Severe BCG immune reconstitution inflammatory syndrome lymphadenitis successfully managed with pre-antiretroviral counseling and a non-surgical approach: a case report.

BACKGROUND: Bacillus Calmette-Guérin (BCG) reactions are the most common cause of immune reconstitution inflammatory syndrome (IRIS) in HIV-positive infants who initiate antiretroviral therapy (ART). There is limited evidence regarding the incidence of BCG-IRIS; however, reports from outpatient cohorts have estimated that 6-9% of infants who initiated ART developed some form of BCG-IRIS within the first 6 months. Various treatment approaches for infants with BCG-IRIS have been reported, but there is currently no widely accepted standard-of-care. CASE PRESENTATION: A 5-month-old male HIV-exposed infant BCG vaccinated at birth was admitted for refractory oral candidiasis, moderate anemia, and moderate acute malnutrition. He had a HIV DNA-PCR collected at one month of age, but the family never received the results. He was diagnosed with HIV during hospitalization with a point-of-care nucleic acid test and had severe immune suppression with a CD4 of 955 cells/µL (15%) with clinical stage III disease. During pre-ART counseling, the mother was educated on the signs and symptoms of BCG-IRIS and the importance of seeking follow-up care and remaining adherent to ART if symptoms arose. Three weeks after ART initiation, he was readmitted with intermittent subjective fevers, right axillary lymphadenopathy, and an ulcerated papule over the right deltoid region. He was subsequently discharged home with a diagnosis of local BCG-IRIS lymphadenitis. At six weeks post-ART initiation, he returned with suppurative lymphadenitis of the right axillary region that had completely eviscerated through the skin without signs of disseminated BCG disease. He was then started on an outpatient regimen of topical isoniazid, silver nitrate, and oral prednisolone. Throughout this time, the mother maintained good ART adherence despite this complication. After 2.5 months of ART and one month of specific treatment for the lymphadenitis, he had marked mass reduction, improved adenopathy, increased CD4 count, correction of anemia, and resolution of his acute malnutrition. He completely recovered and was symptom free two months after initial treatment without surgical intervention. CONCLUSIONS: This case details the successful management of severe suppurative BCG-IRIS with a non-surgical approach and underlines the importance of pre-ART counseling on BCG-IRIS for caregivers, particularly for infants who initiate ART with advanced HIV.

Cervical lymphadenitis from Mycobacterium avium complex.

We present an instructive case of cervical lymphadenitis in a young man without a history of HIV infection. The patient developed spontaneous left-sided neck swelling that progressed over 4 months. CT imaging demonstrated a necrotic left-sided neck mass within the cervical lymph node chain. He was initially prescribed azithromycin and rifampin for presumed cat scratch disease with improvement but incomplete resolution of symptoms. Blood cultures ordered 2 months later grew Mycobacterium avium complex (MAC) and the patient had an excellent clinical response to MAC therapy. Here, we review the case, including presentation and management, and describe the implications for the immune status of the host and long-term considerations for treatment.

Activity of biogenic silver nanoparticles in planktonic and biofilm-associated Corynebacterium pseudotuberculosis.

Corynebacterium pseudotuberculosis is a gram-positive bacterium and is the etiologic agent of caseous lymphadenitis (CL) in small ruminants. This disease is characterized by the development of encapsulated granulomas in visceral and superficial lymph nodes, and its clinical treatment is refractory to antibiotic therapy. An important virulence factor of the Corynebacterium genus is the ability to produce biofilm; however, little is known about the characteristics of the biofilm produced by C. pseudotuberculosis and its resistance to antimicrobials. Silver nanoparticles (AgNPs) are considered as promising antimicrobial agents, and are known to have several advantages, such as a broad-spectrum activity, low resistance induction potential, and antibiofilm activity. Therefore, we evaluate herein the activity of AgNPs in C. pseudotuberculosis, through the determination of minimum inhibitory concentration (MIC), minimum bactericidal concentration (MBC), antibiofilm activity, and visualization of AgNP-treated and AgNP-untreated biofilm through scanning electron microscopy. The AgNPs were able to completely inhibit bacterial growth and inactivate C. pseudotuberculosis at concentrations ranging from 0.08 to 0.312 mg/mL. The AgNPs reduced the formation of biofilm in reference strains and clinical isolates of C. pseudotuberculosis, with interference values greater than 80% at a concentration of 4 mg/mL, controlling the change between the planktonic and biofilm-associated forms, and preventing fixation and colonization. Scanning electron microscopy images showed a significant disruptive activity of AgNP on the consolidated biofilms. The results of this study demonstrate the potential of AgNPs as an effective therapeutic agent against CL.

Kawasaki disease in neonates: a case report and literature review.

BACKGROUND: Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology that affects infants and young children but is extremely rare in neonates, especially afebrile KD. We present a case of KD without fever in a neonate and review the literature on KD in neonates. CASE PRESENTATION: A newborn female was hospitalized because her peripheral blood leukocytes increased for half a day. The admission diagnosis was considered neonatal sepsis and bacterial meningitis. She had no fever since the admission, but a rash appeared on her face by the 7th day. On day 11 after admission, there was a desquamation on the distal extremities. On day 15 after admission, ultrasound showed non-suppurative cervical lymphadenopathy. Echocardiogram revealed coronary artery aneurysms in both sides. Finally, the patient was diagnosed with incomplete KD (IKD). The follow-up echocardiogram showed that the internal diameter of both coronary arteries returned to normal three months after birth. CONCLUSIONS: Fever, rash, and distal extremity desquamation during the recovery phase are the most common symptoms of IKD. When newborns present with clinical manifestations such as rash, distal extremity desquamation and cervical lymph adenitis and with an increased peripheral blood leukocyte count and progressive increase in platelets simultaneously, the medical staff should be highly alert to the possibility of KD even without fever. The echocardiogram needs to be performed promptly. The incidence of coronary artery lesions is significantly higher if neonatal KD patients miss timely diagnosis and treatment.

Successful treatment of sebaceous adenitis with oclacitinib and low-dose prednisolone in a dog.

Finding an effective and well-tolerated treatment for canine idiopathic sebaceous adenitis (ISA) can be challenging. This case report describes an 8-year-old male Rottweiler with ISA successfully treated with a combination of oclacitinib and low doses of prednisolone.

Encontrar um tratamento eficaz e bem tolerado para a adenite sebácea idiopática (ISA) pode ser desafiador. Este relato descreve o caso de um cão Rottweiler macho de oito anos de idade tratado satisfatoriamente com uma associação de oclacitinib e baixas doses de prednisolona.

Encontrar un tratamiento efectivo y bien tolerado frente a la adenitis sebácea idiopática (ISA) puede ser un reto difícil. Este artículo describe el caso de un perro Rottweiler de ocho años tratado con eficacia con una combinación de oclacitinib y dosis bajas de prednisolona.

Trouver un traitement efficace et bien toléré pour l'adénite sébacée idiopathique canine (ASI) peut s'avérer difficile. Ce rapport clinique décrit le cas d'un Rottweiler mâle de 8 ans atteint d'ASI et traité avec succès par une combinaison d'oclacitinib et de faibles doses de prednisolone.

The use of ketotifen as long-term anti-inflammatory prophylaxis in children with PFAPA syndrome.

Introduction: Periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA) is the most frequent periodic fever syndrome in children. Its pathogenesis is still unknown, but some disease-modifying factors were observed. Several medications were tested for the long-term prophylaxis of inflammatory flares; however, none are standardly used. Methods: This prospective clinical trial enrolled 142 children (71 girls, 50%) meeting diagnostic criteria for PFAPA syndrome. We analysed selected clinical characteristics and compared laboratory parameters during the flare and attack-free period (at least two weeks after the attack). Moreover, we assessed the possible therapeutic effect of ketotifen on the duration of attack free-periods and clinical picture. Results: The mean age of patients was 6.81 ± 3.03 years and the mean age of onset of symptoms was 2.31 ± 2.02 years. No significant differences were observed between genders.We recorded a positive family history for PFAPA in 31.69% of patients. Attacks lasted for 2.8 ± 1.2 days, with intervals between attacks of 4 ± 1 weeks. We administered ketotifen in 111 (77.8%) patients, and a positive effect was observed in 86 (77.5%) of patients. We observed prolonged attack-free intervals in patients treated with ketotifen (14.7 ± 8.9 days in comparison with 4.4 ± 1.9 days before the treatment; p<0.001). The used dose of ketotifen was 0.08 ± 0.01 mg/kg/day. Mild side effects were observed in four patients (restlessness, irritability, agitation and constipation). Discussion: Our data supports the use of ketotifen for long-term prophylaxis in children with PFAPA syndrome with positive effects on the attenuation of disease activity and the prolongation of attack-free periods. Further well-designed studies should confirm the preliminary data.

Case Report: Tuberculosis lymphadenitis with systemic lupus erythematosus in a young woman: a case report.

Background: Tuberculosis is a chronic infectious disease and can be categorised into pulmonary TB and extra-pulmonary TB based on its spread. TB lymphadenitis is one of the extra-pulmonary TB diseases. Patients with a weakened immune system in systemic lupus erythematosus (SLE) have an increased incidence of TB. Case: Here we present a case report of a 21-year-old female patient with SLE diagnosed with tuberculous lymphadenitis at dr. Zainoel Abidin Hospital, Banda Aceh, Indonesia. The patient complained of a lump in the right neck 4 months ago with a diameter of 4 cm, accompanied by fever, decreased appetite, and weight loss. Other than that, the patient also experiences joint pain, hair loss and sun sensitivity since 12 months ago. Chest radiography showed no abnormalities, and fine-needle aspiration biopsy results confirmed tuberculous lymphadenitis. Antinuclear antibody test was borderline. The patient had been taking steroids and hydroxychloroquine for the past 10 months. Currently, the patient is taking the advanced phase of antituberculosis drugs FDC. After undergoing the intensive phase of antituberculosis drugs, the submandibular lump got smaller to a diameter of 2 cm. Conclusion: TB lymphadenitis is a rare case but can occur in conditions of decreased immunity like SLE. It involves some of the immune disorders caused by the long-term use of immunosuppressive therapy.

Non-Tuberculous Mycobacterial Cervicofacial Lymphadenitis in Children-10-Year Experience in a Tertiary Pediatric Center.

BACKGROUND: Lymphadenitis is the most common manifestation of non-tuberculous mycobacteria (NTM) infection in children. We describe the epidemiology and clinical characteristics of NTM lymphadenitis, determine diagnostic yield from tissue sampling, and review management and outcomes. METHODS: This was a 10-year retrospective review of children aged 0-16 years diagnosed with NTM cervicofacial lymphadenitis who were seen in a pediatric infectious disease clinic in a tertiary public hospital. Data relating to patient demographics, clinical features, surgical and antimicrobial treatment, complications, and outcomes were retrieved from patients' electronic medical records and analyzed. RESULTS: There were 48 episodes of NTM cervicofacial lymphadenitis in 45 children (17 males and 28 females). Of these episodes, 43.7% manifested as a unilateral single node, mostly parotid (39.6%) and submandibular (29.2%). All patients underwent diagnostic fine-needle aspiration or surgery. Surgical excision more frequently yielded positive histological findings (P = .016). NTM was identified in 22/48 episodes (45.8%) via culture or molecular sequencing. Mycobacterium abscessus was most commonly found (47.8%). Thirty-eight children (79.2%) received antibiotics. Outcomes in 43 episodes revealed full resolution in 69.8%, while 25.6% had de novo disease and 4.6% experienced recurrence at the same site. Overlying skin changes and multiple or bilateral nodal diseases were significantly associated with de novo disease or recurrence (P = .034 and .084, respectively). Complications occurred in 11/70 (15.7%) procedures. Antibiotic-associated adverse effects occurred in 14/38 (36.8%) episodes. CONCLUSIONS: NTM lymphadenitis remains a challenging condition. More aggressive management with surgical excision and antibiotics is recommended for those with overlying skin changes and extensive nodal disease.

Cervical lymphadenitis caused by Mycobacterium avium in a patient treated with Janus kinase inhibitors.

A woman diagnosed with rheumatoid arthritis and treated with Janus kinase (JAK) inhibitors presented with a gradually enlarging bilateral submandibular lymph nodes swelling that had lasted several weeks. A lymph node biopsy showed epithelioid granulomatous lymphadenitis with caseous necrosis. Mycobacteria grew in acid-fast bacteria culture and were identified as Mycobacterium avium by polymerase chain reaction. The patient was diagnosed with cervical lymphadenitis caused by M. avium. A computed tomography scan showed no evidence of a mass or infection at other sites, including the lungs; therefore, the mass was excised without any antimicrobial treatments. Her neck mass had not recurred at 9 months after the excision. JAK inhibitors have emerged as an important new class of oral therapy for rheumatoid arthritis and other diseases. Physicians should be aware of the relatively rare complications, such as cervical lymphadenitis caused by nontuberculous mycobacteria, when using JAK inhibitors.

Case Report: Lepromatous Leprosy Masquerading as Acute Suppurative Lymphadenitis.

Leprosy is a chronic cutaneous infection. It is usually characterized by thickened nerves and maculo-anesthetic patches. Leprosy often has an unusual presentation, which is a diagnostic challenge. In this case report, we present a case of an elderly male who presented with fever and chronic pus-draining axillary, cervical, and inguinal lymph nodes. He also had a weak left foot for the previous 5 months. During his hospital stay, he developed additional papular lesions over his extremities. We performed fine needle aspiration from the lymph nodes and skin biopsy, which were suggestive of lepromatous leprosy. We initiated him on antileprosy medication. On follow-up, he was responsive to therapy. Although skin and nerve involvement in leprosy is common, this case had an atypical presentation of discharging lymph nodes.

Adult-onset Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis Syndrome Responsive to Colchicine.

We herein report a rare case of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome that occurred in an 18-year-old man. He visited our hospital with recurrent episodes of a fever, pharyngitis and adenitis without suggestive findings of infection. These episodes resolved within 5 days and recurred quite regularly, with an interval of about 30 days. As the febrile episodes significantly impaired his quality of life, he was treated with colchicine (0.5 mg) as prophylaxis. This completely prevented the episodes during six months of follow-up. Colchicine may therefore be effective in cases of adult-onset PFAPA syndrome.

Acute bacterial lymphadenitis in children: a retrospective, cross-sectional study.

Acute bacterial lymphadenitis is a common childhood condition, yet there remains considerable variability in antibiotic treatment choice, particularly in settings with low prevalence of methicillin-resistant Staphylococcus aureus such as Europe and Australasia. This retrospective cross-sectional study reviewed children presenting with acute bacterial lymphadenitis to a tertiary paediatric hospital in Australia between 1 October 2018 and 30 September 2020. Treatment approaches were analysed with respect to children with complicated versus uncomplicated disease. A total of 148 children were included in the study, encompassing 25 patients with complicated disease and 123 with uncomplicated lymphadenitis, as defined by the presence or absence of an associated abscess or collection. In culture-positive cases, methicillin-susceptible S. aureus (49%) and Group A Streptococcus (43%) predominated, while methicillin-resistant S. aureus was seen in a minority of cases (6%). Children with complicated disease generally presented later and had a prolonged length of stay, longer durations of antibiotics, and higher frequency of surgical intervention. Beta-lactam therapy (predominantly flucloxacillin or first-generation cephalosporins) formed the mainstay of therapy for uncomplicated disease, while treatment of complicated disease was more variable with higher rates of clindamycin use.    Conclusion: Uncomplicated lymphadenitis can be managed with narrow-spectrum beta-lactam therapy (such as flucloxacillin) with low rates of relapse or complications. In complicated disease, early imaging, prompt surgical intervention, and infectious diseases consultation are recommended to guide antibiotic therapy. Prospective randomised trials are needed to guide optimal antibiotic choice and duration in children presenting with acute bacterial lymphadenitis, particularly in association with abscess formation, and to promote uniformity in treatment approaches. What is Known: • Acute bacterial lymphadenitis is a common childhood infection. • Antibiotic prescribing practices are highly variable in bacterial lymphadenitis. What is New: • Uncomplicated bacterial lymphadenitis in children can be managed with single agent narrow-spectrum beta-lactam therapy in low-MRSA prevalence settings. • Further trials are needed to ascertain optimal treatment duration and the role of clindamycin in complicated disease.

Microbiology Culture Results and Antibiotic Prescribing in Pediatric Patients With Lymphadenitis.

BACKGROUND: Bacterial lymphadenitis is a common reason for antibiotic treatment and hospitalization in children. The literature available addressing the bacterial etiology of lymphadenitis recommends the use of narrow-spectrum agents to cover common pathogens. We suspect that patients at our institution receive unnecessarily broad-spectrum antimicrobial agents. The primary objective of this study was to characterize the microbiology and antibiotic use in lymphadenitis patients. METHODS: Retrospective review of children admitted over a 10-year period with an International Classification of Diseases Ninth or Tenth Edition code for lymphadenitis. Patients were included if they were <18 years old, admitted to the inpatient ward, and had intraoperative lymph node cultures collected. RESULTS: A total of 131 patients admitted with lymphadenitis had lymph node cultures collected and were included. Seventy-two (72/131; 55%) patients had positive lymph node culture results with pathogenic bacteria. The predominant pathogens were Staphylococcus aureus (56/72; 77.8%) and Streptococcus pyogenes (10/72; 13.9%). The most common inpatient empirical regimen was ampicillin-sulbactam. Of the 72 patients with typical pathogens identified, 80.6% were sensitive to a first-generation cephalosporin, whereas 86.1% were sensitive to a ß-lactam/ß-lactamase inhibitor. CONCLUSION: Patients presenting to our institution with acute bacterial lymphadenitis were predominantly found to have methicillin-susceptible S. aureus lymphadenitis that could be empirically treated with cefazolin. At our institution, there is little advantage to the most commonly used broad-spectrum agent, ampicillin-sulbactam.

Does having MEFV gene sequence variants affect the clinical course and colchicine response in children with PFAPA syndrome?

The primary aim of this study was to document the treatment modalities used in periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome and look for the efficacy and safety of colchicine in the treatment of PFAPA patients. The secondary aim was to search for whether having MEFV (Mediterranean fever) gene sequence variants affect the clinical course and response to colchicine. The study was conducted in 2 pediatric rheumatology centers. The patients that have been diagnosed with PFAPA syndrome between December 2017 and December 2021 were evaluated retrospectively. The study included 157 patients with PFAPA syndrome (54.8% boys and 45.2% girls). The median follow-up duration was 18 (IQR: 12-30) months. One hundred and fifty-five patients (98.7%) had exudative pharyngitis, 120 patients (76.4%) had aphthous stomatitis, and 82 patients (52.2%) had cervical lymphadenitis during the attacks. Clinical features during attacks were not affected by the presence or absence of the MEFV gene sequence variants. Corticosteroid treatment during attacks was given to 152 patients (96.8%). The frequency of fever attacks did not change in 57 patients (37.5%), increased in 57 patients (37.5%), and decreased in 38 patients (25%) after corticosteroid use. Colchicine was given to 122 patients (77.7%) in the cohort. After colchicine treatment, complete/near-complete resolution of the attacks was observed in 57 patients (46.7%). Colchicine led to partial resolution of the attacks in 59 patients (48.4%). In only 6 patients (4.9%), no change was observed in the nature of the attacks with colchicine treatment. The median duration of the attacks was 4 (IQR: 4-5) days before colchicine treatment, and it was 2 (IQR: 1-2.5) days after colchicine treatment. Also, a significant decrease in the frequency of the attacks was observed before and after colchicine treatment [every 4 (IQR: 3-4) weeks versus every 10 (IQR: 8-24) weeks, respectively, (p < 0.001)]. The overall response to colchicine was not affected by MEFV sequence variants. It was seen that the frequency of fever attacks decreased dramatically in both groups, and children with MEFV variants had significantly less attacks than children without MEFV variants after colchicine treatment (every 11 weeks vs every 9.5 weeks, respectively, p: 0.02). CONCLUSION: Colchicine seems to be an effective and safe treatment modality in PFAPA treatment. It led to a change in the nature of the attacks either in the frequency, duration, or severity of the attacks in 95.1% of the patients. This study has shown that having MEFV gene sequence variants did not affect the clinical course or response to colchicine. We recommend that colchicine should be considered in all PFAPA patients to see the response of the patient, irrespective of the MEFV gene mutations. WHAT IS KNOWN: • Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is the most common periodic fever syndrome in the world. Familial Mediterranean fever (FMF) is the most common cause of periodic fever syndrome in Turkey. • Colchicine has become a new treatment option in PFAPA. WHAT IS NEW: • Some PFAPA patients have Mediterranean fever (MEFV) gene variants, and it is speculated that PFAPA patients with MEFV gene mutations respond better to colchicine. • The aim of this study was to look for this hypothesis. We have seen that the clinical phenotype and colchicine response of PFAPA patients were not affected by MEFV gene sequence variants.

Efficacy of canakinumab in a patient with adult-onset glucocorticoid-resistant periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome.

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome, a polygenic or multifactorial condition, is the most frequent autoinflammatory disease in children. There is increasing evidence that some patients may have a disease onset during adulthood. With regard to PFAPA syndrome treatment, single medium-to-high doses of glucocorticoids during flares constitute the therapy of choice in children and adults, colchicine may be useful in some patients, and tonsillectomy has been reported of utility mainly in paediatric patients. Interleukin-1 (IL-1) blockers have been sporadically used with good response in glucocorticoid-resistant cases. We report a patient with an adult onset of glucocorticoid-resistant PFAPA syndrome and inconsistent response to colchicine and anakinra, who later achieved a complete and sustained response to canakinumab. Although canakinumab seems to be a good therapeutic option in paediatric and adult patients with refractory PFAPA syndrome, the best anti-IL-1 agent and the sequence of administration have to be still determined in well-designed clinical trials.

Purulent lymphadenitis due to Halomonas hamiltonii: a case report.

Halomonas hamiltonii is a gram-negative rod bacterium isolated from highly saline environments. H. hamiltonii has rarely been reported as a human pathogen. Herein, we present the first case report of a purulent lymphadenitis caused by H. hamiltonii worldwide. The patient was a previously healthy girl aged 1 year who was referred to our hospital for left axillary lymphadenitis. Although oral amoxicillin was administered, lymphadenitis did not improve, and an abscess developed. After incision and drainage, the abscess was reduced. No recurrence of lymphadenitis was observed. The pus culture was negative. However, the 16S ribosomal DNA was amplified by the melting temperature mapping method. The amplified 16S ribosomal DNA sequence revealed 99.7% identity of H. hamiltonii. To the best of our knowledge, this is the first case of H. hamiltonii infection in a lymph node. This pathogen should be considered when diagnosing purulent lymphadenitis in healthy patients with lymphadenopathy of unknown origin.

Clinical, Microbiological Profile and Treatment Outcomes of Infants with BCG Adenitis: A Retrospective Study.

BACKGROUND: Bacille Calmette-Guérin (BCG) adenitis is an uncommon complication following BCG vaccination. In rare cases, infants can develop other complications. Controversy exists regarding the diagnosis and management of these cases. Not much information is available in literature regarding their microbiological and immunological characteristics. METHODS: Electronic medical records of children presenting to the Pediatric Infectious Diseases clinic in a tertiary care hospital from January 2011-December 2020 with a diagnosis of BCG adenitis were retrospectively reviewed. Their clinical, microbiological, treatment and follow-up data were noted and analyzed. FINDINGS: During the study period, 40 infants presented with a probable diagnosis of BCG adenitis with or without disseminated BCG. Median age at symptom onset was 4(2.5-5.9) months. Nine infants had disseminated disease at presentation. Fifteen infants were suspected to have underlying immune deficiency of whom 12 had proven defects in immune function. On multivariable logistic regression analysis, presence of disseminated disease was the only factor predictive of underlying immunodeficiency. Isoniazid monoresistance was seen in seven cases (32%) of the 22 samples sent for TB cultures. CONCLUSIONS: Though BCG adenitis runs a benign course, it could rarely be the first manifestation of an underlying immune defect. There is sizable isoniazid monoresistance, hence sending tissue samples for microbiologic evaluation is necessary to guide anti-tubercular therapy.

Challenges in the diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome in developing countries-A decade of experience from North India.

Background: Reports of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome from developing countries are sparse. Recognizing PFAPA is often challenging in these regions due to a higher incidence of infectious illnesses and significant resource constraints. Herein, we present our experience from North India regarding the diagnosis and management of PFAPA syndrome. Methods: We reviewed cases of non-monogenic periodic fever syndrome diagnosed at our center from January 2011 to December 2021. A total of 17 children who fulfilled the Marshall criteria for PFAPA syndrome were included. Data regarding basic clinical features, treatment/outcome, and performance of the recently proposed Eurofever/PRINTO and Takeuchi criteria were analyzed. Results: Besides recurrent fever, the triad of oral aphthae, pharyngitis, and adenitis was noted in only 18% of patients. Episodes of exudative pharyngitis/tonsillitis were documented in 24%. These figures were lower than the values reported from developed countries. The Takeuchi and Eurofever/PRINTO criteria were fulfilled in 76% and 71% cases, respectively. In addition to antipyretics and supportive care, intermittent steroid therapy was the main treatment modality used. Additional treatment with colchicine (n = 3) and thalidomide (n = 1) was used successfully in a few patients. Before the diagnosis of PFAPA, all patients had received multiple courses of antimicrobials (without microbiological confirmation). These included multiple courses of antibacterials for fever, pharyngotonsillitis, and/or cervical adenitis in all patients and antivirals for fever and aphthous stomatitis in a patient. Empiric antitubercular therapy had also been administered in two patients. Conclusions: A significant proportion of patients with PFAPA seem to remain undiagnosed in the Indian subcontinent. Increased awareness and improvement in basic healthcare facilities are crucial in enhancing the recognition of PFAPA, which would eliminate the unprecedented scale of undesirable antimicrobial use in such children.