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To evaluate the proportion of children with juvenile idiopathic arthritis (JIA) who met criteria for comorbid juvenile fibromyalgia (FM) using the Pain and Symptom Assessment Tool (PSAT), and to identify clinical and demographic differences among JIA patients with and without juvenile FM. Patients ages 11–17 years with JIA were recruited from 4 North American pediatric rheumatology centers. Each patient completed the PSAT. Additional clinical and disease activity measures included pain visual analog scale, patient global assessment of disease activity (PtGA) and physician global assessment of disease activity (PhGA), the Functional Disability Inventory (FDI), and the Pain Catastrophizing Scale in children. Of 129 patients, 11 met criteria for juvenile FM. FDI scores were markedly higher in patients who tested positive for juvenile FM, with a mean of 24.8 compared to 6.9 in patients without juvenile FM (P< 0.001). Pain catastrophizing scores were also significantly higher, by ~14 points, in patients with juvenile FM. There was a significant tendency for patients to give higher disease activity scores than physicians, which was more marked among patients with juvenile FM. In patients with juvenile FM, PtGA scores exceeded PhGA scores by a mean of 3.7, compared to a mean of 0.7 among patients without juvenile FM (P< 0.001). A minority of JIA patients (8.5%) met criteria for juvenile FM. This group demonstrated markedly more functional impairment. PtGA scores were strikingly higher than PhGA scores among patients with JIA who met juvenile FM criteria, suggesting that providers might consider a more expansive approach to chronic pain and non‐musculoskeletal symptom assessment and treatment in JIA patients.

Autoimmune inner ear disease (AIED) is a rare, but treatable cause of sudden sensorineural hearing loss in children. Most cases present acutely and involve both ears. The precise mechanism of hearing loss in AIED is not known. Many suspected etiologies have been proposed including infections, vascular abnormalities, and trauma. However, 70% of cases are defined as idiopathic. There are no standardized diagnostic criteria for AIED, and the diagnostic process may be challenging. Positive auto antibodies and response to immunosuppressive therapy support the diagnosis. Treatment may include corticosteroids and steroid-sparing immunosuppressive medications. A high index of suspicion is recommended as the hearing loss may be stabilized or even reversed with early treatment. Long-term medical treatment failures generally have good outcomes with cochlear implantation. Autoimmune inner ear disease (AIED) is a rare, but treatable cause of sudden sensorineural hearing loss in children. Most cases present acutely and involve both ears. The precise mechanism of hearing loss in AIED is not known. Many suspected etiologies have been proposed including infections, vascular abnormalities, and trauma. However, 70% of cases are defined as idiopathic. There are no standardized diagnostic criteria for AIED, and the diagnostic process may be challenging. Positive auto antibodies and response to immunosuppressive therapy support the diagnosis. Treatment may include corticosteroids and steroid-sparing immunosuppressive medications. A high index of suspicion is recommended as the hearing loss may be stabilized or even reversed with early treatment. Long-term medical treatment failures generally have good outcomes with cochlear implantation. [[Pediatr Ann.2019;48(10):e391–e394.]

Mucosal-associated lymphoid tissue (MALT) lymphoma is rare in the pediatric population, but primary Sjogren syndrome is a well-established risk factor for this malignancy. This report describes 2 cases of MALT lymphoma in children with Sjogren syndrome. A 15-year-old girl developed MALT lymphoma of the parotid gland as the presenting symptom of Sjogren syndrome. In the second case, a 15-year-old boy with known Sjogren syndrome presenting mainly with arthritis was diagnosed with MALT lymphoma, also of the parotid gland. With early diagnosis and treatment, outcomes in pediatric MALT lymphoma are generally encouraging. Pediatric oncology specialists should be aware of the association of MALT lymphoma with Sjogren syndrome and have a high index of suspicion for this malignant complication.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome characterized by a dysregulated hyperinflammatory response associated with aberrant activation of lymphocytes and macrophages that results in hypercytokinemia. It is classically divided into two types: (1) primary or familial HLH and (2) secondary HLH. Familial HLH is generally an autosomal recessive condition, whereas secondary HLH is usually associated with infectious diseases, autoinflammatory and autoimmune diseases (where it is more commonly known as macrophage activation syndrome), malignancy, immunosuppression, hematopoietic stem cell transplantation, organ transplantation, HIV infection, and metabolic diseases. Although its clinical presentation is often similar to bacterial sepsis or systemic inflammatory response syndrome, HLH can be life-threatening. As such, it is imperative to recognize and diagnose HLH in a timely manner to optimize care. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome characterized by a dysregulated hyperinflammatory response associated with aberrant activation of lymphocytes and macrophages that results in hypercytokinemia. It is classically divided into two types: (1) primary or familial HLH and (2) secondary HLH. Familial HLH is generally an autosomal recessive condition, whereas secondary HLH is usually associated with infectious diseases, autoinflammatory and autoimmune diseases (where it is more commonly known as macrophage activation syndrome), malignancy, immunosuppression, hematopoietic stem cell transplantation, organ transplantation, HIV infection, and metabolic diseases. Although its clinical presentation is often similar to bacterial sepsis or systemic inflammatory response syndrome, HLH can be life-threatening. As such, it is imperative to recognize and diagnose HLH in a timely manner to optimize care. [[Pediatr Ann.2017;46(8):e309–e313.]

A 14-year-old boy presented with months of severe widespread musculoskeletal pain. He was profoundly fatigued and unable to attend school. Laboratory evaluation, including complete blood count, comprehensive metabolic panel, inflammatory markers, and thyroid function, was unrevealing. Physical examination was also normal except for multiple tender points. The patient was diagnosed with juvenile primary fibromyalgia syndrome and referred for multidisciplinary treatment including physical therapy, exercise, and counseling, and his daily functioning gradually improves. Juvenile fibromyalgia is a complex syndrome that often severely limits patients’ activities and can impede normal adolescent development. Effective treatment requires an understanding of the biologic, psychologic, and social factors contributing to the perpetuation of chronic pain. The author reviews the diagnostic criteria, pathophysiology, and treatment of juvenile fibromyalgia. Medications, particularly antidepressants and anticonvulsants, can be useful adjuncts to therapy. However, multimodal pain management including intensive physical therapy, exercise, counseling, and sleep hygiene is most effective in treating fibromyalgiaA 14-year-old boy presented with months of severe widespread musculoskeletal pain. He was profoundly fatigued and unable to attend school. Laboratory evaluation, including complete blood count, comprehensive metabolic panel, inflammatory markers, and thyroid function, was unrevealing. Physical examination was also normal except for multiple tender points. The patient was diagnosed with juvenile primary fibromyalgia syndrome and referred for multidisciplinary treatment including physical therapy, exercise, and counseling, and his daily functioning gradually improves. Juvenile fibromyalgia is a complex syndrome that often severely limits patients’ activities and can impede normal adolescent development. Effective treatment requires an understanding of the biologic, psychologic, and social factors contributing to the perpetuation of chronic pain. The author reviews the diagnostic criteria, pathophysiology, and treatment of juvenile fibromyalgia. Medications, particularly antidepressants and anticonvulsants, can be useful adjuncts to therapy. However, multimodal pain management including intensive physical therapy, exercise, counseling, and sleep hygiene is most effective in treating fibromyalgia. [. [Pediatr Ann. 2015;44(6):e136–e141.]

A 15-year-old girl presented with knee pain, associated with a positive antinuclear antibody (ANA). She denied joint swelling or morning stiffness and remained physically active despite the pain. A physical examination was unremarkable except for articular hypermobility. Laboratory results were also unremarkable. Therefore, the positive ANA was determined to be nonspecific, and not concerning. In the evaluation of children with musculoskeletal complaints, unusual rash, or fatigue, an ANA assessment is frequently considered. When is this test most likely to be useful? What is the appropriate follow up for a positive result? Which results are concerning for an autoimmune process? This article reviews the literature to address these practical concerns. Understanding the indications for ordering an ANA, and the correct interpretation of a positive ANA, may reduce unnecessary referrals and costly tests. Moreover, the misperception that a positive ANA indicates a rheumatologic disease can cause significant patient and parental anxiety. A 15-year-old girl presented with knee pain, associated with a positive antinuclear antibody (ANA). She denied joint swelling or morning stiffness and remained physically active despite the pain. A physical examination was unremarkable except for articular hypermobility. Laboratory results were also unremarkable. Therefore, the positive ANA was determined to be nonspecific, and not concerning. In the evaluation of children with musculoskeletal complaints, unusual rash, or fatigue, an ANA assessment is frequently considered. When is this test most likely to be useful? What is the appropriate follow up for a positive result? Which results are concerning for an autoimmune process? This article reviews the literature to address these practical concerns. Understanding the indications for ordering an ANA, and the correct interpretation of a positive ANA, may reduce unnecessary referrals and costly tests. Moreover, the misperception that a positive ANA indicates a rheumatologic disease can cause significant patient and parental anxiety. [[Pediatr Ann. 2015;44(6):e131–e135.]