Kawasaki Disease: an Update.

PURPOSE OF REVIEW: Provide the most recent updates on the epidemiology, pathogenesis, and treatment advances in Kawasaki disease. RECENT FINDINGS: Treatment advances in complex, IVIG-refractory cases of Kawasaki disease. Multisystem inflammatory syndrome, a newly reported inflammatory condition with Kawasaki-like features and an association with the 2019 Coronavirus (COVID-19). Kawasaki disease (KD) is a rare systemic inflammatory disease that predominately affects children less than 5 years of age. Pathogenesis of KD remains unknown; the leading theory is that an unknown stimulus triggers an immune-mediated inflammatory cascade in a genetically susceptible child. Classic KD is a clinical diagnosis based on set criteria and excluding other similar clinical entities. Patients who do not fulfill complete diagnostic criteria for KD are often referred to as atypical (or incomplete) KD. The most feared complication of KD is coronary artery abnormality development, and patients with atypical KD are also at risk. Administration of intravenous immunoglobulin (IVIG) and aspirin has greatly reduced the incidence of coronary lesions in affected children. Several other immune-modulating therapies have recently been utilized in complex or refractory cases.

Disability in Children and Adolescents With Irritable Bowel Syndrome and/or Fibromyalgia.

To compare disability and emotional health in individuals with irritable bowel syndrome (IBS), fibromyalgia, or both, patients completed the Questionnaire on Pediatric Gastrointestinal Symptoms-Rome III, childhood Functional Disability Inventory (FDI), and the Behavior Assessment System for Children, Second Edition. Patients' (age range 8-18 years, 19 IBS, 12 fibromyalgia, and 12 both) FDI scores showed greater disability than scores from historically healthy patients. Fibromyalgia (FDI 22.5 ±â€Š12.7, P = 0.018) and patients with both (FDI 26.2 ±â€Š13.8, P = 0.001) averaged greater disability than those with IBS (FDI 10.6 ±â€Š7.9). Disability was correlated with anxiety and depression symptoms. Disability and psychological symptoms are important when evaluating individuals with fibromyalgia and IBS.

Kawasaki disease: pathophysiology, clinical manifestations, and management.

Kawasaki Disease, a systemic vasculitis of unknown origin with specific predilection for the coronary arteries, is the most common cause of childhood-acquired heart disease in western countries. Despite its world-wide incidence, the pathophysiology of this enigmatic disease is still under investigation. Diagnosis is made on a clinical basis, with supportive laboratory evidence and imaging. Once identified, timely initiation of treatment is imperative in order to quell the inflammatory response and decrease the incidence of long-term sequelae, specifically coronary artery aneurysms. Finally, longitudinal follow-up should be implemented based on risk stratification and individualized to each patient.

Recurrence of granulomatous interstitial nephritis in transplanted kidney.

Sarcoidosis is a multisystemic disease of unknown etiology. Minor renal involvement is not rare but kidney failure is uncommon and only rare cases of recurrent disease in a kidney transplant have been published. We report a patient who at age 10 yr developed ESRD secondary to renal sarcoidosis with GIN. Her disease subsequently recurred in the transplanted kidney despite standard immunosuppression with prednisone, tacrolimus, and mycophenolate mofetil. The recurrent disease appeared to respond to increased immunosuppression, which included infliximab. However, the patient died of disseminated histoplasmosis three yr post-transplant.

Successful emergent coronary thrombolysis in a neonate with Kawasaki's disease.

This report describes the case of a 29-day-old infant with Kawasaki's disease who presented in extremis with multiple coronary aneurysms and coronary thromboses, myocardial ischemia, and congestive heart failure. The infant successfully underwent emergent coronary thrombolysis with tissue plasminogen activator.

Familial Mediterranean fever presenting as anti-cyclic citrullinated peptide antibody negative palindromic rheumatism.

A 64-year-old Egyptian man who resides in the United States presented to the rheumatology clinic with 6 months history of episodic recurrent pauci--arthritis along with constitutional symptoms. His Mediterranean ancestry, anti-cyclic citrullinated peptide negativity, and cyclical palindromic rheumatism prompted an investigation for familial Mediterranean fever gene mutation. He was found to have heterozygous 694I gene mutation during MEFV analysis. He also met Liveneh 1 major and 1 minor criteria for the diagnosis of familial Mediterranean fever.

Systemic vasculitis in childhood.

Systemic vasculitis is a group of disorders with multiorgan involvement. These disorders have diverse clinical manifestations associated with significant morbidity and mortality. The most common vasculitides in children--Henoch-Schönlein purpura and Kawasaki disease--are self-limiting conditions. The lifelong and chronic vasculitides (eg, giant cell arteritis, Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome, polyarteritis nodosa, and Takayasu arteritis) are rarely seen in children. Therefore, the outcome in general is more favorable in children. This article offers an overview of the epidemiologic, etiologic, pathophysiologic, and clinical features of vasculitis in children, with emphasis on common conditions.

Kawasaki disease: clinical spectrum of 88 patients in a high-prevalence African-American population.

Kawasaki disease (KD) is a systemic vasculitis that predominantly affects infants and young children. Asian race has been described as an important factor for the development of this disease. We reported our experience in a cohort of patients with KD. Clinical and laboratory data from initial presentation and follow-up visits were reviewed in 88 patients with KD treated at Children's Hospital of New Orleans between March 1993 and November 2004. Forty-nine (56%) patients were African American, 35 (40%) Caucasian, two (3%) Asian and two (3%) Hispanic. Coronary artery aneurysms (CAAs) were detected in 15 (17%) patients. CAAs were less frequent in African-American patients compared to Caucasians [relative risk (RR)=0.2, 95% CI: 0.04400-0.8405, p=0.0164]. Eighty-three patients responded to one dose of intravenous human immunoglobulin (IVIG), five patients responded to a second infusion of IVIG, and two who were IVIG resistant responded only following methylprednisolone pulse. Hemoglobin (Hb) levels of <10 g/dl and presence of polymorphic rash were significantly correlated with development of CAAs. No deaths were observed.

Childhood sarcoidosis: Louisiana experience.

A retrospective chart review was conducted to detect patients with sarcoidosis seen by pediatric rheumatology service from the period of 1992 to 2013 at Children's hospital of New Orleans. Twenty-seven patients were identified. The average duration of symptoms before diagnosis was 5 (range 1-120) months. Five patients had onset before the age of 5 years and were diagnosed with early-onset sarcoidosis. The most common manifestations at presentation were constitutional symptoms (62 %) followed by ocular (38 %). During the course of illness, 19/27 (70 %) had multiorgan involvement. Common manifestations included uveitis/iritis (77 %), fever (50 %), hilar adenopathy (42 %), arthritis (31 %), peripheral lympadenopathy (31 %), hepatosplenomegaly (31 %), parenchymal lung disease (27 %), and skin rash (19 %). Unusual manifestations included granulomatous bone marrow disease (3 cases), hypertension (2), abdominal aortic aneurysm (large vessel vasculitis; 1), granulomatous hepatitis (1), nephrocalcinosis (1), membranous nephropathy (1), refractory granulomatous interstitial nephritis with recurrence in transplanted kidney (1), CNS involvement (2), parotid gland enlargement (1), and sensorineural hearing loss (1). Biopsy specimen was obtained in 21/27 (77 %) patients, and demonstration of noncaseating granuloma associated with negative stains for mycobacteria and fungi was seen in 18 patients. Elevated angiotensin-converting enzyme level was seen in 74 % of patients. Treatment with oral prednisone was initiated in symptomatic patients with significant clinical improvement. Low-dose methotrexate (MTX) 10-15 mg/m(2)/week orally, as steroid-sparing agent, was administered in 14 patients. Other immunomodulators included cyclophosphamide (2 patients), etanercept (2), infliximab (2), mycophenolate mofetil (1), and tacrolimus (1). Childhood sarcoidosis is prevalent in Louisiana. Most of the affected children present with a multisystem disease associated with manifestations similar to those of adult patients. Low-dose MTX seems to be effective, steroid sparing, and safe adjunct to treat sarcoidosis with multiorgan involvement. Early-onset disease is less common and associated with increased morbidity, flares, and poor prognosis.

Simultaneous occurrence of diabetes mellitus and juvenile dermatomyositis: report of two cases.

The simultaneous occurrence of juvenile dermatomyositis (DMS) and diabetes mellitus is described in 2 pediatric patients. Both these patients presented with significant weight loss, polyuria, and polydypsia within a short time of being diagnosed with JDMS, while these patients were taking oral prednisone (40-60 mg/day in divided doses). Laboratory evaluation detected ketonuria, significant hyperglycemia (696 and 913 mg/dL) and low serum levels of insulin and C-peptide. Both these patients were treated with high doses of insulin. Islet cell and GAD65 antibodies were found to be positive in 1 of the patients, pointing toward a diagnosis of insulin-dependent diabetes mellitus. The other patient tested negative for these antibodies and required insulin therapy for approximately 6 months. Steroid-induced diabetes mellitus seemed highly likely in this case. We hypothesize that a common environmental trigger possibly a viral infection might have been responsible in causing 2 different autoimmune pathologies in these genetically predisposed individuals.

Management of septic arthritis.

Septic arthritis in children remains a serious disease with the potential for significant systemic and musculoskeletal morbidity. Staphlococcus aureus is the most common cause of bone and joint infections in all age groups. Microbial invasion of the synovial space occurs typically results from hematogenous seeding. Diagnosis in neonates and young infants can be difficult since the clinical signs are much less specific in these age groups. Early diagnosis by needle aspiration of the affected joint and prompt initiation of appropriate antimicrobial therapy in conjunction with drainage of the affected joint is critical to avoid destruction of the articular cartilage and prevent disability. Septic arthritis in infants and children should always be managed by a pediatrician in close consultation with an orthopedic surgeon. Empiric antibiotic regimens should always include adequate anti-staphylococcal coverage. Antibiotic treatment should be started with appropriate doses of intravenous antibiotics. Switch to oral antibiotic therapy can be made when patient demonstrates clinical improvement. A minimum of 3-4 weeks of therapy is recommended. Close follow-up is warranted to monitor the growth of the affected limb until skeletal maturity.

Clinical case of the month. 3-year-old boy presenting with a hand rash. Juvenile dermatomyositis.

Of the diseases within the spectrum of the juvenile idiopathic inflammatory myopathies, juvenile dermatomyositis (JDM) is the most common. As the name implies, JDM affects the muscles and skin most commonly, but can involve other organ systems as well. Dermatologic manifestations often precede other signs and symptoms by months or even years and frequently are the primary reason the patient seeks medical attention. In the case presented here, a 3-year-old boy initially developed a hand rash that brought him to his primary care physician. By the time muscle weakness had developed, the patient had already been evaluated for dermatomyositis and therapy had been initiated. An understanding of these early clinical findings will enable physicians to make a timely diagnosis and commence therapy promptly in order to prevent life-threatening sequelae of the disease.