Treatment challenges in an atypical presentation of tubulointerstitial nephritis and uveitis (TINU)

Purpose: To describe an atypical presentation of Tubulointerstitial Nephritis and Uveitis (TINU), with challenges in treatment course. Observations: A 12-year-old Hispanic female presented to the National Eye Institute\u27s Uveitis clinic with bilateral blurred vision, red eyes and photophobia, not responsive to topical steroids. On exam, she had bilateral severe panuveitis with areas of subretinal fluid. During her evaluation, she was noted to have elevated serum creatinine. A kidney biopsy confirmed the presence of severe tubulointerstitial nephritis and interstitial fibrosis. She was treated with oral steroids with excellent resolution of symptoms and subretinal fluid. She continued to have anterior segment flares with attempts to taper oral prednisone which lead to treatment with multiple immunomodulatory agents. Associated hypertension and kidney damage complicated the choice of a secondary immunosuppressive agent. Conclusions and Importance: Although rare, TINU can present as panuveitis with choroidal involvement which may or may not be preceded by tubulointerstitial nephritis. A renal biopsy is required for definitive diagnosis, but abnormal urinalysis or renal function should raise suspicion for TINU. © 201

Treatment challenges in an atypical presentation of tubulointerstitial nephritis and uveitis (TINU)

Purpose: To describe an atypical presentation of Tubulointerstitial Nephritis and Uveitis (TINU), with challenges in treatment course. Observations: A 12-year-old Hispanic female presented to the National Eye Institute's Uveitis clinic with bilateral blurred vision, red eyes and photophobia, not responsive to topical steroids. On exam, she had bilateral severe panuveitis with areas of subretinal fluid. During her evaluation, she was noted to have elevated serum creatinine. A kidney biopsy confirmed the presence of severe tubulointerstitial nephritis and interstitial fibrosis. She was treated with oral steroids with excellent resolution of symptoms and subretinal fluid. She continued to have anterior segment flares with attempts to taper oral prednisone which lead to treatment with multiple immunomodulatory agents. Associated hypertension and kidney damage complicated the choice of a secondary immunosuppressive agent. Conclusions and Importance: Although rare, TINU can present as panuveitis with choroidal involvement which may or may not be preceded by tubulointerstitial nephritis. A renal biopsy is required for definitive diagnosis, but abnormal urinalysis or renal function should raise suspicion for TINU. Keywords: Immunosuppression, Inflammation, Tubulointerstitial nephritis, Uveiti

Survey of current practices in the management of anti-TNF failure in juvenile spondyloarthritis

OBJECTIVES: To evaluate the current practices in management of patients with juvenile spondyloarthritis (JSpA) who failed anti-tumour necrosis factor agents (anti-TNF). METHODS: An online survey was distributed to Childhood Arthritis and Rheumatology Research Alliance (CARRA) members of the JIA workgroup. Data collection included estimated number of JSpA patients who have failed anti-TNF therapy over two-year period, reasons for discontinuing anti-TNF therapy and other medications used afterward. The JSpA population was de ned as the following subtypes: enthesitis-related arthritis, psoriatic arthritis, undifferentiated spondyloarthritis, juvenile ankylosing spondylitis (AS) i.e. meeting modi ed NY criteria for AS before age 16, and reactive arthritis. Findings were summarised using descriptive statistics. RESULTS: The survey response rate was 36% (n= 60/169). The majority of participants were paediatric rheumatologists (93%). Many physicians have JSpA patients who failed anti-TNF therapy (63%). The most common reason for changing anti-TNF therapy was secondary non-response (72%). Sacroiliitis was the most important factor considered when assessing response to an anti-TNF agent and the most common reason for primary non-response (45%). When assessing anti-TNF failure for sacroiliitis, many (65%) felt imaging of the sacroiliac joints was the most important aspect in their decision making. The majority try a second anti-TNF agent after initial anti-TNF failure (87%) and switch to another medication class after 2 anti-TNF agents have failed (62%). CONCLUSIONS: More than half of paediatric rheumatologists surveyed have at least one JSpA patient who failed anti-TNF therapy. The majority failed because of secondary non-response. Sacroiliitis is an important but challenging aspect to manage for patients with JSpA

Modified Juvenile Spondyloarthritis Disease Activity Index in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry

OBJECTIVE: To validate the Juvenile Spondyloarthritis Disease Activity (JSpADA) index, and modified versions thereof, in a North American cohort of patients with enthesitis-related arthritis (ERA). METHODS: We utilized the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry database ERA cohort to validate the JSpADA and its modifications (JSpADA6-no Schober, no CRP/ESR; JSpADA7- no Schober; and JSpADA7- no CRP/ESR) using OMERACT principles of face validity, discriminative validity, and responsiveness to change. RESULTS: There were 51 subjects (64 visits) with complete JSpADA data with a mean age of 13.7 years and disease duration of 30.9 months. Subjects were predominantly white (84.3%) with half of them, male (56.9%), and HLA-B27 positive (50%). The JSpADA showed high correlation with the cJADAS-10 (r=0.81), moderate-to-high correlation with physician global (r=0.69), and low-to-fair correlation with CHAQ (0.22). The modifications of JSpADA (JSpADA7- no Schober, JSpADA7-no CRP/ESR, and JSpADA6 - no Schober, no CRP/ESR) performed similarly with high correlation with cJADAS-10 (r=0.81, 0.79, and 0.80, respectively), moderateto- high correlation with physician global (r=0.65, 0.67, 0.64), and low-to-fair correlation with CHAQ (r= 0.35, 0.34, 0.39). All modified versions of JSpADA had good responsiveness to change. All versions of JSpADA had excellent discriminative validity. CONCLUSION: We propose the term modified JSpADA for the modification of JSpADA with 6 elements (JSpADA6 - no Schober, no CRP/ESR). This shorter disease activity index may improve implementation of JSpADA in both clinical practice and research trials