA positivity was detected in 12 (28.5%) patients with sarcoidosis (1/100 in 10 patients, 1/320 in two patients), in 19 of RA patients (42.2%), and in two of healthy volunteers in low titer (P < 0.001). In the subgroup analysis made by immunblot test, one patient had anticentromere antibody, one had anti-Ro antibody, one had anti-Scl-70 antibody, one had anti-dsDNA antibody, and eight patients were negative. The two patients who had anticentromere and anti-Scl-70 antibodies had also Sjögrens syndrome and scleroderma diagnosis, respectively. Discussion. The prevalence of ANA in patients with sarcoidosis was found to be significantly higher than healthy control group and lower than RA patients. This result shows that ANA may have an important role in the pathogenesis of sarcoidosis and also could be important in revealing the overlap syndromes of sarcoidosis-connective tissue diseases. Further studies with larger series are necessary in this subject.

Intraarticular adalimumab in a patient with pigmented villonodular synovitis.

Pigmented villonodular synovitis is a chronic inflammatory disease progressing with histological villonodular hyperplasia in synovium, fibrosis and accumulation of hemosiderin. Knee joint is frequently involved leading to severe joint damage and disability in untreated cases. Treatment options include surgical intervention or radio-synovectomy with intraarticular yttrium-90. The case presented here includes significant clinical and radiological disease regression after intraarticular adalimumab in a patient diagnosed with radiologically and histologically confirmed pigmented villonodular synovitis who did not consent to surgical intervention.

An autumn tale: geriatric rheumatoid arthritis

Rheumatoid arthritis (RA) is a chronic inflammatory disease characterized by erosive arthritis and systemic organ involvement. The disease may affect all ages and both sexes; usually it is seen in young women aged 25–45. Recent studies have shown that RA is among the most common inflammatory disease in older age groups. While elderly-onset rheumatoid arthritis (EORA) is still discussed in the literature, it is generally accepted as a disease beginning after 65 years of age. Compared with young-onset rheumatoid arthritis (YORA), it was found that EORA had different characteristics. EORA is characterized by more equal gender distribution, higher frequency of acute onset with constitutional symptoms, more frequent involvement of large joints, and lower frequency of rheumatoid factor (RF) positivity. Earlier diagnosis, less erosive disease and less disease-modifying antirheumatic drug usage were reported as distinguishing EORA from YORA patients. These various clinical presentations may cause difficulties in diagnosis and differential diagnosis of EORA. However, different clinical and treatment approaches may be needed in these patients. In this article, the clinical and laboratory characteristics, prognosis and treatment principles of EORA will be discussed in light of recent literature data.