Joe Thomas

The prevalence and associations of sleep disturbances in patients with systemic lupus erythematosus

The aim of this study was to analyze sleep complaints in patients with systemic lupus erythematosus (SLE) and to determine its prevalence and associations. Fifty outpatients with SLE and an equal number of age- and sex-matched controls were included in the study. Sleep quality was assessed using the Pittsburgh Sleep Quality Index (PSQI) in both cases and controls. Depressed mood, functional disability and pain severity were assessed in patients using standardized questionnaires. Disease severity, cumulative damage and presence of fibromyalgia were determined by clinical examination. Bivariate associations between sleep quality and disease-related variables as well as demographic variables were calculated. A series of hierarchical regression analyses were computed to determine the independent determinant of sleep quality. PSQI scores were significantly higher in patients with SLE. Prevalence of sleep disturbance was 62%. Functional disability, disease activity and depressed mood correlated positively with sleep disturbances. 36% of the patients satisfied ACR criteria for fibromyalgia. In multiple regression analyses disease activity was found to be an independent determinant of sleep quality. The prevalence of poor sleep quality in patients with SLE was higher than it is generally perceived to be. Functional disability, disease activity and depressed mood contributed significantly to sleep disturbances in SLE.

Low Body Mass Index Is Adversely Associated with Radiographic Joint Damage in Indian Patients with Early Rheumatoid Arthritis

Objective. Various factors affect joint damage in rheumatoid arthritis (RA). The influence of body mass index (BMI) is not adequately known. As BMI is potentially modifiable, we studied its influence on radiological joint damage in patients with RA. Methods. Treatment-naive patients with early RA (< 24 mo) were included. Demographic data were collected along with swollen joint count (SJC), tender joint count (TJC), erythrocyte sedimentation rate (ESR), and IgM-rheumatoid factor (IgM-RF). Radiographs of hands and feet were obtained. BMI and Disease Activity Score for 28-joint count (DAS28-ESR) were calculated. Joint damage was assessed using the Simplified Erosions Narrowing Score (SENS). Results. A total of 101 patients were studied (81 women; mean age 41.91 ± 11.99 yrs). Mean disease duration was 10.77 ± 6.73 months; 55 patients (54.5%) were IgM-RF-positive. Mean BMI was 22.82 ± 4.66 kg/m2 with 24 (23.8%) patients having low, 42 (41.6%) normal, and 35 (34.7%) high BMI. Mean SENS score was 16.81 ± 11.10; mean DAS28 was 6.23 ± 0.96. Significant correlation was noted between SENS and DAS28 (r = 0.28; p < 0.005). There was significant negative correlation between BMI and SENS (r = −0.509; p < 0.0005). In patients with low BMI, mean SENS (26.62 ± 13.45) was significantly higher than in patients with normal (15.88 ± 8.38; p < 0.001) and high BMI (11.20 ± 7.32; p < 0.001). Patients with normal BMI also had significantly higher SENS scores than those with high BMI (p < 0.05). One-way ANOVA did not reveal significant differences in DAS28 between groups. SENS was significantly higher in the IgM-RF-positive group (19.55 ± 11.36) than in the IgM-RF-negative group (13.54 ± 9.94; p < 0.01); DAS28 was not different between the 2 groups (6.22 ± 0.98 vs 6.26 ± 0.96, respectively). Within the 2 IgM-RF groups, a significant negative correlation was seen between BMI and SENS. Multiple regression analysis revealed RF, DAS28, and BMI were independently associated with SENS. BMI accounted for 23.04% of the variance in SENS independent of DAS28 and IgM-RF. Conclusion. Low BMI is adversely associated with joint damage in patients with early RA.

Successful treatment of pyoderma gangrenosum associated with juvenile idiopathic arthritis with a combination of topical tacrolimus and oral prednisolone

Dear Editor, A 16-year-old boy, known to have Juvenile Idiopathic Arthritis–extended oligo-articular (JIA), presented with a pustule over the left leg rapidly progressing to a large, exceedingly painful ulcer. On examination he had pallor, flexion deformity of right elbow and active arthritis of both wrists. An ulcer measuring 10×5 cm was there over left shin with central necrosis and undermined erythematous edges (Fig. 1a). A diagnosis of pyoderma gangrenosum (PG) was made and a biopsy from ulcer margin was consistent with this diagnosis. There was no evidence of malignancy or infection. He was initiated on oral prednisolone (1 mg/kg/day), methotrexate (10 mg/week) and topical tacrolimus (0.03% w/w) twice a day under occlusive dressing. After 3 weeks, the ulcer had significantly healed with complete resolution of pain (Fig. 1b). PG is a rare and a serious ulcerating disease of the skin of unknown cause [1]. Abnormal neutrophil chemotaxis has been implicated [2]. Affection for children is rare [3]. About a quarter of patients with PG have arthritis the severity of which is unrelated to PG [4]. PG has been more commonly reported in context of seropositive RA and spondyloarthropathies and rarely in JIA [1, 5]. Extra-cutaneous lesion may involve lungs and bones (multifocal sterile recurrent osteomyelitis) and can precede cutaneous lesions [6, 7]. The timing and site of the biopsy determine the histopathology of PG [8]. The principle of biopsy is to exclude other conditions such as malignancy and infections. Treatment includes local care with moist dressings. Various topical and systemic therapies have been tried with varying success. Initial response to treatment can be seen within days to weeks but complete remission may require months to years [1]. In our patient, methotrexate was initiated primarily for the treatment of JIA and also because of reports of its efficacy in PG. The ulcer however responded dramatically to corticosteroid and topical tacrolimus. To the best of our knowledge, this is the first report of JIAassociated PG to be successfully treated in such a short span of time with a combination of topical tacrolimus and oral corticosteroid. Clin Rheumatol (2009) 28:489–490 DOI 10.1007/s10067-008-1066-y

Melioidosis--an unusual cause of septic arthritis.

Melioidosis is an infection caused by Burkholderia pseudomallei. It is an important human pathogen in the tropical area. The clinical manifestations are protean with multisystem involvement. Septic arthritis and prostatic abscess are rare but well-recognized forms of the disease. Herein we report a case of melioidosis presenting with a rare combination of septic arthritis, prostatic abscess, and septicemia.

Kimura's disease--an unusual cause of lymphadenopathy in children.

Kimura’s disease is a rare inflammatory disorder of unknown cause primarily seen in young Asian males. The disease is characterized by a triad of painless subcutaneous masses in the head or neck region, blood and tissue eosinophilia, and markedly elevated serum immunoglobulin E levels. We describe a 3-year-old Indian boy with Kimura’s disease who presented with generalized lymphadenopathy. The diagnosis was based on the characteristic histopathologic findings after surgical excision in conjunction with peripheral eosinophilia and elevated serum immunoglobulin E levels.

Stenotrophomonas maltophilia: a very rare cause of tropical pyomyositis

Pyomyositis is a purulent infection of skeletal muscles that arise from hematogenous spread associated with abscess formation. Most often caused by Staphylococcus aureus in more than 90–95% of cases but other rare organisms can cause this infection. Herein we report a rare case of strenotrophomonas maltophilia as a cause of pyomyositis which is a rare occurrence, especially in immunocompetent adults. Strenotrophomonas is a multidrug‐resistant aerobic non‐fermentative, non‐sporulating, Gram‐negative bacillus which usually produces nosocomial infections, but community‐acquired infections are also rarely reported. This a first case report of strenotrophomonas maltophilia causing pyomyositis in an immunocompetent adult.

Cutaneous vasculitis as a presenting manifestation of acute myeloid leukemia

One of the rare causes of secondary vasculitides is malignancy. Hematological malignancies produce secondary vasculitis more frequently than solid malignancies. Here in we report a case of acute myeloid leukemia presenting with anti‐neutrophil cytoplasmic antibody‐positive vasculitis. This case highlights the importance of looking for underlying malignancies, especially leukemias in patients presenting with features of systemic vasculitides.

Simplified Disease Activity Index as an index of disease activity in patients with rheumatoid arthritis: a comparison with DAS28

Abstract Introduction Various factors are known to determine the disease activity in patients with rheumatoid arthritis (RA). The main objective of this study was to establish the validity of the new tool of measurement of disease activity, Simplified Disease Activity Index (SDAI), in patients with RA in Indian population. Methods All patients with RA fulfilling the ACR classification criteria attending the rheumatology clinic at Nizams Institute of Medical Sciences, Hyderabad over a period of 3 months were included in the study. A detailed assessment of each patient including their demographic characteristics, duration of the disease, number of tender and swollen joint counts, Westergrens ESR and C-reactive protein (mg/dL), patients and physicians global assessment by VAS (0–10) were recorded. DAS28 and SDAI were calculated for each patient. Statistical analysis was done. Results Two hundred and sixteen patients were included in the study comprising 184 women and 32 men. Mean age of the patients was 42.94 ± 11.23 years and mean duration of disease was 4.10 ± 4.02 years. Mean DAS28 and SDAI were 5.19 ± 1.48 and 24.2 ± 16.06 respectively. ROC curve revealed that discriminative ability of SDAI was better than that of DAS28. The optimal cut-off points for treatment changes were 4.46 for DAS28 (sensitivity 88%, specificity 87.5%) and 12.6 for SDAI (sensitivity 92%, specificity 83%). Conclusion SDAI is a valid tool for measurement of disease activity in RA in Indian population and is as good as DAS28 in its ability to assess the patients status.

Systemic lupus erythematosus and dermatomyositis with symptomatic bilateral sacroiliitis: an unusual and interesting association

Systemic lupus erythematosus (SLE) is a multisystem autoimmune connective-tissue disease with a complex phenotype and varying disease course. Although SLE often causes a polyarticular synovitis, there are only a few case reports of abnormalities of the synovial lined sacroiliac joints. We report a case with an overlap syndrome of SLE and dermatomyositis who subsequently developed bilateral symptomatic sacroiliitis. We conclude that sacroiliitis, although rare, can occur in patients with active SLE as a manifestation of the disease per se.

Muckle wells syndrome associated with multifocal clinically isolated syndrome

Dear Editor, A 21-year-old girl presented to our neurology department with a 2-week history of intractable vomiting and vertigo. Two days earlier, she had developed numbness of the left side of the body and dysarthria. The day before admission, she developed numbness of the right palm. She had been diagnosed with possible rheumatic heart disease at the age of 5 years. Examination revealed gaze-evoked nystagmus, right-sided lower motor neuron facial palsy, right Xth nerve palsy, tandem ataxia to right, left intention tremor and left-sided hypoaesthesia. Magnetic resonance imaging (MRI) of the brain showed irregularly enhancing lesions in the left lenticular region, internal capsule, lower midbrain and pons with restricted diffusion, suggestive of demyelination (Fig. 1). Screening of the spine was normal. Her laboratory parameters were normal except for an elevated erythrocyte sedimentation rate (ESR) of 84 mm/h. Serum ferritin levels, C3 and C4 complement levels, vasculitic workup, antinuclear antibodies, serum protein electrophoresis, viral markers and serum immunoglobulin M (IgM) were normal. Echocardiogram was normal. Lumbar puncture showed four cells with normal protein and sugar. Oligoclonal bands were not checked. Visual evoked potential was within normal limits. She was treated with intravenous methylprednisolone 1 g for 5 days and improved remarkably. At the time of discharge she was asymptomatic. One year later she presented to us with recurrent non-pruritic urticaria (which she retrospectively reported from the age of 8 years). She also reported episodic arthralgia and arthritis involving two or three joints along with recurrent short-lasting episodes of fever of 1–2 days every 2–3 months. She also had recurrent episcleritis, headaches and aphthous ulcers, as well as episodic abdominal pain lasting for a few days every couple of months. A repeat hepatitis B surface antigen and hepatitis C virus were negative. Acute phase reactants were high (ESR and C-reactive protein). Audiometry revealed moderate-to-severe sensorineural Figure 2 Low-power (Left) and high-power (right) histopathology slides showing dermal perivascular inflammatory infiltrates. Figure 1 Magnetic resonance imaging composite. Top panels: fluid-attenuated inversion recovery images show hyperintensities in the pons anterior to the fourth ventricle and the deep cerebral white matter. Bottom panels: post-contrast T1weighted images show irregular left hemispheric periventricular lesional enhancement.