Ultrasound-Based Radiomics for the Classification of Henoch-Schönlein Purpura Nephritis in Children.

Henoch-Schönlein purpura nephritis (HSPN) is one of the most common kidney diseases in children. The current diagnosis and classification of HSPN depend on pathological biopsy, which is seriously limited by its invasive and high-risk nature. The aim of the study was to explore the potential of radiomics model for evaluating the histopathological classification of HSPN based on the ultrasound (US) images. A total of 440 patients with Henoch-Schönlein purpura nephritis proved by biopsy were analyzed retrospectively. They were grouped according to two histopathological categories: those without glomerular crescent formation (ISKDC grades I-II) and those with glomerular crescent formation (ISKDC grades III-V). The patients were randomly assigned to either a training cohort (n = 308) or a validation cohort (n = 132) with a ratio of 7:3. The sonologist manually drew the regions of interest (ROI) on the ultrasound images of the right kidney including the cortex and medulla. Then, the ultrasound radiomics features were extracted using the Pyradiomics package. The dimensions of radiomics features were reduced by Spearman correlation coefficients and least absolute shrinkage and selection operator (LASSO) method. Finally, three radiomics models using k-nearest neighbor (KNN), logistic regression (LR), and support vector machine (SVM) were established, respectively. The predictive performance of such classifiers was assessed with receiver operating characteristic (ROC) curve. 105 radiomics features were extracted from derived US images of each patient and 14 features were ultimately selected for the machine learning analysis. Three machine learning models including k-nearest neighbor (KNN), logistic regression (LR), and support vector machine (SVM) were established for HSPN classification. Of the three classifiers, the SVM classifier performed the best in the validation cohort [area under the curve (AUC) =0.870 (95% CI, 0.795-0.944), sensitivity = 0.706, specificity = 0.950]. The US-based radiomics had good predictive value for HSPN classification, which can be served as a noninvasive tool to evaluate the severity of renal pathology and crescentic formation in children with HSPN.

Contrast-enhanced ultrasonography for assessing histopathology in pediatric immunoglobulin A nephropathy and Henoch-Schönlein purpura nephritis.

BACKGROUND: Glomerular disease, including immunoglobulin A nephropathy (IgAN) and Henoch-Schönlein purpura nephritis, is one of the most common kidney diseases in children. The diagnosis of these diseases depends on pathological biopsy, although this procedure is seriously limited by its invasive and high-risk nature. OBJECTIVE: To investigate the potential of contrast-enhanced ultrasonography (CEUS) for evaluating the histopathological severity of IgAN and Henoch-Schönlein purpura nephritis (HSPN). MATERIALS AND METHODS: We investigated a total of 13 children with IgAN and 12 children with HSPN confirmed by renal histopathology. We reevaluated the pathological lesions of the children according to the Oxford classification and the Lee grading system and then all the children underwent CEUS. Using SonoLiver software, we constructed time-intensity curves of CEUS for regions of interest in the renal cortex. We analyzed CEUS quantitative parameters for IgAN and HSPN and used Spearman correlation analysis to examine the correlation between CEUS parameters and clinicopathological indexes in the study cohort. RESULTS: The CEUS parameters rise time (RT) and time to peak (TTP) were significantly higher in children with Lee grade IV than in those with Lee grades II or III. Spearman correlation analysis revealed a positive correlation between rise time and time to peak with Lee grade in the overall cohort of children, and a positive correlation between rise time and time to peak and severity of crescents in the Oxford classification scoring system. CONCLUSION: Contrast-enhanced US may be used as a noninvasive imaging technique to evaluate the severity of renal pathology and formation of crescents in children with IgAN and HSPN.

Gastrointestinal involvement in adult IgA vasculitis (Henoch-Schönlein purpura): updated picture from a French multicentre and retrospective series of 260 cases.

OBJECTIVES: To describe the clinical presentation, treatments and prognosis of gastrointestinal (GI) involvement in adult IgA vasculitis (IgAV). METHODS: Data from 260 adults with IgAV included in a French multicentre retrospective survey were analysed. Presentation and outcomes of patients with (GI+) and without (GI-) GI involvement were compared. RESULTS: One hundred and thirty-seven (53%) patients had GI involvement. Initial manifestations were abdominal pain in 99%, intestinal bleeding in 31%, diarrhoea in 26% and acute surgical abdomen in only 4%. Abdominal imaging revealed thickening of intestinal wall in 61%, and endoscopies revealed abnormalities in 87%, mostly mucosal ulcerations. GI+ vs GI- patients were younger (46 ± 18 vs 54 ± 18 years; P = 0.0004), had more constitutional symptoms (43% vs 23%; P = 0.0005) and joint involvement (72 vs 50%; P = 0.0002), and higher CRP levels (3.7 vs 1.9 mg/dl; P = 0.001). Clinical response and relapse rates were comparable between groups, and all causes mortality (2 vs 4%) and IgAV-related mortality (1% vs 2%) as well. GI-related deaths were due to intestinal perforation and mesenteric ischaemia. CONCLUSION: GI involvement is frequent in adult IgAV. GI involvement is frequent in adult IgAV. Mortality is not uncommon but does not seem to be specifically related to GI. Immunosuppressants should not be preferred as first-line therapy for GI+ patients but may be required in case of acute surgical abdomen.

Effect of intraocular anti-VEGF on cystoid macular edema associated with Henoch-Schonlein purpura-a case report.

BACKGROUND: To report a bilateral cystoid macular edema associated with Henoch-Schonleinpurpura. CASE PRESENTATION: A 21-year-old man presented a bilateral, painless, and bilateral blurred vision for 5 weeks with visual acuity (VA) of 6/12 on the right eye and 6/48 on the left. FA and OCT showed bilateral cystoid macular edema, and the fundus photograph showed retinal hemorrhages. Using intravenous dexamethasone could reduce macular edema, but it reoccurred shortly after switching to oral prednisone. Repeated intraocular injection of anti-VEGF in both eyes was performed and VA improved to 6/6 on the right eye and 6/7.5 on the left with the regression of edema after 6 months follow-up. CONCLUSIONS: Intraocular anti-VEGF might be an alternative choice to glucocorticoid in cases of bilateral cystoid macular edema associated with Henoch-Schonlein purpura.

Coronary artery thickening with mucosal lesions in Henoch-Schönlein purpura.

Henoch-Schönlein purpura (HSP) is the most common vasculitis of childhood. It is usually self-limiting and is characterized by an immune complex-mediated vasculitis associated with IgA deposition. We present an unusual case of HSP with mucosal lesions and coronary artery thickening.

Intramuscular Hematoma as a Manifestation of IgA Vasculitis.

We describe an atypical pediatric case of immunoglobulin A vasculitis (IgAV), also referred to as Henoch-Schönlein purpura, in which formation of spontaneous hematoma of the paraspinal muscles developed. Spontaneous or unprovoked hematomas rarely occur in IgAV. These manifestations have not been described specifically in the pediatric literature as coinciding with IgAV. These findings are alarming for nonaccidental trauma, particularly in a patient without underlying blood dyscrasia. Our objective for this report is to highlight the possible association of muscular hematoma formation with IgAV and to help providers consider this association when trauma and hemophilia has been ruled out.

Recurrent Henoch-Schönlein Purpura with bullous rash and pulmonary nodules.

BACKGROUND: Henoch-Schönlein purpura (HSP) is the most common vasculitis of childhood. It has a characteristic rash described as palpable purpura that most frequently affects the distal lower extremities and buttocks. HSP rarely presents with bullous rash nor pulmonary nodules. CASE PRESENTATION: We present a novel case of a 12-years-old female with recurrent pediatric HSP with a combination of the rare manifestations of bullous rash and pulmonary nodules. She initially presented with the bullous rash, chest pain, cough, and abdominal pain. Patient was successfully treated with intravenous pulse corticosteroids followed by a high dose oral corticosteroid taper, with resolution of the bullous rash and pulmonary nodules. CONCLUSION: The rare manifestations of scarring bullous rash and pulmonary nodules can be presenting features of pediatric HSP, the combination of which has not been previously reported. The treatment of intravenous corticosteroid resolved patient's abdominal symptoms, rash and pulmonary nodules.

Significance of bowel wall abnormalities at ultrasound in Henoch-Schönlein purpura.

OBJECTIVES: To describe the polymorphism of intestinal ultrasound findings in children with gastrointestinal symptoms of Henoch Schönlein purpura (HSP) and to investigate the clinical relevance of these findings in terms of symptoms and length of hospitalization. PATIENTS AND METHODS: Clinical and ultrasound data from 43 consecutive children with HSP (36 with and 7 without abdominal symptoms) were reviewed. Patients with abdominal symptoms of HSP were divided into 4 groups (0-III) representing at ultrasound normal appearance and differentiated, pseudodifferentiated, and dedifferentiated bowel wall thickening, respectively. The diagnostic value of ultrasound in diagnosing gastrointestinal involvement of HSP (grades I-III) was calculated using as the standard of reference the absence or presence of clinical symptoms. Average duration of symptoms and hospitalization in the 4 groups was compared. RESULTS: The respective sensitivity, specificity, positive predictive value, and negative predictive value of ultrasound for the diagnosis of gastrointestinal involvement of HSP was 83.3%, 100%, 100%, and 53.8%. Groups 0 to III contained, respectively, 5/36 (13.9%), 6/36 (16.7%), 22/36 (61.1%), and 3/36 (8.3%) patients. The groups' average duration of symptoms, respectively, was 2.20 +/- 2.06, 5.67 +/- 1.88, 6.29 +/- .94, and 17.67 +/- 2.66 days, whereas the length of hospitalization, respectively, was 4.80 +/- 2.96, 9.17 +/- 2.70, 11.46 +/- 1.35, and 24.67 +/- 3.82 days. The duration of both symptoms and hospitalization was significantly higher in group III than in the other groups (P < 0.05). CONCLUSIONS: In children with gastrointestinal involvement of HSP, dedifferentiated wall thickening as shown by ultrasound is associated with a poor clinical prognosis.

Pulmonary hemorrhage associated with Henoch-Schönlein purpura in a child.

Swelling of the right ankle and purpura on the bilateral lower extremities of a 6-year-old boy indicated a diagnosis of Henoch-Schönlein purpura (HSP). The patient was referred to our hospital because of severe abdominal pain that was unresponsive to prednisolone. Respiratory symptoms and pulmonary infiltrates on chest X-ray were absent upon admission. Methylprednisolone pulse therapy gradually improved the abdominal symptoms, but tachypnea developed, and insufficient aeration of the right lung was accompanied by a decline in percutaneous oxygen saturation from 99% to 90% and a rapid decrease in hemoglobin levels from 11.7 to 7.6 g/dL. Chest X-rays and high-resolution computed tomography scans showed widespread consolidation and patchy opacities predominantly in the right lung fields, suggesting acute pulmonary hemorrhage. Intravenous cyclosporin A (CyA) gradually resolved the pulmonary hemorrhage and respiratory insufficiency. Pulmonary hemorrhage, as a complication of HSP, is extremely rare and sometimes fatal. Aggressive steroid and immunosuppressive treatment is required to address severe complications of HSP.

Characterization of scrotal involvement in children and adolescents with IgA vasculitis.

OBJECTIVE: To characterize scrotal involvement in children and adolescents with IgA vasculitis. METHODS: A cross-sectional retrospective study included 296 IgA vasculitis (EULAR/PRINTO/PRES criteria) patients, 150/296 (51%) were males and assessed by demographic/clinical/laboratory and treatments. Scrotal involvement was defined by the presence of scrotal edema and/or pain/tenderness in physical examination and/or testicular Doppler ultrasound abnormalities. RESULTS: Scrotal involvement was observed in 28/150 (19%) IgA vasculitis patients. This complication was evidenced at IgA vasculitis diagnosis in 27/28 (96%). Acute recurrent scrotal involvement was observed in 2/150 (1%) and none had chronic subtype. Further analysis of patients with scrotal involvement at first episode (n = 27) compared to those without this complication (n = 122) revealed that the median age at diagnosis [4.0 (2.0-12) vs. 6 (1.3-13) years, p = 0.249] was similar in both groups. The frequency of elevated serum IgA was significantly lower in IgA vasculitis patients with scrotal involvement versus without this manifestation (18% vs. 57%, p = 0.017), whereas glucocorticoid (93% vs. 49%, p < 0.0001) and ranitidine use (63% vs. 30%, p = 0.003) were significantly higher in the former group. CONCLUSIONS: The scrotal involvement occurred in almost one fifth of IgA vasculitis patients and was commonly evidenced as acute subtype at diagnosis. Scrotal signs/symptoms improved after a prompt use of glucocorticoid and was associated with low frequency of elevated IgA serum levels.

Imaging findings in systemic childhood diseases presenting with dermatologic manifestations.

PURPOSE: Many childhood diseases often present with skin abnormalities with which radiologists are largely unfamiliar. Knowledge of associated dermatologic manifestations may aid the radiologist in confirming the diagnosis and recommending targeted imaging of affected organs. METHODS: We review the imaging findings in childhood diseases associated with dermatologic manifestations. FINDINGS: Diseases include dermatologic findings which herald underlying malignancy (Neuroblastoma, leukemia/lymphoma, Langerhans cell histiocytosis),are associated with risk of malignancy (Epidermolysis Bullosa, basal cell nevus syndrome, Cowden's syndrome, Tuberous Sclerosis),or indicate a systemic inflammatory/immune disorder (Kawasaki's disease, Henoch Schonlein Purpura, systemic lupus erythematosus, scleroderma, sarcoidosis, dermatomyositis and immune thrombocytopenic purpura). CONCLUSION: Familiarity with pertinent findings in childhood diseases presenting with dermatologic manifestations in childhood diseases aids the radiologist in confirming the diagnosis and guiding imaging workup.

Accumulation of Tc-99m-HMPAO-labeled leukocytes in cutaneous lesions of Henoch-Schonlein purpura.

A 70-year-old man with complicated cruris fracture, treated with an arthrodesis and skin transplantation, complained about persistent pain of the right ankle and skin rashes. Bone and leukocyte scintigraphy were performed because osteomyelitis was suspected. Bone scintigraphy showed abnormal uptake in the ankle. Leukocyte scintigraphy showed only multiple hot spots in the soft tissue. A cutaneous biopsy specimen revealed Henoch-Schonlein vasculitis.

Fludeoxyglucose positron emission tomography-computed tomography scan showing polyarthritis in a patient with an atypical presentation of Henoch-Schönlein vasculitis without clinical signs of arthritis: a case report.

BACKGROUND: Henoch-Schönlein vasculitis is the most common systemic vasculitis in children. Arthritis or arthralgia occurs in 80 % of patients. We believe this to be the first case report to describe the finding of polyarthritis in a fludeoxyglucose positron emission tomography-computed tomography scan in a patient with Henoch-Schönlein vasculitis without clinical signs of arthritis. CASE PRESENTATION: A 4.5-year-old Caucasian boy presented with fever of 4 days' duration followed by debilitating migratory arthralgia and inflammation. He underwent a fludeoxyglucose positron emission tomography-computed tomography scan to exclude a possible malignant cause or to detect any infectious or autoimmune focus of his symptoms. Fludeoxyglucose uptake was observed in multiple large joints and in multiple tendons. These findings suggested active polyarthritis and polytendinitis. However, physical and ultrasound evaluations did not show any signs of arthritis in our patient, despite his evident arthralgia. CONCLUSIONS: Fludeoxyglucose positron emission tomography-computed tomography might be able to detect inflammatory activity in painful joints that cannot yet be detected clinically or with ultrasound evaluation in a patient with Henoch-Schönlein vasculitis. Therefore, fludeoxyglucose positron emission tomography-computed tomography can be of additional value in the diagnostic workup of patients with an unresolved diagnosis of suspected autoimmune disease, especially in patients with unresolved arthralgia and fever of unknown cause.

Vasculitis IgA en el adulto. Informe de caso / IgA vasculitis in adults. Case report

RESUMEN La vasculitis IgA, también conocida como púrpura de Schönlein-Henoch, es una vasculitis leucocitoclástica que involucra pequeños vasos con depósito de inmunocomplejos IgA. Puede abarcar piel, articulaciones, riñones y tracto gastrointestinal. Su presentación en adultos es rara, y las formas clínicas suelen ser más agresivas. Es objetivo del presente trabajo describir el curso y evolución de vasculitis IgA, en un paciente de 59 años, con púrpuras en miembros inferiores y tronco, hematuria macroscópica, y edema de miembros inferiores. Los complementarios mostraron creatininas elevadas, proteinuria de rango nefrótico, elevación de la IgA y anticuerpos contra el citoplasma de los neutrófilos negativos. Se descartaron causas neoplásicas. El estudio anatomo-patológico del riñón concluyó una vasculitis IgA.

ABSTRACT IgA vasculitis, also known as Henoch-Schönlein purpura, is a leukocytoclastic vasculitis that involves small vessels with deposition of IgA immune complexes. It can include skin, joints, kidneys, and gastrointestinal tract. Its presentation in adults is rare, and the clinical forms are usually more aggressive. The objective of this study is to describe the course and evolution of IgA vasculitis, in a 59-years-old patient, with purples in the lower limbs and trunk, macroscopic hematuria, and lower limb edema. The complementary ones showed elevated creatinines, nephrotic range proteinuria, elevated IgA and negative antibodies against the cytoplasm of neutrophils. Neoplastic causes were dismissed. The anatomical-pathological study of the kidney concluded IgA vasculitis.

Henoch-Schönlein purpura as a clinical presentation of small cell lung cancer.

Although exceptionally, Henoch-Schönlein purpura (HSP) may appear as a paraneoplastic syndrome. We report a patient presenting with HSP. A chest X-ray showed a pulmonary nodule, while biopsy of a transthoracic needle aspiration revealed small cell lung cancer. To the best of our knowledge, HSP as a clinical presentation of small cell lung cancer has not been previously reported.

Diagnosis of Henoch-Schönlein purpura by sonography and radionuclear scanning in a child presenting with bilateral acute scrotum.

Acute scrotum presenting as the only initial manifestation of Henoch-Schönlein purpura (HSP) is so unusual that the diagnosis can easily be missed. We report this condition in a 4-year-old boy admitted with bronchopneumonia. Bilateral painful scrotal swelling with ecchymosis occurred on the second day of hospitalization. Scrotal sonography was performed and a good blood supply was documented. Scrotal nuclear scanning was performed and was consistent with bilateral epididymoorchitis. Multiple purpuric lesions over the lower extremities and perineal region developed on the third day of hospitalization. Intermittent abdominal pain and knee pain developed thereafter. HSP was diagnosed and steroids were prescribed. The symptoms subsided gradually and no complication was noted. This case reminds us that an acute scrotum may be the only initial manifestation of HSP. Sonography and nuclear scanning can help rule out other diseases.

The value of colour Doppler ultrasonography for small bowel involvement of adult Henoch-Schönlein purpura.

Three patients presented with a non-thrombocytopenic purpuric rash on their upper and lower limbs, abdominal pain, diarrhoea, and arthralgia. Grey scale ultrasound showed abnormally thickened walls of the small bowel. Colour Doppler showed blood flow signals in the diseased bowel wall in all patients. Subsequent barium and endoscopic studies showed oedematous bowel loops with petechial lesions. Biopsy from the purpuric rash of the skin demonstrated vasculitis of subdermal small vessels. The clinical diagnosis of Henoch-Schönlein purpura was made in each case. This paper describes the efficacy of grey scale and colour Doppler ultrasonography in the assessment of the small bowel involvement of Henoch-Schönlein purpura.