Negative Rheumatoid Factor Research Articles

HLA-DR/DQ Genotypes in Kurd Patients with Rheumatoid Arthritis: Relation to Disease Activity.

Specific alleles present at the HLA-DR/DQ loci seem to be associated with disease activity of rheumatoid arthritis. In the present study, our aim was to investigate the distribution of HLA-DR/DQ alleles among Kurd patients with rheumatoid arthritis and to ascertain their relationship with disease activity. Sixty five patients with rheumatoid arthritis (RA) and 65 apparently healthy subjects participated in the study. Diagnosis and disease activity were confirmed. Blood analyses, including those of laboratory markers of disease activity, were done. The 28 joint disease activity score (DAS-28) was calculated. HLA-DR/DQ typing was performed by polymerase chain reaction (PCR).The association between HLA-DR/DQ genotypes and disease activity was determined. The most frequent alleles which were identified in RA patients were HLA-DRBI*01(23.1%) and HLA-DQBI*6(34.6%), whereas in healthy subjects, they were HLA-DRBI*11(17.7%) and HLA-DQBI*03(35.4%). Patients with active disease had high frequencies of HLA-DQBI*6 (40.0%) as compared to those with moderate disease activity (16.7%); OR=3.33. Patients with severe RA had increased frequencies of HLA-DQBI*6 (56.3%) as compared to those with mild RA (10.0%); OR = 11.57. Patients with positive rheumatoid factor (RF) and positive Anti-citrullinated peptide antibody (Anti-CCP), also had high frequencies of HLA-DQBI*06 (38.4% and 39.4%) as compared to frequencies of 11.1% and 15.4% which were seen in patients with negative rheumatoid factor and negative anti-CCP (OR= 4.98 and3.10) respectively. HLA-DQBI*06 was found to be more common in Kurd patients and it was significantly associated with disease activity; this may indicate a high risk for developing a more progressive type of the disease.

Atypical Presentation of Juvenile Rheumatoid Arthritis in a Patient with Monosomy 1p36

Monosomy 1p36 is a congenital genetic disorder characterized by moderate to severe global developmental and growth delay, hearing and vision impairment, hypotonia, seizures, and distinct facial features. We present the first published description of a patient with monosomy 1p36 with an atypical arthropathy, a feature also not currently described either standalone entity or as part of the monosomy 1p36 syndrome. The patient described is a 11-year-old male who was referred to the pediatric rheumatology clinic for morning stiffness, pain, swelling, and remarkable redness in his all proximal interphalangeal joints (PIP), right wrist and in his right hip with reduced range of motion. The patient had no family history of autoimmune diseases or inflammatory arthritis, tick bites, injuries or recent flu like illness. He resided on a farm in rural Missouri. Upon evaluation the patient was found to have inflammation and swelling in all the PIPs, right wrist as well as pain and limitations in internal rotation of right hip joint. The joints were significantly erythematous and angry looking. Further laboratory evaluations revealed the patient had normal white cell counts (WBC), normal ESR and C-reactive Protein, negative Rheumatoid Factor, was negative for HLAB27 gene and negative serologic tests for streptococcal antigens, mycoplasma and parvovirus. The Contrast MRI scan of his joints showed soft tissue swelling with no synovial enhancement or bone marrow edema. The patient was started on Meloxicam, with no response for 12 weeks. He then received triamcinolone acetonide intra articular injections in his all the PIP joints, and wrist joints with good response. Unfortunately within a month’s time his mid foot and interphalangial joints of several toes were inflamed. He was started on oral methotrexate immunosuppression to which he responded well and currently in remission on tapering doses of methotrexate. This report details a steroid-responsive atypical arthropathy found in association with monosomy 1p36. This arthropathy may be an atypical variant of juvenile rheumatoid arthritis in a patient with chromosomal anomaly, or perhaps an arthropathy associated with monosomy 1p36,an entity which is yet to be recognized. This arthropathy responded to therapy with immunosuppressant agents.

Adult onset Still’s disease: a comparison of two clinical cases

Still’s disease is a disease of unknown etiology that was identified for the first time in 1897 by George Still who noted the association of fever, arthralgias and cutaneous rash in a group of 22 children. In 1971, Bywaters noticed that this symptomatology could also be found in adult patients. High fever, arthralgias, a diffuse cutaneous rash with sore throat, increased spleen volume and lymph nodes are the clinical manifestations of this disease. However, it shows high variability in its clinical presentation (monocyclic, polycyclic and chronic forms). Blood tests show high levels of white blood cells, increased ferritin levels, and negative autoantibodies and rheumatoid factor. We examined 2 clinical cases that strongly suggested adult Still’s disease: a 38- year old woman and a 30-year old man. The woman came to our attention because of the following symptomatology: fever for more than eleven days before hospitalization and polyarthralgias. Blood tests showed high inflammatory markers (ferritin >35,000), low platelets and increased white blood cells. A diffuse rash and oral aphthae were then observed. The second case presented the following symptoms: fever (38.4°C), cutaneous rash, polyarthralgias, and sore throat. Blood tests showed high levels of inflammatory markers and blood cell count showed an increase in neutrophil levels. Abdominal ultrasonography showed hepatosplenomegaly. Concentrating on these elements allowed us to formulate the diagnosis of adult Still’s disease, both patients showing a highly suggestive clinical profile. Yamaguchi’s criteria were satisfied and different diseases could be excluded according to the pharmacological response to anti-inflammatory drugs. This strengthened our diagnostic hypothesis and allowed us to focus our attention on this unknown disease, often unrecognized because of the problems of diagnosis. In fact, the clinical characters are initially aspecific (sore throat and fever). Furthermore, there is a large inter-individual variability during the disease course that could lead to misunderstanding. However, satisfaction of Yamagouchi’s criteria and the pharmacological response to corticosteroid drugs suggested adult Still’s disease.

Non-systemic juvenile idiopathic arthritis outcome after reaching clinical remission with anti-TNF-α therapy: a clinical practice observational study of patients who discontinued treatment

TNF-alpha-blocking agents (anti-TNF) used in juvenile idiopathic arthritis (JIA) are well established; however, time to withdraw is unclear. Neither prolonged nor tapering treatment seems to influence risk of relapse. Our aim was to assess relapse percentage after anti-TNF withdrawal of our non-systemic JIA patients after reaching clinical remission. A retrospective review of our non-systemic JIA patients in whom anti-TNF had been withdrawn due to inactive disease was achieved, between December 2000 and November 2011. We analyzed percentages of relapse according to JIA categories and antinuclear antibodies (ANA) positivity. n = 18 patients were included. Eighty-two percentage of patients relapsed after treatment withdrawal, and mean time to relapse was 3.04 months (SD 2.03). The percentage of relapse after anti-TNF discontinuation in the main JIA category was 88 % of negative rheumatoid factor polyarticular JIA and 80 % of persistent oligoarticular JIA. We did not find significant statistical differences according to ANA positivity (9 of 14 were ANA positive), and mean time to relapse (days) was 85.0 (SD 69.4) for ANA-positive versus 102.4 (SD 47.7) for ANA-negative patients (p = NS). Relapse percentage following anti-TNF discontinuation was high (82 %) and occurred within the first 3 months after it. No relationship regarding JIA subtype and ANA positivity was found.

Gammaglobulin Treatment in an Unusual Meningococcal Case

To the Editor: Prolonged fever is a possible complication of meningococcal meningitis, appearing in approximately 10 % of cases. Defined as fever lasting from four to ten days after the symptoms of meningitis begin, it can be classified as persistent, secondary or recurrent [1]. In the persistent type, the fever never disappears, and intracranial causes such as brain abscesses, subdural empyema, and vasculitis must be ruled out [1]. The secondary fever re-appears atleast 24 h after being afebrile. Extracranial causes should be suspected, including viral and bacterial infections [2, 3]. Finally, with the recurrent fever, a parameningeal focus of infection and congenital defects should be investigated [1]. We report an 11-mo-old male infant with meningococcal meningitis septicemia, who remained afebrile between 24 and 72 h after starting the antibiotic therapy. Fever re-appeared on the fourth day of antibiotic treatment, associated with palpable nodules on his left arm. Four days later, despite non-steroidal anti-inflammatory drugs (NSAIDs) being administered, vasculitis by immune complexes was suspected, and oral steroid treatment with prednisolone was initiated with doses of 2 mg/ kg/d. He remained afebrile afterwards and was discharged two days later, receiving antibiotic treatment and oral steroids for a week. Fever re-appeared 24 h after steroid treatment was completed. Palpable nodular elements persisted together with the appearance of new ones, and arthralgia and a systolic murmur were detected. We restarted 2 mg/kg/d prednisolone dose with subsequent tapering to 0.3 mg/kg/d, with new fever recurrence after it was suspended. Laboratory studies showed declining levels of C-reactive protein and the erythrocyte sedimentation rate, rising levels of complement factors, negative antinuclear antibody and rheumatoid factor. An echocardiography and urinalysis were normal. The immune complex vasculitis-arthritis was controlled with steroid treatment but was not resolved yet. Two doses of 2 g/kg/d of intravenous gammaglobulin were needed, after which the fever and arthralgia disappeared. The presence of palpable nodules, arthralgia and increased acute phase reactants lead to the diagnosis of vasculitis-athritis by immune complexes [4, 5]. Although there are no protocols involving immunoglobulins for vasculitis management, it has been an effective and extensive treatment over the years in medical practice. Our patient required two doses of gammaglobulin with subsequent complete remission of symptoms.

A case of primary Sjögren'/INS;s syndrome presented with dysautonomia as initial manifestation

Neurological involvement occurs in 10–/INS;42% of the primary Sjö/INS;gren's syndrome (pSS). Distal sensory neuropathy is the most common, pandysautonomia without sensory neuropathy is extremely rare in the pSS as initial manifestation. We describe the case of pSS presented with bladder dysfunction, paralytic ileus and postural intolerance. /INS;A 27-year-old woman developed paresthesia in the both arms 1 week before admission and progressed paresthesia in the legs and trunk below the chest with myalgia. At admission she couldn't urinate and defecate. On examinations she was revealed with /INS;abdominal distension, decreased deep tendon reflexes and sensation for the temperature in the extremities. Autonomic symptoms and signs included no bowel sound during auscultation, increased perspiration on the whole body, bladder distension and postural intolerance with normal pupil sizes and light reflex. Abdomen CT revealed urinary bladder distension and diffuse dilated colon without obstruction suspected paralytic ileus. She had positive anti-SS-A/Ro Ab, anti SS-B/La Ab, positive anti-nuclear antibody (titer 1:40, homogeneous pattern) and anti dsDNA antibody of 38.05 IU, but with negative rheumatoid factor, anti-Sm Ab and Anti-RNP Ab. Salivary scan revealed decreased activity of both the /INS;parotid glands and sublingual glands. Motor and sensory nerve conduction studies of median, ulnar, radial, tibial, peroneal and sural nerves are mild delayed terminal latency with normal amplitude and velocity. During months her symptoms recovered without steroid or IVIG. This case suggests that pSS should be listed in the differential diagnosis of acute autonomic failure as well as chronic form.

AB0371 In RA patients with very strongly positive serology there is a striking discordance in presentation between those with very strongly positive ACPA levels associated with negative or low-positive rf levels and those with very strongly positive RF levels

BackgroundRA has been viewed for many years as a heterogeneous collection of rheumatic diseases. Previously rheumatoid factor (RF) positive and negative patients were regarded as distinct clinical subtypes. Additionally, within...

THU0340 Cytokines’ study in patients with different subtypes of juvenile idiopathic arthritis

BackgroundCytokines play a significant role in pathogenesis of different subtypes of Juvenile Idiopathic Arthritis (JIA). Cytokines’ profile investigation enables differentiation of these subtypesObjectivesTo investigate cytokines, their receptors and agonists in...

OP0181 New Autoantibodies as Biomarkers for Early and Seronegative Rheumatoid Arthritis

BackgroundApproximately one third of rheumatoid arthritis (RA) patients test negative on the current diagnostic biomarkers rheumatoid factor (RF) and antibodies directed against cyclic citrullinated peptides (ACCP) leading to a difficult...

Undifferentiated seronegative spondyloarthritis with inflammatory bowel disease and a family history of psoriasis. Sicca syndrome

Background: Seronegative spondyloarthritis is characterized by the presence of subcutaneous nodules, asymmetrical peripheral arthritis, sacroileitis with or without spondylitis, and rheumatoid-factor negativity. Other common clinical manifestations include oral ulcers, conjunctivitis, and cutaneous lesions such as psoriasis. Familial aggregation has also been described. According to the 1986 classification, corresponding clinical entities include ankylosing spondylitis, psoriatic arthritis, Reiter’s syndrome, arthritis associated with inflammatory bowel disease (IBD), and undifferentiated spondyloarthritis. The disease is also frequently associated with the HLA B27 antigen. From the clinical point of view, there are often incomplete forms of spondyloarthritis, such as reactive arthritis triggered by asymptomatic infections, psoriatic arthritis without psoriasis itself, initial phases of specific forms of spondyloarthritis or the phase of ankylosing spondylitis characterized by sacroiliac lesions, and all forms that remain undifferentiated for long periods of time. Moreover, there are close relations between arthropathy and IBDs, such as Crohn’s disease, ulcerative colitis, and Whipple’s syndrome. Recently, microscopic inflammatory bowel lesions and psoriatic arthritis have been described. Case report: A 30-year-old man (HLA B27-negative) who had been vaccinated against TBC and HBV presented with a 6-year history of recurrent episodes of predominantly left-sided sciatica. The pain was worse at night and during rest. He was suffering from bilateral sacroileitis without spondylitis. Three to five times a day, usually after eating, he passed watery feces containing mucous and small amounts of bright red blood. Colonoscopy revealed pancolitis with histological evidence of chronic inflammation interspersed with areas of acute inflammation, edema, hyperemia, and glandular distortion. One year later, the clinical manifestations and histological findings were essentially unchanged: glandular distortions, chronic and acute inflammation of the lamina propria and crypt microabscesses. There were no granulomas and no evidence of uveitis. The inflammatory index was positive; FR, ENA, ANA titers were negative. He began therapy with adalimumab (loading dose 80 mg followed by 40 mg every 15 days) and mesalazine (2.4 g per os), and the clinical manifestations of the disease improved significantly.

Diagnosis and surgical treatment of progressive pseudorheumatoid dysplasia in an adult with severe spinal disorders and polyarthropathy

Progressive pseudorheumatoid dysplasia (PPD) is a rare autosomal-recessive disorder. The polyarthritis of PPD has been detailed before. However, the spinal disorder and surgical treatment been rarely mentioned. A 44-year-old patient who has been misdiagnosed as juvenile rheumatoid arthritis (JRA) and given unilateral total hip replacement yet, suffers mainly from severe spinal disorder this time. The platyspondyly, Scheuermann-like lesions of the spine and JRA-like features of the peripheral joints were found on radiographic films, combining negative inflammatory and rheumatoid factors, which most suggested the diagnosis of PPD. As the homozygous nucleotide deletion was found in WISP3 gene, diagnosis of PPD was definite. Neurological examination and further imaging examination indicated severe compression of thoracic and lumbar spinal cord which might lead to his conspicuous spinal disorder. Decompressive laminectomy, posterior fusion and fixation were performed. And an excellent clinical outcome was achieved 1year after the decompression and fusion: leg pain and hypoesthesia resolved and osseous fusion performed. This is the first reported decompression in the adult spine of PPD. Surgical treatment could receive satisfactory result in PPD, however, it is a palliative therapy which has less help to prevent the development of this disease. Early diagnosis and rehabilitation interventions remain the most important. Clinical, radiographic and genetic features in PPD are crucial in the differential diagnosis.

Unique Correlation Between Mutated Citrullinated Vimentine IgG Autoantibodies and Markers of Systemic Inflammation in Rheumatoid Arthritis Patients

Rheumatoid arthritis (RA) is the most common inflammatory systemic autoimmune disease, primarily affecting the peripheral joints. Anti-mutated citrullinated vimentin autoantibodies (anti-MCV) of IgG isotype were shown to be a useful diagnostic marker of RA especially in RA patients who were anti-cyclic citrullinated protein autoantibodies (anti-CCP) negative. Nevertheless, published data correlates rheumatoid factor (RF), anti-CCP or anti-MCV antibodies with either erythrocyte sedimentation rate (ESR) or serum C-reactive protein (CRP) as markers of disease activity, not investigated the possible correlations of RA autoantibodies towards ESR and CRP in comparison. Herein, we aim to evaluate the usefulness of anti-MCV as a dependable marker in established RA compared with anti-CCP and RF antibodies and to examine correlations between RF, anti-CCP and anti-MCV antibodies towards ESR and serum CRP. Serum RF-IgA, RF-IgM, anti-CCP and anti-MCV levels were measured in 30 patients with RA and 40 patients with other autoimmune diseases (non-RA) compared with 20 normal subjects. Specificity, sensitivity and AUC for RF antibodies, anti-CCP and anti-MCV were calculated towards RA diagnosis. Our results showed that ESR and CRP had significantly higher values in both RA and non-RA patients compared with our healthy controls with observed significant increment in RA patients compared with non-RA patients. An important finding from our study is that 33.3% of RA patients were anti-CCP negative but being positive towards anti-MCV. Also, in-between 36.7 up to 40% of RA patients were RF-IgA and RF-IgM negative while being anti-MCV positive. Anti-MCV antibodies showed the highest specificity and sensitivity (97.5 and 86.6%, respectively) towards RA diagnosis with the highest AUC value (0.920) compared with anti-CCP and RF antibodies. Correlation analyses revealed that there was no significant correlation between ESR along with CRP towards RF-IgA, RF-IgM and anti-CCP while profound highly significant correlation exhibited between ESR and CRP towards anti-MCV data (r=0.879 and 0.994, respectively). Thus, our data suggest that the assessment of serum anti-MCV autoantibodies along with ESR and CRP considered as a simple laboratory regime for monitoring RA patients to assess and follow-up disease activity. The addition of anti-MCV autoantibodies to serologic markers in the ACR/EULAR classification criteria for RA will add points for patients with negative anti-CCP and RF antibodies.

Sensitivity and specificity of the Classification of Psoriatic Arthritis criteria in early psoriatic arthritis

To assess the sensitivity and specificity of the Classification of Psoriatic Arthritis (CASPAR) Study Group criteria in early psoriatic arthritis (PsA) and to compare them with the sensitivity and specificity of the Moll and Wright criteria. The CASPAR Study Group criteria were applied to patients with early PsA (<24 months symptom duration) and to control patients with other new-onset inflammatory arthritides. Both groups were naive to all disease-modifying antirheumatic drugs. The gold standard diagnosis was confirmed by the consulting rheumatologist using radiography and magnetic resonance imaging where required. Proportions of patients and control patients meeting the criteria were compared using McNemar's tests. We recruited a total of 111 patients with early PsA and 111 control patients with other forms of inflammatory arthritis (82 with rheumatoid arthritis, 13 with undifferentiated arthritis, 9 with spondylarthritis, 4 with inflammatory osteoarthritis, and 3 with crystal arthritis) to the study. The sensitivity of the CASPAR Study Group criteria in classifying early PsA was 87.4% compared to 80.2% for the Moll and Wright criteria. The specificity for both criteria was 99.1%. When considering different cut points for the CASPAR Study Group criteria, the best cut point for classification remained a score of ≥ 3 as in the original CASPAR Study Group analysis. Considering a score of ≥ 2 gave a higher sensitivity of 99.1% but resulted in a drop in specificity to 94.6%. Regression analysis determined that psoriasis and rheumatoid factor negativity were the most important features that differentiated PsA, followed by nail psoriasis and current or previous dactylitis. The CASPAR Study Group criteria are more sensitive than the Moll and Wright criteria in classifying early PsA. Although their sensitivity for early PsA is lower than that for established disease, the CASPAR Study Group criteria are valid for use as inclusion criteria for trials in early PsA.

Jaccoud’s arthropathy: proper classification criteria and treatment are still needed

A deforming arthropathy was first described in patients with rheumatic fever (RF) by Francois-Sigismond Jaccoud more than a century ago [1], and it has been widely known in the literature as Jaccoud’s arthropathy (JA). Presently, the majority of the cases of JA are seen in systemic lupus erythematosus (SLE) [2]. Either in RF or in lupus, its prevalence is around 5 %. Such arthropathy has also been described in other connective tissue diseases, neoplasias and infections [3]. JA is characterized clinically by ‘‘reversible’’ joint deformities such as swan neck, thumb subluxation, ulnar deviation and ‘‘boutonniere.’’ However, the term ‘‘reversible’’ seems to be inappropriate because once the deformity is installed it lasts forever. Likewise, although the ‘‘reducible’’ nature of the arthropathy is demonstrated in the majority of the cases, sometimes the longstanding process of fibrosis in soft tissues of the joints can lead to a limitation of their mobility, making the diagnosis of JA still more difficult. Although mainly observed in hands, JA has also been seen in other sites such as feet, knees and shoulders [4]. The classical imaging feature of JA is the absence of articular erosions on a plain radiograph. The mechanisms responsible for the development of JA are not entirely known, but it is recognized that the deformities are a result of soft tissue abnormalities including laxity of ligaments and joint capsule and secondary deviation of the tendon from its axis with the contribution of muscular imbalance. Synovitis may contribute to the process, but it is not as aggressive as in rheumatoid arthritis (RA). Some studies have suggested an association of JA with hypermobility syndrome, and others have attempted to establish an association with different antibodies in SLE patients, but their findings did not lead to any definite conclusion. A limitation on the study of JA is the lack of definite diagnostic or classification criteria. Previous attempts to classify JA were made based on the presence of ‘‘reversible deformities’’ and absence of erosions on X-rays and rheumatoid factor negativity [5] or as ‘‘any deviation of the metacarpus finger axes assessed by a goniometer’’ [6]. Spronk et al. [7] developed a diagnostic ‘‘index’’ which allowed for the presence of different deformities and attributing JA a score of over five points. None of these sets of criteria has been universally accepted. Furthermore, to make the definition of JA more complicated, it has also been observed in individuals without any associated disease, a form referred to as ‘‘idiopathic’’ or in the elderly population as ‘‘senescent’’ [8, 9]. In fact, it is not rare to find typical ‘‘swan neck’’ deformity in normal population as an individual phenotypical feature. Sometimes, other members of the same family may have such deformities. In these cases generally, there is no other type of joint deformity. Thus, we understand the definition of JA is more appropriate if the criteria below are fulfilled:

Profiling anti-cyclic citrullinated peptide antibodies in patients with juvenile idiopathic arthritis

BackgroundAnti-citrullinated protein/peptide antibodies (ACPA), have high specificity for rheumatoid arthritis (RA). Some children with juvenile idiopathic arthritis (JIA), phenotypically resemble RA and test positive for rheumatoid factor (RF) a characteristic biomarker of RA. We investigated the prevalence of ACPA and its relationship to other serologic markers associated with RA in a well-characterized JIA cohort.MethodsCases were 334 children with JIA, 30 of whom had RF + polyarticular JIA. Sera from all cases and 50 healthy pediatric controls were investigated by ELISA at a single time point for anti-cyclic citrullinated peptide (anti-CCP) IgG, RF IgM, IgA and IgG, anti-RA33 IgG, and antinuclear antibodies (ANA). Comparisons between cases and controls were made using Chi-square or Fisher exact tests and T-tests.ResultsThe prevalence of RF was 8% among controls, and 12% among cases (ns). The prevalence of ACPA was 2% in controls and 14.3% in cases (OR 8.2, p <0.01). Children who were ACPA-positive and RF-negative (n = 23) had a significantly earlier onset-age (4.6 years vs. 12.1 years, p <0.00001) and had fewer HLA-DRB1 shared epitope alleles than those positive for both RF and ACPA (n = 25). Prevalence of anti-RA33 was not different between cases and controls.ConclusionsACPAs are detectable in 14% of children with JIA. Children with positive ACPA but negative RF are frequent, and may define a distinct subset of children with JIA. ACPA testing should be included in the classification of JIA.

Lichen Planus in Association with Adult-onset Still’s Disease Successfully Treated with Mycophenolate Mofetil

To the Editor: A 55-year-old woman presented with acute onset of spiking fever, symmetric polyarthritis with intense myalgia, and sore throat. Onset was associated with diffusely itchy purple papules involving the trunk, anterior chest wall, and upper and lower limbs. Initial laboratory investigation showed elevated erythrocyte sedimentation rate (90 mm/h), elevated C-reactive protein (17.8 mg/dl), negative rheumatoid factor (RF), negative anti-citrullinated protein antibody, and negative antinuclear antibody (ANA). Complete blood count showed leukocytosis with white blood cell count 14.7 × 103/μl, anemia with hemoglobin 9.6 g/dl, thrombocytosis with platelet count 802 × 103/μl, and 2-fold increase in serum ferritin levels (350 ng/ml, normal values up to 150 ng/ml). Other laboratory investigations showed elevated liver enzymes [aspartate transaminase 69 IU/l, alanine transaminase 125 IU/l, and elevated creatine phosphokinase (CPK) levels 320 IU/l, normal 40–120 IU/l] with negative virology screening for viral hepatitis. Skin biopsy showed focal thinning of the epidermis with focal destruction of basal layers and focal dermal mononuclear infiltrate, features consistent with the diagnosis of lichen planus … Address correspondence to Dr. Emad; E-mail: yasseremad68{at}yahoo.com

Kikuchi Fujimoto's Disease Appearing as Systemic Lupus Erythematosus

A 22-year-old woman with no personal history came to the hospital due to fever and cervical lateral adenopathy which started 15 days prior. Laboratory tests show leukopenia 2800 per mm3 (with no lymphopenia), normocytic, normochromic anemia (hemoglobin 11 g/dl, hematocrit 34%, mean corpuscular volume 85.9, mean corpuscular hemoglobin 28.6 pg) and ESR 85 mm/h. The blood chemistry and urine sediment shows no abnormalities. HIV, B and C hepatitis, toxoplasma, rubeola, Epstein–Barr virus and cytomegalovirus were negative. Blood cultures and Mantoux tests were negative. Autoimmunity tests showed positive ANA with a 1/320 titer (homogeneous pattern), negative antiDNA, negative rheumatoid factor and normal complement levels. Thorax and abdominal CT showed multiple cervical and inguinal adenopathies. A bone marrow aspirate-biopsy was performed, as well as cultures, which were all negative. A cervical lymph node biopsy was performed, showing paracortical necrosis with cell detritus and numerous hystiocytes, compatible with necrotizing lymphadenitis or KFD (Fig. 1). In the following years the patient remained stable, with no further episodes of fever, although she did present self-limited cervical adenopathy. She repeatedly presented painful oral ulcers and further analysis confirmed leukopenia (without lymphopenia). Three years later she presented painful erythematous nodules on the forehead and cheeks (Fig. 2). Lab-

Remitting Seronegative Symmetrical Synovitis with Pitting Edema Syndrome in a Chronic Hemodialysis Patient

A 75-year-old male who was undergoing chronic hemodialysis developed abrupt-onset pitting edema and pain in the dorsum of both hands and feet. Biochemical analysis disclosed increased C-reactive protein, and negative rheumatoid factor and antinuclear antibody. Radiological examination showed no bony erosion. Computed tomography and gallium scintigraphy revealed no active infection or neoplasms. The clinical diagnosis was remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome. The pitting edema and inflammatory response quickly subsided after low-dose prednisolone therapy. This case demonstrates that RS3PE syndrome could be a differential diagnosis in elderly patients undergoing dialysis who develop pitting edema and joint pain.

Inflammatory and Noninflammatory Arthropathy in Patients With 18q Deletion Syndrome

A 7-year-old girl with 18q deletion syndrome developed chronic progressive polyarticular inflammatory arthropathy. Atypical features of her arthritis included lack of morning stiffness, absence of pain and discomfort, normal acute-phase reactants, and the presence of clinodactyly, low-set thumbs, metatarsus adductus of her feet, and overriding nontender swollen toes. She had a positive antinuclear antibody test, negative rheumatoid factor and anti-cyclic citrullinated peptide, and undetectable immunoglobulin A level. Magnetic resonance imaging of the right knee and the result of the synovial biopsy were consistent with synovitis. She was treated with naproxen, short course of prednisone, and methotrexate with good clinical response that plateaued over time. We analyzed the scarce reports of inflammatory arthropathy in 18q deletion syndrome and proposed an outline for investigating arthropathies in patients with chromosomal aberrations.

Anti-CD-20 Therapy in Refractory Adult Still’s Disease

Adult Still’s disease is a relatively rare form of rheumatoid arthritis with systemic inflammatory features. The prevalence is around 1.5 cases per 100,000 - 1000,000. In the current case we display a 30-year-old male patient with refractory adult still’s disease who suffered recurrent attacks of fever 39.5°C, arthritis in proximal interphalangeal joints (PIPs), wrists, tempromandibular joints (TMJs), knees and ankles, stitching chest pain, dyspnea, erythematous rash over the trunk, sore throat, weight loss (15 Kilograms in 4 months). The patients’ disease remained uncontrolled despite of synthetic disease modifying anti-rheumatic drugs and repeated intramuscular corticosteroid injections. Laboratory workup revealed erythrocyte sedimentation rate (ESR) of 95, C-reactive protein (CRP) of 100 mg/L, hemoglobin 10.5 gm%, leukocytosis 12,000/microlitre, mild elevation of liver function tests and dyslipidemia. Serology revealed negative rheumatoid factor, anti-nuclear antibody titre of 1:80, elevated serum ferritin 4000 micrograms/litre. The patient was started on rituximab (375 mg/m2), prednisolone 20 mg/day and selective Cox-2 inhibitor. Follow up for over three months following the completion of his pulse therapy, revealed no relapse of fever or fatigue, with morning stiffness of 5 - 10 minutes, VAS of 3, DAS28 of 3.8, HAQDI of 0.62, ESR 23, CRP 4.9, Hb 12.5 gm%, leucocytic count 9000/microlitre, the dose of prednisolone was successfully reduced to a dose of 5 mg/day orally. Conclusion: Anti-CD20 therapy successfully controlled systemic and articular refractory disease with sustained efficacy over a follow up period of up to 24 weeks.