Alopecia universalis in a patient with rheumatoid arthritis developed during treatment with adalimumab.

Alopecia universalis is an advanced form of alopecia areata, characterized by complete loss of hair on all hair-bearing areas. It is currently believed to be an autoimmune disorder. The development of alopecia during treatment with tumor necrosis factor alpha (TNF-α) inhibitors is a rarely observed adverse event. This case describes a 56-year-old female patient with rheumatoid arthritis (RA), who developed alopecia universalis during treatment with adalimumab. There was no improvement in alopecia despite withdrawal of adalimumab. It is believed that interferon-α is the main cytokine involved in promoting inflammation in hair loss after blocking TNF-α by TNF inhibitors. Risk/benefit ratio of maintenance and discontinuation of the drug should be weighed up when alopecia appears during treatment with TNF inhibitors.

Disease activity, obesity, functional disability, and depression in patients with rheumatoid arthritis : Impact on lipid status, glycoregulation, and risk for coronary heart disease.

OBJECTIVES: This study aims to estimate the impact of disease activity, obesity, functional disability, and depression on lipid status, glycoregulation, and risk for coronary heart disease (CHD) in patients with rheumatoid arthritis (RA). PATIENTS AND METHODS: A total of 36 patients with RA (30 women and 6 men, mean age 54.9 years, mean disease duration 7.9 years) were included in this study. We estimated the impact of age, body mass index, disease activity [assessed by DAS28 index and C-reactive protein (CRP) value], functional ability (estimated using the HAQ disability index), and depression [assessed using the Beck Depression Inventory (BDI)] on glycoregulation, lipid status, and risk for CHD in our patients. Glycoregulation was assessed by measuring insulin resistance, insulin, and glucose in blood. Lipids tested in blood included total cholesterol, HDL and LDL cholesterol, and triglycerides (TG). The 10-year risk for CHD was estimated using the Framingham risk score. RESULTS: Of 36 patients, 11 (30.6 %) fulfilled the criteria for metabolic syndrome (MS). Ten of 11 patients (90.1 %) with MS have a 10-year risk for CHD greater than 10 % compared to only 3 of 25 patients (12 %) without MS (p = 0.0001). Patients with high disease activity had lower HDL values than patients with mild or moderate disease activity (1.4 vs. 1.7 mmol/l, p = 0.04). Significant correlations were observed between CRP level and insulinemia (ρ = 0.57, p = 0.003), as well as CRP level and the HOMA index (ρ = 0.59, p = 0.002). The body mass index (BMI) correlated significantly with total cholesterol (r = 0.46, p = 0.02), LDL (ρ = 0.41, p = 0.04), and TG (ρ = 0.65, p < 0.001) in blood. The HAQ-DI did not correlate either with parameters of glycoregulation or lipid status. There was a significant positive correlation between BDI and BMI (ρ = 0.60, p < 0.001). CONCULSION: Active RA is independently associated with decreased HDL cholesterol and increased insulin resistance. Obesity was found to be an independent risk factor for increased total cholesterol, LDL cholesterol, and TG. Depressed patients with RA tend to be overweight or obese and, therefore, have an unfavorable lipid profile.

Sacral perineural cyst mimicking inflammatory low back pain.

This case describes a 46-year-old woman with local pelvic and perineal pain, persisting for 2 years at presentation. The pain worsened during the night and morning and was alleviated during daily activities. Low back pain was associated with morning stiffness lasting longer than 2 h. Sometimes, she felt pain and numbness along her left S1 dermatome, without overt bladder or bowel incontinence. Lasegue's sign was negative. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were elevated (35 mm/h and 9.4, respectively) and Mennel's sign was present on both sides, indicating possible inflammation of the sacroiliac joints. However, radiographs of the lumbosacral spine and sacroiliac joints were normal. Magnetic resonance imaging (MRI) revealed a large spinal meningeal cyst in the sacrum (60 × 37 × 22 mm) consisting of multiple perineural cysts. The cyst eroded the surrounding sacral bone structures, narrowed several sacral foramina, and compressed neighboring nerve fibers. MRI findings on sacroiliac and hip joints were normal.

Restless legs syndrome in patients with systemic sclerosis. Prevalence and possible causes.

This study aims to assess the prevalence and possible causes of restless legs syndrome (RLS) in patients with systemic sclerosis (SSc). The prevalence of RLS was assessed in 27 patients with SSc and 60 healthy controls. We evaluated the impact of age, disease duration, disease form, antibodies, and metoclopramide on the development of RLS in SSc patients. In order to reveal peripheral neuropathy or iron deficiency as possible causes of the syndrome, patients with RLS underwent electromyoneurography (EMNG) and ferritin level testing. RLS was diagnosed in 40.7% of SSc patients (11 out of 27), compared to 4.9% of healthy controls (p< 0.001). Eight out of 18 patients (44.4%) with diffuse SSc and 3 out of 9 patients (33.3%) with limited SSc exhibited RLS (p = 0.28). Mean age and mean disease duration did not differ significantly between patients with or without RLS. RLS was observed in 47.1% of patients with anticentromere antibodies and 30% of patients with anti-topoisomerase I antibodies (p = 0.22). RLS was diagnosed more frequently (p = 0.02) in patients taking metoclopramide (75%) than in patients who were not being treated with this drug (26.3%). EMNG revealed sensitive polyneuropathy in four, and lumbosacral radiculopathy in two out of 11 patients with RLS. Two patients with SSc and RLS had low levels of ferritin. Three patients had normal EMNG findings and ferritin levels. In conclusion, RLS is a common disorder in patients with SSc. Possible causes include sensitive polyneuropathy, lumbosacral radiculopathy, and iron deficiency. A strong relationship was observed between RLS and metoclopramide, which is sometimes used to treat SSc-related gastrointestinal motility disturbances.

Induced sputum in systemic sclerosis interstitial lung disease: comparison to healthy controls and bronchoalveolar lavage.

BACKGROUND: Induced sputum (IS) is a noninvasive tool, which can be used to collect cellular and soluble materials from lung airways. OBJECTIVE: To evaluate if IS may be a useful and safe tool for the detection of airway inflammation in patients with interstitial lung disease (ILD) in systemic sclerosis (SSc). METHODS: Sixty-eight patients with SSc and ILD as well as 18 healthy individuals (controls) were selected and submitted to IS examination. In 34 of 68 patients with SSc, bronchoalveolar lavage (BAL) was also performed. Safety of IS was assessed by comparison of forced expiratory volume in the first second (FEV(1)), FEV(1)/forced vital capacity ratio and peak expiratory flow before and after the IS procedure. Cell composition in samples collected by BAL and IS was correlated, and IS total and differential cell count in SSc patients and controls were compared. RESULTS: The total number of cells was significantly higher in IS samples of SSc patients compared to those of healthy controls. Mean percentage of neutrophils was also higher in SSc patients (41.79 +/- 23.89 vs. 27.37 +/- 17.90), as well as lymphocytes (17.42 +/- 19.70 vs. 3.13 +/- 2.28) and eosinophils (2.35 +/- 4.43 vs. 0.41 +/- 0.46). On the other hand, mean percentage of macrophages was higher in healthy individuals (69.10 +/- 19.15 vs. 36.96 +/- 20.68). In fluid recovered by BAL, the most frequent cells were macrophages (67.89% +/- 17.26), while neutrophils (14.77 +/- 17.18%) and lymphocytes (15.62 +/- 13.46%) were less frequent and eosinophils (1.66 +/- 2.08%) were rare. A similar pattern of cell composition was found in IS samples (41.15 +/- 21.67% of macrophages, 39.72 +/- 23.15% of neutrophils, 15.28 +/- 19.46% of lymphocytes and 2.56 +/- 5.03% of eosinophils). Strength of correlation between BAL and IS was significant for macrophages and neutrophils. After IS procedure was performed, improvement of FEV(1) (mean value before IS was 85.09 +/- 14.44 and 88.93 +/- 16.40 after IS) and FEV(1)/forced vital capacity (mean value before IS was 98.53 +/- 12.11 and 105.22 +/- 10.78 after IS) was observed. CONCLUSION: The IS method may allow a noninvasive assessment of cell composition in airway fluid and may contribute to the better understanding of upper/medium airway inflammation in SSc. Future studies are needed to verify whether IS can replace invasive procedures for the detection and monitoring of lung inflammation in SSc.

The scleroderma Assessment Questionnaire (SAQ). A new self-assessment questionnaire for evaluation of disease status in patients with systemic sclerosis.

OBJECTIVE: To develop a self-assessment questionnaire and estimate its validity in the evaluation of disease status in patients with systemic sclerosis. PATIENTS AND METHODS: The developed questionnaire (SAQ) consists of 23 questions divided into four groups related to symptoms of vascular, respiratory, gastrointestinal and musculoskeletal dysfunction. One hundred and five patients with systemic sclerosis filled in the SAQ. Answers were assessed on a 0-3 scale and Index of Vascular Status (IVS), Index of Respiratory Status (IRS), Index of Gastrointestinal Status (IGS), Index of Musculoskeletal Status (IMMS) and Index of Disease Status (IDS) were calculated. Mann-Whitney and Kruskal-Wallis tests were used to examine the correlation of index score for particular organ system with various disease damage indicators. RESULTS: Mean score for IVS was significantly higher in patients with finger-tip ulcers or finger-tip osteolysis than in patients without, and was also higher in patients who had more severe capillary damage. Mean score for IRS was significantly higher in patients with pulmonary fibrosis, also in patients with reduced FVC, DLCO and DLCO/VA. Patients with esophageal hypomotility had a higher mean score for IGS than patients with normal esophageal motility. The mean score for IMSS showed a strong correlation with the skin score and was significantly higher in patients with reduced hand motility, joint contractures, muscle weakness or arthralgia/arthritis. The mean score for IDS was significantly higher in patients who had multisystemic involvement. CONCLUSIONS: The Scleroderma Assessment Questionnaire (SAQ) is a sensitive measurement of disease status and level of impairment of different organ systems in patients with systemic sclerosis.