Immunoglobulin A vasculitis (IgAV) is a systemic small vessel vasculitis for which treatment of severe cases is usually based on glucocorticoids and other conventional immunosuppressive drugs. The ...role of rituximab for resistant or refractory cases has been explored in isolated case reports and small series.
To perform a literature review of all pediatric and adult patients with IgAV treated with rituximab (RTX) and to assess disease characteristics, RTX efficacy and safety.
Methods: We conducted a systematic literature review according to PRISMA guidelines by selecting articles with information on IgAV and RTX up to October 2019. We extracted data on patient characteristics, disease course, RTX efficacy and tolerance. The resulting database was analyzed with statistical software package SPSS v 22.0.
Among the initial 161 articles found, 20 studies including 35 well-characterized IgAV patients treated with RTX were finally analyzed. Distribution by sex was similar, and the median age at diagnosis was 26 (range: 2 months to 70 years). Patients included were equally diagnosed at pediatric age and in the adulthood. Almost 90% of patients had renal involvement before RTX treatment and resistant or refractory disease to glucocorticoids or other immunosuppressive agents, mainly with renal impairment, was the reason for RTX administration in 85.7% of patients. RTX was used because of contraindication to these previous agents in 8.6% of patients, and as first line therapy in 5.7% of them. With regard to RTX response, 94.3% of patients presented clinical improvement of any type and 74.3% achieved sustained remission at the end of follow-up. Among the 13 (37.1%) patients who experienced a disease relapse, 11 (31.4%) were treated with a new RTX dose, with good disease control in all cases. In terms of treatment requirements, glucocorticoids and additional immunosuppressants were significantly lower after RTX administration. No deaths were observed and the rate of minor RTX-associated adverse effects was of 8.6%.
RTX seems to be a safe and useful agent in inducing disease remission and reducing previous immunosuppressive treatment in IgAV pediatric and adult patients resistant or refractory to glucocorticoids or other immunosuppressive drugs, and in those patients in whom these agents are contraindicated. Nevertheless, controlled clinical trials in are still warranted to clarify the role of RTX in IgAV.
•Rituximab seems to be useful option for inducing disease remission in IgA vasculitis patients resistant or refractory to conventional treatment.•Rituximab may be a reasonable alternative for IgA vasculitis patients in whom glucocorticoids or other immunosuppressive drugs are contraindicated.•Rituximab administration in patients with IgA vasculitis has been associated with a good safety profile.•To better define the role of rituximab in IgA vasculitis, controlled clinical trials in pediatric and adult patients are still needed.
Cutaneous hypersensitivity reactions in infants present in a variety of patterns. These skin eruptions can be dramatic, causing alarm in parents and medical personnel. Many of these syndromes have ...overlapping features, which adds to the confusion and uncertainty regarding diagnosis and management. This review discusses the spectrum of hypersensitivity responses with a focus on their presentation in infants. The clinical findings, pathophysiology, histopathology, management, and complications of these conditions will be reviewed.
Background: A correlation between decreased blood coagulation factor XIII activity and the severity of organ disorders in pediatric Henoch-Schönlein purpura (HSP) has been demonstrated, but possible ...correlations in adult HSP have not been thoroughly investigated. Objectives: To investigate the association between factor XIII activity with varying clinical severities of HSP and the severity of organ disorders and to examine the efficacy of factor XIII substitution therapy. Methods: The distribution of purpura and the severities of joint, abdominal, and renal symptoms were scored in 44 adults with HSP. Plasma factor XIII activity was measured with the latex agglutination immunoturbidity method. Results: Reduced factor XIII activities were correlated with clinical severity scores (the total of all scores), organ disorder severity scores (the total score excluding the purpura score), joint symptom scores, and abdominal symptom scores but not with renal disorder scores. Factor XIII activities were increased in patients during posttreatment remission. Factor XIII substitution therapy was performed in 7 patients with severe organ disorders. Consequently, joint and abdominal symptoms markedly improved, but renal symptoms did not. Conclusion: Measurement of plasma factor XIII activity in adult HSP is clinically useful because it indicates disease severity and the severity of digestive tract and joint disorders. Factor XIII substitution therapy is effective for joint and abdominal symptoms but not for renal symptoms. Further investigation of the effect of this treatment on renal symptoms is necessary.
Adult Henoch-Schönlein purpura (HSP) is rarely associated with solid-organ malignancies. We describe here three adult patients with HSP diagnosed within 3 months of the diagnosis of associated ...solid-organ malignancies, including pulmonary, prostate, and renal carcinomas. Two patients had complete remission with a combination of immunosuppressive therapies and treatment of the associated malignancy. The third patient had partial remission with immunosuppressive therapies, but never received treatment for the associated malignancy and did not achieve complete remission before his death 10 months after diagnosis of HSP. These cases suggest that HSP associated with solid-organ malignancies may be resistant to immunosuppressive therapies without treatment of the associated malignancy. Therefore, evaluation for solid-organ malignancies should be considered in adult patients without an identifiable cause of HSP, especially if the disease is not self-limited or does not respond appropriately to treatment.
Henoch-Schönlein purpura (HSP) is a small vessel IgA-predominant vasculitis.
To describe adult patients with HSP and determine if the distribution of skin lesions (ie, purpura above the waist or ...purpura below the waist only), is a predictor of significant renal involvement at the time of the skin biopsy and the months following.
A retrospective study on renal function from 72 adult patients with skin-biopsy proven HSP. Longitudinal renal data were analyzed after HSP diagnosis by using baseline renal function for comparison.
Statistical analysis adjusted for sex, age, and baseline creatinine revealed a trend between HSP lesions only on the upper and lower extremities and long-term renal involvement (4.767, P = .067). Moreover, in another analysis adjusted for age and baseline creatinine, lesions located only on the upper and lower extremities significantly increased the odds of having long-term significant renal involvement (6.55, P = .049) in men.
This retrospective study used patient information that was subject to selection bias.
In patients with HSP, skin lesion distribution on the extremities might be predictive of significant long-term renal involvement and might be critical for risk stratification and development of personalized diagnostics and therapeutics.
Resumen El edema agudo hemorrágico del lactante (EAHL) es una vasculitis de pequeño vaso que afecta característicamente a los lactantes, con predominio en varones. Su etiología es desconocida pero ...con frecuencia se asocia a infecciones o fármacos. Las lesiones cutáneas que provoca son purpúricas y asocian edema y febrícula. Su evolución es favorable y sin secuelas, se resuelve en pocas semanas y el tratamiento es sintomático. El caso clínico que presentamos trata de un lactante varón atendido en nuestro centro de salud con episodio de otitis media aguda y posterior aparición de edema agudo hemorrágico.
Immunoglobulin A vasculitis (IgAV) is the most common vasculitis of childhood characterized by petechial or purpuric rash, abdominal pain, arthralgia, and renal involvement. Ophthalmic manifestations ...of IgAV are uncommon. Herein, we describe a case of bilateral upper eyelid erythema presenting in a 6‐year‐old male, leading to a diagnosis of IgAV.
The current study aimed to define evidence-based admission criteria of pediatric Henoch–Schonlein purpura (HSP). In addition, we aimed to better characterize epidemiological and clinical features of ...pediatric HSP in Israel. We performed a retrospective cohort study of all children with HSP admitted during a 15 years period to a single pediatric department. We strictly collected the clinical data of all HSP cases. Each case was categorized as either “necessary admission” or “unnecessary admission.” We compared the two groups, using initially Chi square (
χ
2
) and student “
t
” tests, and thereafter, we employed logistic stepwise regression analysis. One hundred and sixty-three children with HSP were included. A set of six clinical criteria of which the presence of minimum one predicts the need for hospitalization were identified including: orchitis, moderate or severe abdominal pain, arthritis involving more than two joints, proteinuria, clear evidence of gastrointestinal bleeding, and inability to ambulate. In conclusion, we suggest a predictive model for the admission of pediatric patients with acute HSP. The implementation of this model can significantly reduce unnecessary admissions.
PDF
