History Of Juvenile Rheumatoid Arthritis Research Articles

Mesenteric Lymphadenopathy: a Rare Case of Rosai-Dorfman Disease

Introduction: 
 Rosai-Dorfman Disease (RDD) is rare with approximately 100 new cases annually in the United States and a mean age of 20.6 years [1]. RDD is characterized by massive lymphadenopathy and sinus histiocytosis [1]. Bilateral cervical lymphadenopathy is the typical presentation, however extra nodal sites have been noted [1]. This case discusses manifestations of a rare disease state. 
 Case Presentation: 
 A 19 year old male with a history of eczema and juvenile rheumatoid arthritis presented to the emergency room with four days of diffuse abdominal pain localized to the left upper quadrant, radiation to the right lateral ribs with nausea and diarrhea. He reported an unintentional weight loss of 30 pounds in the last three months. Physical exam revealed generalized abdominal tenderness and rebound. Computed tomography of the abdomen and pelvis (CTAP) along with routine labs were ordered. CTAP showed retroperitoneal lymphadenopathy up to two centimeters in the short axis (figure 1) and mesenteric adenopathy with a 15 millimeter lymph node in the right lower quadrant. Histopathology reported necrotizing granulomatous lymphadenitis with benign sinus histiocytes. Referral to rheumatology was made and treatment was initiated with prednisone 20 milligrams daily with plans for repeat abdominal imaging to evaluate for reduction of adenopathy. 
 Discussion: 
 RDD coexists with immunologic disease in 10% of cases.3 It has been associated with systemic lupus erythematous, idiopathic juvenile arthritis, autoimmune hemolytic anemia,24 and one case of RAS-associated autoimmune leukoproliferative disease [1]. The prognosis for RDD is indolent, 50% of patients experiencing resolution, one third with residual asymptomatic adenopathy and 17% with persistent symptomatology [2]. Our case highlights our patient’s associated history of juvenile rheumatoid arthritis now presenting with histological findings of RDD. A methodical approach to assessing patients with diffuse abdominal pain and ensured collaboration amongst different medical specialists will ensure favorable treatment outcomes.

Disseminated herpes zoster with mucosal involvement in an immunosuppressed child.

To the editor: We report the case of a 14-year-old boy who presented with some painful, confluent, and erythematous vesicles on the left lumbosacral side, with a 2-day history of fever, severe headache, and fatigue (Figure 1A). There were no convulsions, abdominal pain, or diarrhea. Over the next 5 days, the skin lesions progressed to scattered vesicles throughout the face, trunk, extremities, and oral mucosa (Figure 1B). The boy had a medical history of juvenile rheumatoid arthritis for 7 years and was taking prednisone and thalidomide. He had experienced chicken pox before. The level of C-reactive protein was 71 mg/L (normal range, <8 mg/L), and the immunoglobulin M antibody against varicella zoster virus (VZV) was positive. A biopsy of the lumbosacral lesion revealed the vesicle ballooning degeneration of intraepidermal keratinocyte, and wedge necrosis of the epidermis and dermis (Figure 1C). Localized lymphocytes and sporadic neutrophils, histiocytes, and plasma cells infiltrated the perivascular area in the superficial dermis. The final diagnosis was disseminated herpes zoster (HZ). The patient was treated with intravenous acyclovir for 2 weeks. The vesicles dried up after 7 days, and the skin lesions were resolved after 2 weeks. Disseminated HZ is a serious complication of VZV infection, which may be related to increased risk and potentially fatal complications.1 This almost always happens in immunocompromised people who present with more than 20 vesicles outside the area of primary or adjacent dermatomes, and where mucous membranes are involved. Disseminated HZ may also occur during medication, including with biological agents and small molecule drugs.2, 3 This patient was immunocompromised owing to the long-term use of prednisone. To summarize the mucosal involvement of HZ, a literature review was performed at PubMed from inception to April 15, 2022. The search strategy included “herpes zoster” AND (“mucosa” OR “mucous membrane”) AND “case”; only English-language studies were included. Thirty-one references with 34 cases were ultimately included. The results of mucosal involvement and complications are listed in Table S1. The reported mucosal involvement included oral, tongue, gingiva, pharyngeal, larynx, esophagus, gastric area, intestine, and ears. Oral mucosal involvement was common in patients with Ramsay Hunt syndrome (RHS).4 Most reported cases with mucosal involvement were adults. Most patients recovered after antiviral treatment but several patients died because of complications and organ failure. Systemic treatment with acyclovir and famciclovir can reduce the duration of skin lesions.5 In this case, the patient was a child without RHS and the lesions were resolved after acyclovir treatment without any complications with post-herpetic neuralgia. In summary, disseminated HZ, as a severe form of HZ, should not be ignored. Both the skin and mucosa can be involved, and early diagnosis and antiviral treatment are important to shorten the duration of the disease and improve the prognosis. This work was supported by Beijing Natural Science Foundation (No. 7222058). Informed consent was obtained from the patient's parent. The authors declare that they have no conflicts of interest. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

Complication after the reconstruction of the old patellar tendon rupture

Introduction. Chronic patellar tendon rupture (PTR) occurs rarely; its frequency and prevalence are unknown. There are very little data on the late patellar tendon reconstruction in rheumatoid arthritis and its complications. Case report. We presented a surgical repair of a PTR with early postoperative rupture of the contralateral patellar tendon for a 21-year-old woman with a past medical history of juvenile rheumatoid arthritis (treated with corticosteroids) who sustained initial injury 11 months prior to the presentation. The contralateral side was used for autograft harvesting. We used bone-tendon-bone (BTB) autograft and allografts followed by double-wire loop reinforcement and immediate postoperative mobilization. The patient was followed for 2 years, and the function of both knees was re-stored completely, with a full active range of motion. In this case, reconstruction of an 11-month-old chronic PTR (with complete resorption of the tendon and completely separated infrapatellar pads, complicated by the contralateral PTR) with BTB autograft and allografts and double wire loop reinforcement gave an excellent functional result. Two years after the surgical treatment, the extensor function of both knees was completely restored with a full range of movements. The patient reported satisfying outcomes and was able to return to all pre-injury activities without the assistance of orthopedic devices. Conclusion. This case report highlights the importance of the early diagnosis and describes operative techniques used in chronic PTR repair and treatments of the early postoperative complications such as rupture of the contralateral tendon.

Bezold Abscess in a patient with Juvenile Rheumatoid Arthritis

A Bezold abscess is a rare complication of acute otitis media and mastoiditis which is characterized by the collection of pus in the sub-muscular spaces from the mastoid cavity. A 27-year-old female with history of Juvenile Rheumatoid Arthritis presented with right ear discharge and post auricular swelling extending along the sternocleidomastoid muscle. A Bezold abscess was diagnosed upon review of the patient’s CT scan. She subsequently underwent incision and drainage of the abscess in addition to a cortical mastoidectomy. Although the development of intra and extra axial complications of acute otitis media are very rare in the post antibiotic and vaccination era, it should be noted that a compromised immune system is a significant risk factor for this complication.

Juvenile rheumatoid arthritis, endocrine arthropathy or hypermobility syndrome?

Оbjective. Evaluation of the frequency of detection of various associated diseases, including endocrine pathology in patients with a history of juvenile rheumatoid arthritis (JRA), which could affect the formation of joint syndrome in adulthood. An important task of the doctor-internist is the ability to differentiate articular syndrome in rheumatic arthropathies from joint damage due to many endocrine disorders, accompanied by a similar clinical picture.Materials and methods. 64 adult patients with history of JRA were examined in a rheumatological department of Alexander Hospital, Kyiv. Particular attention was paid to combination of rheumatic and endocrine diseases that may be accompanied by articular syndrome.Results and discussion. In 12.5% of patients with JRA in anamnesis endocrine pathology was found, in whom 4,7% of patients pituitary pathology was observed, including pituitary adenoma.Conclusions. The combination of rare diseases such as pituitary adenoma and JRA needs further study the impact of these diseases on the development of articular syndrome.

A case of bovine valve endocarditis caused byEngyodontium album

We report the first case of Engyodontium album bioprosthetic valve endocarditis in a 44-year-old male with a history of juvenile rheumatoid arthritis. There is only one other report of Engyodontium album as a human pathogen. The present case supports the increased incidence of fungal endocarditis especially in patients receiving immunotherapy.

Protrusio of a Ceramic Femoral Head Through the Acetabular Metallic Shell, Extensive Metallosis and ‘Bubble Sign’

A 24-year-old patient with a history of juvenile rheumatoid arthritis underwent a primary cementless left total hip arthroplasty (THA). The original THA consisted of an Optifix 54 cup with a 3-mm thick polyethylene liner, an Optifix size 4 stem (Smith & Nephew Richards, Memphis, Tennessee) and a Biolox aluminum 32-mm femoral head. Fourteen years later, radiographs demonstrated extensive wear of the polyethylene liner resulting in direct articulation and abrasion wear of the ceramic femoral head on the cup and a bubble sign. This article presents a case of a catastrophic failure of a ceramic/polyethylene bearing with destruction of the polyethylene liner and the metallic shell and protrusio of the nonfractured ceramic head through the metallic shell. To our knowledge this is the first description of extensive metallosis and subsequent radiograph bubble sign not presenting as a result of wear of a metal-on-metal articulation. At the time of revision surgery-Hydrocel TNT Monoblock 58 cup (Zimmer, Warsaw, Indiana), Wagner 265/14 stem (Zimmer), and a Co/Cr 28-mm head-copious metallic debris was seen both macroscopically and histologically, with the ceramic head protruding behind the metallic shell. Multiple factors may have been responsible for this failure including a thin polyethylene shell, a suboptimal locking mechanism, gamma in air sterilization for polyethylene, multiple screw-holes that reduce the contact surface between shell and polyethylene, the rough surface on the inside of the shell and non-articular wear at the metal polyethylene interface within the acetabular component and the high demands of this active young patient.

Quantifying dynamic contrast‐enhanced MRI of the knee in children with juvenile rheumatoid arthritis using an arterial input function (AIF) extracted from popliteal artery enhancement, and the effect of the choice of the AIF on the kinetic parameters

Quantification of dynamic contrast-enhanced (DCE) MRI based on pharmacokinetic modeling requires specification of the arterial input function (AIF). A full representation of the plasma concentration data, including the initial rise and decay parts, considering the delay and dispersion of the bolus contrast is important. This work deals with modeling of DCE-MRI data from the knees of children with a history of juvenile rheumatoid arthritis (JRA) by using an AIF extracted from the signal enhancement data from the nearby popliteal artery. Three models for the AIFs were considered: a triexponential (AIF1), a gamma-variate plus a biexponential (AIF2), and a biexponential (AIF3). The pharmacokinetic parameters obtained from the model were Ktrans', kep, and V'p. The results from AIF1 and AIF2 showed no statistically significant difference. However, some statistically significant differences were seen with AIF3, particularly for parameters Ktrans' and V'p in the synovium (SNVM). These results suggest the importance of obtaining an appropriate AIF representation in pharmacokinetic modeling of JRA. Specifically, the initial rising part of the AIF should be incorporated for optimal pharmacokinetic modeling results. The pharmacokinetic parameters (mean+/-SD) derived from AIF1, using the average plasma concentration data, were as follows: SNVM Ktrans'(min-1)=0.52+/-0.34, kep(min-1)=0.71+/-0.39, and V'p=0.33+/-0.16, and for the distal femoral physis (DFP) Ktrans'(min-1)=1.83+/-1.78, kep(min-1)=2.65+/-1.80, and V'p=0.46+/-0.31. The pharmacokinetic parameters in the SNVM may be useful for investigating activity and therapeutic efficacy in studies of JRA. Longitudinal studies are necessary to find or demonstrate the parameter that is more sensitive to disease activity.

Increased mortality in adults with a history of juvenile rheumatoid arthritis: A population‐based study

Increased mortality in adults with a history of juvenile rheumatoid arthritis: A population‐based study

Increased mortality in adults with a history of juvenile rheumatoid arthritis: a population-based study.

To assess mortality in a population-based cohort of adults with a history of juvenile rheumatoid arthritis (JRA). The Rochester Epidemiology Project database was used to identify all cases of JRA diagnosed among Rochester, Minnesota residents under the age of 16 between January 1, 1960 and December 31, 1993. Fifty-seven patients in this cohort are now adults (ages 18-53 years, mean age 34.3 years), and this subgroup was contacted for a long-term followup study. The average length of followup from the time of diagnosis was 25.6 years. Four deaths occurred in this cohort of 57 adults with a history of JRA. All 4 deceased patients had other autoimmune illnesses and died of complications of these diseases. The observed frequency of 4 deaths was significantly greater (P < 0.0026 by one-sample log-rank test) than the 1 death that would be expected among Minnesota whites of similar age and sex, and corresponds to a mortality rate of 0.27 deaths per 100 years of patient followup compared with an expected mortality rate of 0.068 deaths per 100 years of followup in the general population. The results indicate a significant, unexpected increase in mortality in this population-based cohort of adults with a history of JRA in comparison with the rate in the general population. The deaths in this group were all associated with other autoimmune disorders, suggesting that special emphasis should be given to the diagnosis and treatment of other autoimmune diseases, including immunodeficiencies, in JRA patients. The frequency of deaths in this cohort suggests that JRA patients are at substantial risk for mortality, and highlights the need for longitudinal followup and care into adulthood.

Intraocular recombinant tissue-plasminogen activator fibrinolysis of fibrin formation after cataract surgery in children

Purpose: To evaluate the efficacy and safety of intracameral recombinant tissueplasminogen activator (rt-PA) application for fibrinolysis of fibrin formation after cataract surgery in children. Setting: Johann Wolfgang Goethe-University, Department of Ophthalmology; Frankfurt am Main, Germany. Methods: This study comprised 11 eyes of 10 patients aged 3 to 13 years (mean7.2 ± 3.68 [SD]) who developed severe fibrin formation after cataract surgery and IOL implantation despite intensive topical steroid therapy. Under general anesthesia, fibrinolysis was performed with 10 μg of rt-PA 7.18 ± 2.04 days after intraocular surgery. Follow-up included slitlamp examination, tonometry, visual acuity testing, and ophthalmoscopy. Anterior chamber flare measurements could be performed in 6 eyes. Results: Complete resolution of fibrin formations occurred in 90% of the patients. Inthese cases, no recurrent fibrinous reaction or adverse effects were noted. In 2 eyes of the same patient with a history of juvenile rheumatoid arthritis and chronic uveitis, fibrin clot dissolution was incomplete. A recurrent fibrinous formation could be observed after 2 and 4 weeks, respectively. A beginning band kenatopathy excluding the central and limbal cornea, was noted after 6 and 8 weeks, respectively. Conclusion: Intraocular application of rt-PA 'appears to be a safe and efficacious therapeutic approach in the management of severe fibrinous reactions after pediatric cataract surgery.

Central nervous system involvement in amyloid A type amyloidosis

We report a 38-year-old male with recurrent cerebral infarction, who had a history of juvenile rheumatoid arthritis and suffered from ischemic necrosis of the jejunum at the age of 34 years. When the patient showed gastrointestinal symptoms, amyloid deposits were found with colon biopsy. The patient experienced recurrent episodes of headache and left homonymous lower quadrantanopsia at the age of 38 years. CT and MRI of the head revealed cerebral infarction in the right parieto-occipital region. At this time, amyloid deposits were detected in the mucosa of the stomach, duodenum, and rectum. Immunostaining revealed that amyloid fibrils were composed of amyloid A (AA) protein. It is possible that AA type amyloidosis may be of importance in the etiology of cerebral infarction in the young adult.

Circulating nucleated red blood cells following splenectomy in a patient with congenital dyserythropoietic anemia

A 24-year-old white female with a 10-year history of juvenile rheumatoid arthritis and splenomegaly developed numerous circulating nucleated red blood cells (NRBC) following splenectomy for chronic abdominal pain. Subsequent evaluation revealed the presence of a congenital dyserythropoietic anemia (CDA) with atypical features. Circulating NRBCs have been reported following splenectomy in three other cases of CDA, each of which had atypical features and did not fit into the customary classification of types I-IV. Follow-up of our patient at 4 years revealed no untoward consequences of persistent NRBCs in her circulation.

Pregnancy in patients with a history of juvenile rheumatoid arthritis

The present investigation was undertaken to study the relationship between pregnancy and juvenile rheumatoid arthritis (JRA), with regard to possible reactivation of the disease, complications at delivery, and postpartum complications. Data on 76 pregnancies in 51 JRA patients were collected retrospectively. Comparison of pre-pregnancy disease activity with the course during gestation showed that pregnancy did not cause reactivation of the symptoms of quiescent JRA. Patients with minor symptoms at conception and the majority of those with active inflammation experienced improvement or total remission in the second half of gestation. Four JRA patients with active anterior uveitis had active eye disease during pregnancy also. Seventy-four of the pregnancies resulted in births of infants that were healthy and of normal birth weight; 1 infant had low birth weight and 1 was stillborn. Twenty children were delivered by cesarean section, and in 15 cases this was related to sequelae of JRA. A flare 3-6 months postpartum was reported after 45 pregnancies. However, in none of the patients whose disease was quiescent before or during pregnancy did this cause permanent reactivation of the JRA. Comparison of these 51 patients with 45 age-matched female patients without children revealed that disease severity and functional impairment were the limiting factors in the decision for or against having children.

Granuloma Annulare and Gold Therapy

To the Editor.— Dermatologic lesions occur frequently in the course of gold therapy for rheumatoid arthritis. Many are transient and may resolve despite continuation of therapy. Of those that can be classified, the majority resemble eczema, lichen planus, or pityriasis rosea. 1 Our patient, a 17-year-old girl with a one-year history of juvenile rheumatoid arthritis, acquired asymptomatic papular lesions on the flexor and extensor surfaces of several digits, and on the extensor surfaces of the elbows and knees, within five days of the institution of gold sodium thiomalate therapy (25 mg weekly). The lesions were shiny, 2- to 3-mm papules arranged primarily in annular fashion, but also in linear configurations. On biopsy, they were characterized by some necrobiosis of dermal collagen outlined by prominent patchy zones of chronic inflammation with lymphocytes and histiocytes as the dominant cell types. There was also an increased cellularity in the adjacent dermal connective