Tokyo Metropolitan Children’s Medical Center opened a specialized and certified nurse-led outpatient clinic in November 2021 to provide nursing care that meets the needs of pediatric patients and their families. The nurse-led outpatient clinic for patients and families with pediatric rheumatic diseases objectively views medical treatment from the perspective of health promotion, identifies individual needs, and provides support so that they can undergo treatment life with peace of mind. In addition, in order to provide better care for patients and their families, we collaborate with other professions and practice nursing in close cooperation with them. Therefore, I present two cases of patients with juvenile idiopathic arthritis in the specialized and certified nurse-led outpatient clinic and report my practice that meet the needs of patients and their families. I also consider the issues facing the coordination of other professions at the outpatient clinic and the improvement of the quality and expansion of the role of the outpatient clinic.

Polyarteritis nodosa (PAN) is one of diseases which is difficult to diagnose, because it causes inflammation of medium arteries in multiple organs and produces a variety of symptoms. A 12-year-old boy was referred to our hospital, because he had a fever lasting seven days, weight loss and a markedly increased level of inflammatory reaction. Echocardiography performed on day 13 of illness revealed coronary artery dilatation, and he was diagnosed with atypical Kawasaki disease (KD) although no major symptoms other than fever and coronary artery lesions were observed. Predicted high-dose intravenous immunoglobulin (IVIG) therapy refractory, steroid was administrated in combination with IVIG followed by good response. Oral steroids were discontinued after administration for 17 days, but he had fever and increased inflammatory reaction again from the next day and we judged that KD had recurred. IVIG and ulinastatin were administered, and infliximab was further delivered to obtain pyretolysis, but the inflammatory reaction was not completely negative. Ultimately, he was diagnosed with PAN by FDG PET/CT imaging showing vasculitis of bilateral femoral arteritis, and cyclophosphamide pulse therapy was used in combination with steroid administration. Some cases with an atypical course of KD may require differentiation from other vasculitis syndromes such as PAN, and FDG PET/CT scans may be useful if the diagnosis cannot be made by CT angiography or other imaging tests.

Systemic lupus erythematosus (SLE) is an autoimmune disease that can cause complications in various organs. Protein losing enteropathy (PLE) is a disease that presents with generalized edema, abdominal symptoms, and hypoproteinemia due to protein leakage from the intestinal tract. SLE is rarely complicated by PLE, which is diagnosed Lupus associated protein losing enteropathy (LUPLE). We report a case of a child with LUPLE. The patient is an 11-year-old girl, who had periorbital and leg edema, but no abdominal symptoms or fever. Blood tests showed high antinuclear antibody titers, hypoproteinemia, hypoalbuminemia, hypocomplementemia, and high anti-SS-A antibody titers. Urine test showed urine protein was negative. Albumin scintigraphy indicated intestinal accumulation and increased clearance of α1-antitrypsin in stool was observed, leading to the diagnosis of PLE. On the other hand, a renal biopsy revealed Class II lupus nephritis, which suggested SLE. Based on the above results, we diagnosed her with LUPLE. Lupus enteritis and CD55 deficiency with hyperactivation of complement, angiopathic thrombosis, and PLE (CHAPLE) syndrome, which is associated with hypocomplementemia and PLE, were ruled out. We collected 13 cases of childhood-onset LUPLE, from the literature in addition to the present case, and compared them to systematic reviews of adult-onset LUPLE, concluding that childhood-onset LUPLE and adult-onset one share similar clinical characteristics. It should be noted that PLE can be a complication of SLE as well as the first manifestation of SLE.

We report the case of a 13-year-old girl with Takayasu arteritis who presented with a headache and loss of pulse without fever. At the age of 10 years, she developed a headache with visual field narrowing. At the age of 13 years, she was referred to our hospital because her blood pressure could not be measured in her right upper arm and there was no pulse in the right radial artery. Cervical echocardiography showed 98% stenosis of the right common carotid artery. Contrast-enhanced Computed Tomography(CT) of the head to pelvis showed circumferential wall thickening and increased periprosthetic fatty tissue density from the aortic arch to the proximal portion of the descending aorta, bilateral subclavian, and bilateral common carotid arteries. Positron Emission Tomography/CT showed increased wall thickening of the brachiocephalic artery, both common carotid arteries, and both subclavian arteries from the aortic arch. She was diagnosed with Takayasu arteritis with a type IIB vascular involvement pattern and treated with steroids, azathioprine, and tocilizumab. Her headache gradually decreased and blood pressure in the right upper arm became measurable. In pediatric patients with Takayasu arteritis who do not present with fever, the syndrome should be suspected based on other nonspecific symptoms. Furthermore, aggressive imaging and other diagnostic procedures should be initiated when a left-right difference in blood pressure or absent of wrist pulses is observed.

Systemic juvenile idiopathic arthritis (sJIA) is an autoinflammatory disease characterized by recurrent systemic inflammation, where the overproduction of cytokines, including interleukin (IL)-6 and -18. Tocilizumab (TCZ) is an effective IL-6 inhibitor for treating sJIA. Because TCZ masks signs of macrophage activating syndrome (MAS), such as fever and elevated serum ferritin levels, making MAS development less likely to be recognized, the timing of TCZ introduction requires careful consideration. We recently encountered two cases of sJIA where TCZ was introduced after achieving disease control through various treatments, including plasma exchange. In Case 1, a 5-year-old girl experienced persistent fever despite three courses of intravenous methylprednisolone pulse therapy (IVMP). We were concerned about MAS development due to decreasing platelet levels and a high IL-18 level of 92,200 pg/mL. TCZ was introduced after observing a decrease in IL-18 levels, following plasma exchange and administration of cyclosporin A (CyA). This approach resulted in success in controlling the disease. In Case 2, a 12-year-old girl exhibited thrombocytopenia during IVMP and was suspected to have MAS. TCZ was introduced after plasma exchange treatment, CyA, and dexamethasone palmitate. Following TCZ administration, this approach also resulted in success in controlling the disease. To avoid complications or exacerbations of MAS, it is essential to promptly evaluate platelet counts, IL-18, and ferritin levels, and initiating plasma exchange could be a treatment option.

In children with childhood-onset primary Sjögren’s syndrome (pSS), extra glandular manifestations such as fever, arthralgia, skin rash, and cervical lymphadenopathy frequently contribute to the diagnosis of pSS. We have experienced 15-year-old twin girls with pSS, who exhibited febrile cervical lymphadenitis from infancy, which was indicative of an extra-glandular manifestation of pSS. The patients did not present with sicca symptoms and were initially suspected of having subacute necrotizing lymphadenitis（SNL） due to recurrent febrile cervical lymphadenitis complicated with mild leukopenia. However, a cervical lymph node biopsy performed on one of the twins did not reveal the necrotizing lymphadenitis characteristic of SNL. Given the suspicion of an autoimmune disorder, both patients were tested and found positive for anti-SS-A/B antibodies, leading a diagnosis of pSS. A second review of the histological findings showed an increase in the number of lymph follicles and enlargement of the paracortex, which are characteristic findings observed in autoimmune diseases. Recurrent or persistent cervical lymphadenitis should be considered a potential indicator of autoimmune disease, as demonstrated in this case where pathological findings facilitated the diagnosis of pSS.