Cardiac involvement, which may remain clinically silent, is a major determinant of prognosis in eosinophilic granulomatosis with polyangiitis (EGPA), and remission induction generally requires glucocorticoids combined with cyclophosphamide or rituximab. In contrast, treatment selection may be challenging in women wishing to preserve fertility because cyclophosphamide carries a risk of gonadal toxicity. We report a 40-year-old woman with a history of asthma, eosinophilic chronic rhinosinusitis, and eosinophilic otitis media who developed postpartum-onset EGPA and presented with neurological and cutaneous manifestations. Elevated cardiac troponin I levels and newly developed electrocardiographic abnormalities suggested subclinical myocardial injury despite the absence of cardiac symptoms. Intravenous methylprednisolone pulse therapy was initiated immediately. Because cardiac troponin I levels showed no early decline after glucocorticoid initiation, and glucocorticoid monotherapy was considered potentially insufficient to control eosinophil-mediated myocardial injury, additional eosinophil-targeted therapy was selected after shared decision-making that balanced fertility preservation with the need for prompt disease control. Benralizumab was chosen because of its potent eosinophil-depleting effect. Peripheral eosinophils rapidly decreased to zero, troponin I normalized by Day 26, and glucocorticoids were successfully discontinued within 5 months. To our knowledge, this is the first report of benralizumab used as part of a fertility-preserving remission-induction strategy to avoid cyclophosphamide in a woman with EGPA and cardiac involvement. This approach may represent an individualized therapeutic option for carefully selected patients in whom fertility preservation is an important treatment consideration.
Juvenile Systemic Lupus Erythematosus (jSLE) is a rare disorder that presents with greater severity compared to adult Systemic Lupus Erythematosus. It typically manifests in adolescent females with constitutional symptoms, multisystem involvement, and a malar rash. In this case, a 9-year-old African American pre-pubertal girl presented with a progressive facial and scalp rash, weight loss, night sweats without fever, and abdominal pain following recent penicillin exposure. On laboratory results and clinical examination, leucopenia, auricular chondritis, palatal ulceration, and marked facial rash were found on physical exam and initial lab testing, alongside a negative infectious disease workup. Autoimmune workup demonstrated an elevated ANA and anti-dsDNA with low complement levels. Therefore, she met diagnostic criteria for jSLE. She improved rapidly with corticosteroids. Over the following year, she subsequently developed lupus nephritis. This case illustrates an atypical presentation of jSLE with early-onset prior to puberty and uncommon features, including chondritis, discoid lesions, and isolated mucocutaneous symptoms, with no associated joint pain or organ damage at initial presentation. Atypical jSLE presentations require high clinical suspicion to prevent delayed diagnosis and progression to complications such as lupus nephritis.
Pregnancy after anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis-associated interstitial lung disease is rarely described, and preconception management remains uncertain. A 29-year-old woman presented with fever, arthralgia, cough, characteristic Gottron's and inverse Gottron's papules, normal muscle enzyme levels, mild interstitial lung abnormalities on high-resolution computed tomography, and positivity for anti-melanoma differentiation-associated gene 5 antibody. Because of the potential risk of rapid pulmonary deterioration, she received prednisolone, a calcineurin inhibitor, and intravenous cyclophosphamide, followed by maintenance immunosuppression. Mycophenolate mofetil was discontinued as part of preconception planning 24 months after disease onset. After two biochemical pregnancies that ended before ultrasonographic confirmation of a gestational sac, a subsequent pregnancy was confirmed approximately 36 months after disease onset while she was receiving prednisolone 10 mg/day and tacrolimus. Pregnancy was not complicated by relapse of dermatomyositis or interstitial lung disease, hypertensive disorder, gestational diabetes, fetal growth restriction, or non-reassuring fetal status. She delivered a healthy female infant at 38 weeks and 3 days of gestation by vaginal delivery. At more than 4 years postpartum, she remained relapse-free, and the antibody remained negative. This case suggests that planned pregnancy may be feasible in carefully selected patients after sustained preconception disease control, discontinuation of teratogenic drugs, and continuation of pregnancy-compatible immunosuppression under multidisciplinary surveillance; serial anti-MDA5 antibody measurements may provide adjunctive information when interpreted alongside clinical, pulmonary function, and imaging assessments.
Chronic non-bacterial osteomyelitis/chronic recurrent multifocal osteomyelitis (CNO/CRMO) is an autoinflammatory, sterile bone disorder that predominantly affects children but can also occur in adults. The nonspecific nature of its presentation, as well as relative lack of awareness of the condition, often results in significant diagnostic delay. In this article, we present the case of a female patient who began experiencing sternal pain at the age of 14, initially thought to be due to costochondritis but ultimately discovered to be secondary to CNO/CRMO. We demonstrate her long and tortuous diagnostic journey including key imaging and bone biopsy findings. We also report good clinical and radiographic response to tumour necrosis factor-α (TNF-α) inhibitors, despite having to switch from adalimumab (ADA) to etanercept (ETN) and then to methotrexate (MTX) due to side effects and anti-drug antibody production.
Sjögren's disease (SjD) is a systemic autoimmune disorder with glandular and extra-glandular manifestations. Pleural effusion is a rare complication of SjD among different types of pulmonary involvement. Herein, we report a case of primary anti-centromere antibody (ACA)-positive primary SjD with pleural effusion. A 79-year-old woman was admitted to our hospital with a 3-month history of leg oedema. She was diagnosed with primary SjD based on positive Schirmer and Saxon tests, and her anti-Sjögren syndrome-related antigen/Ro antibody test was negative, but ACA and anti-Sjögren syndrome-related antigen B/La tests were positive. The patient's symptoms promptly improved following prednisolone treatment. This is the first reported case of ACA-positive primary SjD complicated with pleural effusion, and the possible presence of pleural effusion should be considered in SjD, even in patients with ACA-positive primary SjD.
Lupus myocarditis and peripartum cardiomyopathy can both present with acute heart failure symptoms during the peripartum period, leading to diagnostic uncertainty. Accurate and timely recognition of lupus myocarditis is crucial due to its therapeutic implications and high mortality risk if misdiagnosed. This case report presents a 34-year-old female patient with systemic lupus erythematosus who presented to the emergency department 1 week postpartum with palpitations. In the presence of cardiac enzymes and a low ejection fraction on echocardiography, the patient was initially diagnosed with peripartum cardiomyopathy and subsequently accepted as lupus myocarditis due to clinical and laboratory findings suggestive of high systemic lupus erythematosus disease activity. The patient showed clinical improvement after pulse steroid and intravenous immunoglobulin therapy.
Eosinophilic granulomatosis with polyangiitis (EGPA) is an antineutrophil cytoplasmic antibody-associated vasculitis, affecting various organs. Although ocular involvement occurred in 6.8-11.1% of patients, anterior ischaemic optic neuropathy (AION) is rare. We present the case of a 57-year-old woman with an 11-year history of asthma who developed sudden, painless visual loss in her left eye. On admission (3 days after symptom onset), visual acuity was counting fingers in the left eye and 20/20 in the right eye. Funduscopy showed left optic disc oedema. Magnetic resonance imaging revealed intravitreal protrusion of the left optic nerve head with restricted diffusion and local contrast enhancement of the left intraorbital and retrobulbar fat. Given also peripheral eosinophilia, myeloperoxidase-antineutrophil cytoplasmic antibody positivity, mononeuritis multiplex, nasal polyps, sinusitis, and pulmonary involvement, EGPA with AION was diagnosed. Intravenous methylprednisolone pulse was urgently administered, followed by mepolizumab 2 weeks later. Although eosinophilic inflammation and imaging findings improved, visual acuity remained unchanged over 1 year. A literature review identified 14 cases of EGPA-associated AION. Including our case, 11 cases with available data (14 eyes) were analysed. Overall, visual recovery was observed in 29%. Among 12 eyes with severe visual impairment before treatment, visual recovery occurred only in those treated earlier or with cyclophosphamide except for one, whereas no recovery occurred in those with delayed treatment or without cyclophosphamide. This case highlights the potential of AION as an initial manifestation of EGPA. Both earlier initiation and adequate intensity of immunosuppressive therapy, particularly including cyclophosphamide when clinically appropriate, may be important for visual recovery in EGPA-associated severe AION.
Giant cell arteritis is the most common vasculitis in those over 50 years old with a peak incidence between 70 and 80. Symptoms include a new-onset headache, scalp tenderness, jaw/tongue claudication, and visual symptoms, which can be sight-threatening. We report a case of an 82-year-old lady who presented with typical symptoms of giant cell arteritis and bilateral halo sign on an ultrasound scan. Despite initial steroid treatment she deteriorated and developed neurological symptoms including mononeuritis multiplex. Following further investigations, it was found that she was suffering from granulomatosis with polyangiitis and treatment was adjusted accordingly with additional immunomodulating treatment preventing further progression of symptoms. This case highlights how granulomatosis with polyangiitis can mimic giant cell arteritis and should be considered in refractory cases.
INTRODUCTION:Prosthetic joint infection (PJI) represents a serious and challenging complication in a patient with rheumatoid arthritis. We report a case of PJI following revision total elbow arthroplasty with rheumatoid arthritis that was successfully managed with debridement, antibiotics, and implant retention augmented by continuous local antibiotic perfusion (CLAP). CASE PRESENTATION:A 69-year-old woman with a 39-year history of rheumatoid arthritis previously underwent revision total elbow arthroplasty for aseptic loosening. Two years following the revision surgery, she developed left elbow swelling, pain, and erythema, resulting in a diagnosis of PJI. Because the implant remained stable without evidence of loosening, an emergency debridement, antibiotics, and implant retention procedure was performed in conjunction with CLAP in attempt to salvage the prosthesis. Intraoperative cultures were positive for methicillin-susceptible Staphylococcus aureus. Following surgery, the patient received targeted systemic antibiotic therapy alongside continuous local gentamicin perfusion via the CLAP system. Local perfusion was discontinued on postoperative Day 18, and oral antibiotic suppressive therapy was continued for 3 months. At the 2-year follow-up examination, the patient showed no clinical evidence of recurrent infection and had maintained her preoperative elbow range of motion. CONCLUSION:The integration of CLAP with the debridement, antibiotics, and implant retention procedure represents a promising, prosthesis-preserving therapeutic strategy for managing PJI following revision total elbow arthroplasty.
Abstract Eosinophilic granulomatosis with polyangiitis is a heterogeneous vasculitic and eosinophilic disorder in which patients who are positive for myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) often develop glomerulonephritis and peripheral neuropathy and may require prolonged immunosuppressive therapy. Although rituximab (RTX) and mepolizumab have been used in selected patients with eosinophilic granulomatosis with polyangiitis, relapse during RTX maintenance remains difficult to manage. We report the case of a 59-year-old woman with adult-onset asthma and chronic rhinosinusitis who presented with severe eosinophilia, mononeuritis multiplex, MPO-ANCA positivity, and pauci-immune necrotising glomerulonephritis. Induction therapy with high-dose glucocorticoids, intravenous immunoglobulin, and RTX achieved clinical remission and allowed the tapering to a low dose of prednisolone. Despite RTX maintenance, she later relapsed with recurrent sinonasal symptoms, worsening neuropathic complaints, and a marked rise in MPO-ANCA levels. In this setting, a further escalation of immunosuppression during maintenance, including cyclophosphamide, may be considered; however, given the possibility that residual eosinophilic inflammation contributed to ongoing disease activity, including autoantibody production, mepolizumab was added after retreatment with RTX as sequential therapy. Symptoms stabilised and improved over the subsequent months, MPO-ANCA titres progressively declined to the normal range, and prednisolone was discontinued. Remission has been maintained on mepolizumab without further RTX maintenance. This case suggests that sequential eosinophil-targeted therapy may help consolidate remission after a relapse during RTX maintenance in MPO-ANCA-positive eosinophilic granulomatosis with polyangiitis.
An 84-year-old man with rheumatoid arthritis undergoing treatment with methotrexate presented with fever, general fatigue, and appetite loss. He had a 3-month history of unexplained normocytic anaemia with reticulocytopenia. Bone marrow examination revealed erythroid hypoplasia with giant pro-erythroblasts, leading to a diagnosis of parvovirus B19 infection. In this case, intravenous immunoglobulin therapy improved the patient's anaemia and systemic symptoms. Six months after the treatment, parvovirus B19 DNA remained detectable using polymerase chain reaction; however, the viral load had markedly decreased, indicating a favourable virological response. Twenty cases of parvovirus B19 infection in older adults (aged ≥65 years) were identified in the literature. Immunocompromised older patients frequently develop persistent parvovirus B19 infections that require treatment with intravenous immunoglobulin therapy, whereas persistent infections are not observed in immunocompetent older individuals. Therefore, this case suggests that clinicians should be aware of persistent parvovirus B19 infection in older immunosuppressed patients who develop unexplained anaemia with reticulocytopenia. Moreover, intravenous immunoglobulin therapy may be considered as an effective therapeutic option for persistent parvovirus B19 infection in immunocompromised patients.
Idiopathic multicentric Castleman disease (iMCD) is a benign lymphoproliferative disease characterized by generalized lymphadenopathy and systemic inflammatory symptoms, occurring in individuals without infection with human immunodeficiency virus (HIV) or Kaposi sarcoma-associated herpesvirus (KSHV). iMCD is typically subclassified into iMCD-TAFRO, which is characterized by thrombocytopenia, ascites, fever, reticulin fibrosis, and organomegaly; iMCD with idiopathic plasmacytic lymphadenopathy (iMCD-IPL), which follows a chronic disease course with persistent lymphadenopathy, marked polyclonal hypergammaglobulinemia, and prominent plasma cell infiltration in lymph nodes; and iMCD-not otherwise specified (iMCD-NOS), which lacks features of both TAFRO syndrome and the IPL phenotype. Pleural thickening and effusion are extremely rare manifestations of iMCD-NOS. Herein, we present a rare case of iMCD-NOS presenting with unilateral pleural thickening and pleural effusion. A 76-year-old Japanese man was referred for further evaluation of a massive left-sided pleural effusion with tracheal compression. Fluorodeoxyglucose positron emission tomography/computed tomography showed increased uptake in the thickened pleura and multiple lymph nodes. Histopathological examination of a mediastinal lymph node demonstrated medullary and lymphoid follicular hyperplasia without structural destruction, while biopsy of the thickened pleura showed infiltration of lymphocytes and plasma cells without dysplasia. The patient was treated with corticosteroids and tocilizumab, resulting in marked improvement in symptoms and pleural effusion. This case highlights the importance of considering pleural and lymph node biopsies for accurate diagnosis and of not excluding iMCD in patients with unilateral pleural thickening accompanied by multiple lymphadenopathies.
Neonatal lupus erythematosus (NLE) is an autoimmune disorder in which maternal antibodies, particularly anti-Sjögren's-syndrome-related antigen A autoantibodies (anti-SS-A) or anti-SS-B antibodies, cause complications in foetal or neonatal cases. We report a case of NLE in an infant born to an asymptomatic mother, presenting with fever and elevated C-reactive protein despite the absence of maternal anti-double-stranded DNA (anti-dsDNA) antibodies. The patient was a 35-day-old male infant who presented with a rash and fever. Laboratory findings included elevated C-reactive protein and ferritin, soluble interleukin-2 receptor, hypocomplementaemia, and elevated D-dimer levels. NLE or urticarial vasculitis secondary to possible infantile-onset systemic lupus erythematosus was considered in the differential diagnosis. The infant and mother tested positive for anti-SS-A and anti-SS-B antibodies, with titres decreasing over time, leading to a final diagnosis of NLE. Although anti-dsDNA antibodies are typically maternal in origin, the mother in this case was repeatedly negative, making transplacental transfer unlikely and suggesting possible de novo antibody production by the infant. Although fever is rare in NLE, this case presented with systemic symptoms similar to systemic lupus erythematosus, including fever, elevated C-reactive protein, and hypocomplementaemia. In some cases of NLE, systemic symptoms similar to systemic lupus erythematosus may be observed in addition to the rash, and anti-dsDNA antibodies may also be positive serologically.
Immunoglobulin A vasculitis is uncommon in adults and is frequently associated with an identifiable trigger, most often infection. Invasive meningococcal disease is a rare but life-threatening infection caused by Neisseria meningitidis. We report the case of an 18-year-old Indigenous Australian woman who presented with fever, purpuric rash, ankle synovitis, abdominal pain, and systemic inflammation. A skin biopsy demonstrated leukocytoclastic vasculitis with immunoglobulin A deposition on immunohistochemistry, and blood cultures subsequently identified N. meningitidis serogroup B. Cerebrospinal fluid studies were unremarkable. The patient was treated with intravenous ceftriaxone, with complete clinical resolution and no recurrence at a 6-month follow-up. This case highlights a previously unreported association between immunoglobulin A vasculitis and invasive meningococcal disease in an adult patient, and it expands the spectrum of infectious triggers associated with adult-onset immunoglobulin A vasculitis.
The pathophysiology of organ involvement in eosinophilic granulomatosis with polyangiitis is characterised by vasculitis and eosinophilic inflammation, which can be accompanied by eosinophil extracellular trap cell death (EETosis). Clinically, eosinophilic inflammation is often observed in the lungs, gastrointestinal tract, and heart, whereas vasculitis more frequently affects the peripheral nerves, skin, and kidneys. However, histopathological confirmation of vasculitis and EETosis in different organs within the same patient has rarely been reported. We report a patient with EGPA presenting with both cutaneous and cardiac involvement. Skin biopsy findings were consistent with cutaneous fibrinoid vasculitis with prominent eosinophilic infiltration. Prednisolone therapy led to improvement of the skin lesion and inflammatory markers within 1 month. In contrast, endomyocardial biopsy demonstrated marked eosinophilic infiltration with cytolytic degranulation and extracellular deposition of eosinophil granule proteins, without evidence of fibrinoid vasculitis. Immunofluorescence findings supported the presence of EETosis. Despite prompt methylprednisolone pulse therapy, the cardiac lesion showed limited response and was complicated by refractory arrhythmias. This case suggests that distinct pathogenic mechanisms, namely vasculitis and EETosis, may predominate in different organs even within the same patient, potentially leading to discrepant treatment responses. When EETosis-driven pathology is dominant, conventional immunosuppressive therapy may have limited efficacy, highlighting the need for therapeutic strategies targeting EETosis.
Surgical treatment for forefoot deformities in patients with rheumatoid arthritis has recently shifted toward joint-preserving procedures for all toes. In contrast, resection arthroplasty was previously more commonly performed for the lesser toes. Advances in drug therapy have improved life expectancy, resulting in an increased number of long-term survivors who had previously underwent resection arthroplasty. With increased activity levels, the recurrence of plantar callosities in the forefoot has become more frequent, often leading to gait disturbances. In general, revision surgery for such cases often involves additional bone resection; however, this approach may result in further shortening of the metatarsals and increase the risk of further recurrence. Therefore, we applied a metatarsal-shortening offset osteotomy, which we typically perform as a primary procedure, as a salvage procedure to reduce the risk of recurrence. We report three cases treated using this strategy. In all cases, the second to fifth metatarsal heads had been resected, but the resection stumps were covered with pseudo-cartilaginous tissue. This allowed a metatarsal-shortening offset osteotomy to be performed as a procedure similar to that used in primary surgery, achieving the planned correction. Consequently, both radiographic and clinical improvements were observed. These findings suggest that a metatarsal-shortening offset osteotomy may be a possible salvage option after primary resection arthroplasty for forefoot deformities in patients with rheumatoid arthritis.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of autoimmune disorders that cause inflammation and necrosis of small- to medium-sized blood vessels. It most commonly affects the kidneys and lungs, leading to complications such as rapidly progressive glomerulonephritis and pulmonary haemorrhage. Ocular involvement occurs in up to 50% of patients with AAV, most commonly presenting as scleritis, episcleritis, or orbital inflammatory disease (also known as orbital pseudotumor). Other ocular findings may include keratitis, uveitis, or retinal vasculitis. Eyelid necrosis with destructive orbital inflammation, however, is extremely rare and poses a significant diagnostic challenge because its presentation can mimic infections, malignancies, or other inflammatory orbital conditions. Prompt recognition is critical to prevent irreversible tissue damage and vision loss. We report the case of a 74-year-old Hispanic woman with multiple vascular risk factors who presented with recurrent pustules of the right upper eyelid that progressed to full-thickness eyelid necrosis, medial orbital mass effect, and corneal compromise. Initial management was directed toward presumed orbital cellulitis; however, orbital imaging revealed a destructive extraconal mass with mass effect and associated venous stasis. Sequential biopsies demonstrated extensive necrosis with histiocytoid granulomatous inflammation and focal features of chronic vasculitis. Negative stains and cultures excluded infectious and neoplastic causes. After thorough evaluation and exclusion of alternative etiologies, the findings were most consistent with ANCA-negative ANCA-AAV. The patient was treated with high-dose intravenous corticosteroids and rituximab induction, resulting in stabilization of orbital disease and preservation of vision. This case highlights the potential for destructive orbital vasculitis to masquerade as infection or malignancy and underscores the importance of considering AAV in atypical orbital presentations. Prompt recognition and initiation of immunosuppressive therapy are critical to prevent irreversible morbidity.
We report a 33-year-old woman with systemic lupus erythematosus who developed fulminant multiorgan ischemic manifestations and hematologic abnormalities. She presented with persistent fever, newly developed painful fingertip cyanosis, and acute kidney injury. Laboratory tests showed severe thrombocytopenia, hemolytic anaemia with fragmented red blood cells, positive direct and indirect Coombs tests, hypocomplementemia, and positivity for multiple autoantibodies, including a triple-positive antiphospholipid antibody profile. Within the first week of hospitalisation, in addition to digital ischemia, she developed multiple cerebral infarctions and acalculous cholecystitis, findings consistent with catastrophic antiphospholipid syndrome. Unexpectedly, the activity of a disintegrin and metalloproteinase with thrombospondin type 1 motifs member 13 (ADAMTS13) was severely reduced to 5%, whereas ADAMTS13 inhibitor was not detected by the Bethesda assay. There was no past medical or family history suggestive of congenital thrombotic thrombocytopenic purpura. After treatment with high-dose glucocorticoids and nine sessions of plasma exchange, abdominal pain resolved and digital ischemia improved, accompanied by improvement in hematologic and renal abnormalities and disappearance of fragmented red blood cells. Subsequently, ADAMTS13 activity normalised to 63% and remained stable after completion of plasma exchange. Later, non-inhibitory anti-ADAMTS13 autoantibodies were detected by enzyme-linked immunosorbent assay in a stored serum sample obtained on admission. ADAMTS13 deficiency due to non-inhibitory anti-ADAMTS13 autoantibodies may modify the complications of systemic lupus erythematosus and antiphospholipid syndrome by promoting thrombotic microangiopathy.
Subcutaneous flexor tendon rupture in patients with rheumatoid arthritis is rare compared with extensor tendon rupture, with a reported incidence of approximately one-tenth of that of extensor tendons. The pathophysiology of flexor tendon rupture is not fully understood. We report two cases of spontaneous flexor digitorum profundus tendon rupture of the little finger in patients with rheumatoid arthritis despite low disease activity and the absence of advanced joint destruction. Both patients presented with an inability to actively flex their little fingers without any history of significant trauma. Based on magnetic resonance imaging and intraoperative findings, in Case 1, direct invasion by synovitis was identified as the cause. However, in Case 2, a small bone spur on the hook of hamate was responsible for the rupture. In both cases, the suspected causative synovium or bone spur was excised, and a tendon transfer surgery to the flexor digitorum profundus of the ring finger was performed. The postoperative outcomes were favourable, with improved total active motion and no re-rupture observed during follow-up. These cases highlight that flexor tendon rupture can occur even in patients with rheumatoid arthritis with low disease activity and without advanced joint destruction. Careful evaluation of both the local inflammatory activity and mechanical factors is essential to identify the cause of rupture and prevent re-rupture.
Chronic recurrent multifocal osteomyelitis (CRMO) and chronic nonbacterial osteomyelitis (CNO) are autoinflammatory diseases characterised by sterile bone inflammation. Patients with CNO or CRMO often complain about pain in the extremities, although several unusual manifestations have been reported. We report four paediatric cases of CNO/CRMO, demonstrating marked clinical variability in symptom onset and disease course. All patients presented with bone pain during their disease course, whereas unusual manifestations such as fever of unknown origin prior to the bone pain and cervical pain were confirmed. The diagnosis was established based on clinical features and by ruling out malignancy and infection through biopsy and culture. All patients received nonsteroidal anti-inflammatory drugs as initial therapy, and one required additional treatment with oral methotrexate and adalimumab. Serum cytokine analysis was performed in three cases, revealing elevated levels of both pro- and anti-inflammatory cytokines, such as interleukin-6, interleukin-10, and interleukin-33 in the pretreatment state. These cases underscore the clinical heterogeneity of CNO/CRMO.
