Salazosulfapyridine-induced agranulocytosis in a patient on chronic hemodialysis with seronegative spondyloarthropathy: a case report

Background Salazosulfapyridine is a generally safe drug often used to treat rheumatoid arthritis and ulcerative colitis. However, agranulocytosis is a rare but serious adverse effect of this drug. To date, there have been no reports describing the clinical course of salazosulfapyridine-induced agranulocytosis in a chronic hemodialysis patient. Case presentation The patient was a 64-year-old man with IgA nephropathy who had been on chronic hemodialysis for about 3 years. For 1 month, he had general fatigue, mild fever, and pain in multiple joints of the upper extremities. He was hospitalized and underwent detailed examinations in our department. Laboratory investigations revealed an erythrocyte sedimentation rate of 67 mm/h and a C-reactive protein level of 7.73 mg/dL. Rheumatoid factor and anti-cyclic citrullinated peptide antibody were negative. Musculoskeletal ultrasonography showed inflammation of the tendon sheath in both wrists and the right shoulder joint. Computed tomography scans revealed osteosclerosis and narrowing of the sacroiliac joint. The diagnosis was seronegative spondyloarthropathy. He was started on salazosulfapyridine. Four weeks later, he had a high fever and low granulocyte count. Treatment with granulocyte colony-stimulating factor was started. The agranulocytosis could not be ascribed to any other cause and was considered an adverse effect of salazosulfapyridine, which was then stopped. Nine days later, the granulocyte count had recovered and the fever had resolved. Conclusions Currently, there are no guidelines on the use of salazosulfapyridine in chronic hemodialysis patients. The starting dosage should be smaller for these patients than for patients without renal impairment. Also, the laboratory monitoring interval for complete blood count should be shorter than usual.

Achondroplasia with seronegative spondyloarthropathy resulting in recurrent spinal stenosis : A case report

Background: Achondroplasia is an autosomal dominant condition caused by the G380 mutation of the gene encoding fibroblast growth factor receptor 3 on chromosome 4P. The classical findings include rhizomelic extremities, short stature, and spinal stenosis involving the upper cervical and distal lumbar spine. Rarely, achondroplasia coexisting with seronegative spondyloarthropathy can result in recurrent canal stenosis. Here, we report a 36-year-old male with symptomatic recurrent L3-L4 spinal stenosis 9 years following an original L2-S1 lumbar decompression for stenosis. Case Description: A 36-year-old male with achondroplasia (height of 113 cm and weight 43 kg [BMI-33.7]) presented with low back and right lower extremity sciatica (ODI 39). He had achondroplasia with a short stature. Nine years ago, he had an L2-S1 laminectomy for decompression of stenosis. When the new MRI revealed recurrent severe L3-4 stenosis, he underwent a repeated L3-L4 decompression with fusion. One year later, the patient was neurologically intact with radiographic confirmation of adequate L3-L4 arthrodesis. Conclusion: A 36-year-old male with achondroplasia and a history 9 years ago of an L2-S1 laminectomy for stenosis, presented with symptoms and signs of recurrent L3-L4 stenosis that responded to repeated decompression and fusion.

EP12 Chikungunga arthritis mimicking acute seronegative spondyloarthritis

Case report - Introduction Chikungunya is a tropical arbovirus transmitted by female Aedes Aegypti or Aedes Abopitus mosquitos. It is not indigenous to UK but occurs in epidemics in Africa and Asia. It often presents with pyrexia, arthralgia or arthritis, myalgia and a maculopapular rash and can mimic both peripheral and axial inflammatory arthritis as well as more common forms of viral arthritis. It can also become chronic leading to disabling symptoms. The diagnosis should be considered in all patients presenting with early inflammatory arthritis who have travelled to affected areas. Case report - Case description A 57-year-old female developed sudden onset fever along with a macular rash whilst visiting South East Asia. She then developed widespread joint pains and severe inactivity stiffness, particularly affecting her ankles. The rash and fever settled after a few days, but her arthralgia persisted in her cervical spine and both small and large joints. She had a history of recurrent episcleritis and had been investigated for axial spondyloarthropathy two years previously, but MRI imaging of the spine and sacroiliac joints did not show any inflammatory changes. Examination in the rheumatology clinic confirmed right medial epicondylitis, bilateral shoulder tenderness, tenderness over the extensor tendons of the feet and painful cervical spine movement. Investigations revealed high inflammatory markers; CRP 29 (0-10 mg/L) and ESR 48 (0-15 mm/hr), a positive rheumatoid factor but negative anti CCP antibodies and a normal white cell count. Acute seronegative spondyloarthropathy was suspected but Chikungunya serology was requested at the suggestion of the patient, because of the history of a mosquito bite. IgM and IgG antibodies were positive on immunofluorescence, confirming recent infection. She was initially given intramuscular depomedrone and non-steroidal anti-inflammatory drugs (NSAIDs) with a short response but required oral prednisolone 20mg daily to suppress the inflammation in her feet. An MRI confirmed an ankle effusion and peroneal tenosynovitis. After 6 months her symptoms improved, and she was able to stop prednisolone completely and she remains well 9 months after the initial infection. Case report - Discussion Chikungunya infection causes musculoskeletal symptoms in all affected patients, but the clinical presentation can highly variable, from mild joint pain to erosive arthritis. It can be divided into three phases: incubation phase, acute phase, and chronic phase. The incubation phase varies between one to twelve days after the mosquito bite. The acute phase begins with high fever, headache, polyarthralgia/arthritis, lymphadenopathy, and anorexia. Joint involvement is often distal and symmetrical affecting the hands, wrists, shoulders, knees, ankles, and feet. A maculopapular rash is common. Dengue virus and Zika virus infection can present similarly. Treatment for acute Chikungunya fever is supportive. Analgesic, anti-pyretic and NSAIDs are used for symptom relief. During the chronic phase, infected people develop symmetrical, migratory, oligoarticular or polyarticular arthritis with morning stiffness and joint oedema, which can last from months to years. Our patient had a previous history which was consistent with seronegative spondyloarthropathy, an acute presentation of inflammatory arthritis and results and imaging which supported this diagnosis. The correct diagnosis could easily have been missed if a travel history had not been taken and the patient’s suspicions ignored. The best treatment for chronic Chikungunya arthritis is unclear. NSAIDs are often the first treatment but, as in this case systemic steroids are often necessary. Conventional synthetic DMARDs have also been reported efficacious. Biologic DMARDS have been used in resistant cases. Case report - Key learning points Chikungunya has emerged as a global disease affecting millions of people with significant musculoskeletal morbidity. Any patient has travelled to endemic areas including Africa and Asia, with fever and joint pain should be screened for Chikungunya virus as well as Dengue virus, and Zika virus. Diagnosis is either by RT PCR (positive 0-7 days of infection or Immunoglobulin M (detectable after 5 – 10 day of infection and persists for few months). Treatment is supportive in acute phase, may require low doses of steroids to aid resolution of symptoms. Conventional DMARDS have shown benefit in chronic phase with ongoing synovitis/tenosynovitis. Patients may know more about rare, endemic diseases than their European doctors and their suspicions about potential diagnoses should always be considered.

Psoriasis and Risk of Uveitis: A Systematic Review and Meta-Analysis

Background. Uveitis is a known ophthalmologic manifestation of seronegative spondyloarthropathy, including psoriatic arthritis. However, the data is less clear among patients with psoriasis due to the limited number of published studies. Aims. To investigate whether the risk of incident and prevalent uveitis is elevated among patients with psoriasis using systematic review and meta-analysis technique. Methods. The MEDLINE and EMBASE databases were searched from their inception to May 2019. Eligible studies must have included a psoriasis group and a nonpsoriasis group. Eligible studies must also have investigated for prevalent or incident uveitis, and the magnitude of difference between the study groups must have been reported. Pooled risk ratio and 95% confidence interval (CI) were calculated using random-effect generic inverse variance methods. Results. Of 7,107 potentially eligible articles from the EMBASE and MEDLINE databases, 7 studies were included in the meta-analysis. Two of those studies compared the incidence, and 5 studies compared the prevalence of uveitis between the psoriasis and nonpsoriasis groups. For incident uveitis, a total of 5,865,801 patients (222,083 with psoriasis and 5,643,718 without psoriasis) were analyzed. For prevalent uveitis, a total of 1,343,436 patients (37,891 with psoriasis and 1,305,545 without psoriasis) were studied. The risk of incident uveitis was significantly higher among patients with psoriasis with a pooled risk ratio of 1.23 (95% CI: 1.05-1.45, I2=55%). The risk of prevalent uveitis was also significantly higher among patients with psoriasis with a pooled risk ratio of 1.97 (95% CI: 1.68-2.31, I2=0%). Conclusions. The results of this study revealed significantly increased risk of both prevalent and incident uveitis among patients with psoriasis.