Henoch-Schönlein purpura nephritis associated with monoclonal gammopathy of renal significance: a case report

Monoclonal gammopathy of renal significance (MGRS) can present with different morphologic features and lead to kidney failure. The Henoch-Schönlein purpura nephritis (HSPN) that cannot be relieved by treatment with glucocorticoid and immunosuppressive agents suggests the presence of monoclonal gammopathy in adult patients. The present study reports on a single case of HSPN associated with IgA-κMGRS. The patient who suffered from recurrent skin purpura for 6 months and nephrotic syndrome for 2 months was admitted to our hospital. Bone marrow biopsy showed monoclonal gammopathy of undetermined significance. Kidney biopsy indicated a Henoch-Schönlein purpura nephritis (HSPN, ISKDC classified as type III) with positive staining with κ-light chain in the glomeruli and renal tubular epithelial cells. Furthermore, skin biopsy showed leukocytoclastic vasculitis and negative staining for Congo red and light chain. Given both the renal and cutaneous involvement, the patient was considered to have HSPN associated with IgA-κMGRS. The patient experienced an exacerbation in his purpura-like lesions and clinical status after treatment with glucocorticoid and immunosuppressive agents. Consequently, the patient was put on a regimen that included dexamethasone (20 mg on the 1st, 4th, 8th, and 11th days of each month, iv) and bortezomib (2.4 mg on the 1st, 4th, 8th, and 11th days of each month, iv). Eight weeks after treatment, he had complete resolution of his cutaneous purpura and his biochemical parameters improved. The latent presence of MGRS in cases of HSPN should be considered in adult patients. Increased cognizance and correct treatment options could improve patient outcomes.

Evaluation of TGF-beta1 and MCP-1 expression and tubulointerstitial fibrosis in children with Henoch-Schönlein purpura nephritis and IgA nephropathy: A clinical correlation

OBJECTIVES: Henoch-Schönlein purpura nephritis and immunoglobulin A nephropathy are two diseases with similar clinical presentations but very different prognoses. Transforming growth factor β1 and monocyte chemoattractant protein-1 have been associated with the development of tissue fibrosis. We examined the development of tubulointerstitial fibrosis and its relationship with Transforming growth factor β1 and monocyte chemoattractant protein-1 expression in these patients. METHODS: Renal tissue samples were collected by renal biopsy from 50 children with Henoch-Schönlein purpura nephritis and 50 children with immunoglobulin A nephropathy. Hematoxylin and eosin and Masson's trichrome-stained tissues were examined using light microscopy. Tubulointerstitial fibrosis was graded using the method described by Bohle et al. (1). The immunohistochemical detection of Transforming growth factor β1 and monocyte chemoattractant protein-1 expression was correlated with the tubulointerstitial fibrosis grade. Clinical Trial registration number: ZJCH-2012-0105. RESULTS: Transforming growth factor β1 and monocyte chemoattractant protein-1 expression in the renal tissues was significantly greater in the patients with immunoglobulin A nephropathy than in the patients with Henoch-Schönlein purpura nephritis (both p<0.001). The immunoglobulin A nephropathy patients had a higher tubulointerstitial fibrosis grade than the Henoch-Schönlein purpura nephritis patients (p<0.001). The tubulointerstitial fibrosis grade was in accordance with the Transforming growth factor β1 and monocyte chemoattractant protein-1 expression levels in both diseases (both p<0.001). CONCLUSION: Transforming growth factor β1 and monocyte chemoattractant protein-1 expression was associated with the development of immunoglobulin A nephropathy and Henoch-Schönlein purpura nephritis. Further studies are needed to better evaluate this association.

Púrpura de Schönlein-Henoch en la edad pediátrica / Henoch-Schönlein purpura in the pediatric age group

El Púrpura de Schönlein-Henoch es la vasculitis sistémica más frecuente de la infancia, caracterizada por el depósito de inmunocomplejos de IgA1 en vasos de pequeño ca-libre. Su tétrada clínica clásica incluye púrpura palpable no trombocitopénico, artritis no erosiva o artralgia, dolor abdominal y compromiso renal. Este último es menos frecuente en niños y es marcador de mal pronóstico. Su diagnóstico se realiza según criterios clínicos, siendo pocas veces necesaria la confirmación histológica, que cons-tituye el gold-standard, con manifestaciones de laboratorio inespecíficas. Se descri-ben variadas complicaciones de distinta severidad, tales como invaginación intestinal, hemorragia digestiva, déficit neurológico, insuficiencia respiratoria, torsión testicular, entre otras. Por esta gran variabilidad clínica constituye un importante diagnóstico diferencial en contextos clínicos altamente prevalentes en pediatría tales como el sín-drome purpúrico y el abdomen agudo. El curso en general es autolimitado y el trata-miento es sintomático una vez que se descartan complicaciones.

Henoch-Schönlein purpura is the most common systemic vasculitis in children, char-acterized by deposition of IgA1-immune-complexes in small-vessels. Its classic clin-ical tetrad includes non-thrombocytopenic palpable purpura, arthritis o arthralgia, abdominal pain and renal involvement. The latter is less frequent in children, but it is a poor prognostic marker. Diagnosis is made through clinical criteria, and in only a few cases a histological confirmation is necessary, which is the gold standard, with unspecific laboratory features. Many complications have been described, such as in-tussusception, gastrointestinal bleeding, neurological deficit, respiratory failure and testicular torsion. Because of its great clinical variability, it constitutes an important differential diagnosis in highly prevalent pediatric clinical scenarios, such as purpuric syndrome and acute abdomen. It is usually self-limited, and its treatment is focused in symptom relief once complications are ruled out.

Clinical features and outcomes of diffuse endocapillary proliferation Henoch-Schönlein purpura nephritis in children

OBJECTIVE: To investigate the outcomes of childhood diffuse endocapillary proliferation Henoch-Schönlein purpura nephritis (DEP-HSPN) in response to early diagnosis and prompt treatment. METHODS: Eleven cases of DEP-HSPN in children were investigated in comparison to HSPN without diffuse endocapillary proliferation (non-DEP-HSPN). RESULTS: DEP-HSPN had a higher prevalence of nephrotic syndrome but a lower prevalence of hematuria compared to non-DEP-HSPN. IgA, IgG and IgM antibody deposition was found in DEP-HSPN by histopathological examination. Proteinuria cleared in all 11 cases through treatment with steroids and/or immunosuppressive drugs. However, half of the DEP-HSPN patients continuously had hematuria after treatment. CONCLUSION: The early diagnosis and prompt initiation of immunosuppressive treatment based on renal biopsy are important for achieving favorable outcomes.

Púrpura de Schõnlein- Henoch en adultos / Henoch-Schõnlein purpura in adults

La púrpura de Schõnlein-Henoch es una vasculitis sistémica mediada por inmunoglobulina A, caracterizada clínicamente por púrpura palpable, dolor abdominal, artralgias y hematuria o proteinuria en el laboratorio. La inmunofluorescencia directa de las lesiones cutáneas, así como la del riñón, revelan la presencia de depósitos perivasculares de este anticuerpo, dato de valor para confirmar el diagnóstico. Se presentan cinco pacientes adultos con PSH, insuficiencia renal aguda y neoplasias malignas en tres de ellos.

Henoch-Schönlein Purpura is a systemic vasculitis mediated by Ig A, clinically characterizedby non-thrombocytopenic palpable purpura, abdominal pain, arthritis and proteinuria orhematuria. Histologically, it is characterized by deposition of immunoglobulin A in the skinand kidneys, being these features essential for the diagnosis. We report five adult patientswith Henoch-Schönlein purpura with acute kidney failure, and malignant neoplasms onthree of them.

An aetiological & clinicopathological study on cutaneous vasculitis.

Background & objectives: Cutaneous vasculitis has protean clinical manifestations. It may be idiopathic or associated with a spectrum of conditions such as infections, drugs, etc. Skin is involved in both small vessel vasculitis (SVV) and medium vessel vasculitis (MVV). Overlapping features are seen between SVV and MVV. The histopathological features may not always relate with the clinical lesions. The aim of the present study was to evaluate the aetiological factors and clinicopathological association in patients with cutaneous vasculitis. Methods: In this cross-sectional study, detailed history and clinical examination were done on patients with biopsy proven cutaneous vasculitis. Two skin biopsies were taken from each patient for routine histopathology and direct immunofluorescence. Results: Of the 61 patients studied, hypersensitivity vasculitis (HSV) [23 (37.7%)] and Henoch Schonlein purpura (HSP) [16 (26.2%)] were the two most common forms. Systemic involvement was seen in 32 (52.45%) patients. Drugs were implicated in 12 (19.7%) cases, infections in 7 (11.4%) and connective tissue disorders in 4 (6.5%) cases. Histologically SVV was the most common pattern, seen in all the clinically diagnosed patients with SVV (47), and in 12 of the 14 clinically diagnosed patients with MVV. Direct immunofluorescence showed positivity for at least one immunoreactant in 62 per cent of the patients and the most common deposit was C3 followed by IgG, IgA and IgM. Interpretation & conclusions: Majority of our patients with cutaneous vasculitis were idiopathic. Histologically, SVV was seen in most of our patients. No association was seen between history of drug intake and tissue eosinophilia and also between histologically severe vasculitis and clinical severity. The presence of immunoreactant IgA was not specific for HSP.

Edema agudo hemorrágico da infância: relato de três casos / Acute hemorrhagic edema of infancy: report of three cases

O Edema Agudo Hemorrágico da Infância é uma vasculite leucocitoclástica pouco frequente, que ocorre, quase exclusivamente, em crianças entre 4 meses e 2 anos de idade. Caracteriza-se, clinicamente, pela tríade febre, lesões purpúricas na face, pavilhões auriculares e extremidades e edema. Embora os achados cutâneos sejam dramáticos e de surgimento rápido, o prognóstico é favorável, com resolução espontânea dentro de 1 a 3 semanas. Descrevem-se três casos cujos achados clínicos e histopatológicos são característicos de edema agudo hemorrágico da infância.

Acute Hemorrhagic Edema of Infancy is an infrequent leukocytoclastic vasculitis which occurs almost exclusively in children between 4 months and 2 years of age. It is clinically characterized by the triad fever, purpuric lesions on the face, auricular pinna and extremities, and edema. Although the cutaneous findings are dramatic and of rapid onset, the prognosis is favorable, with spontaneous resolution within 1 to 3 weeks. Three cases are described in which clinical and histopathological findings are characteristic of acute hemorrhagic edema of infancy.

Study on epidemiological, clinical and laboratory characteristics of children with Henoch-Schoenlein Purpura

Henoch-Schoenlein Purpura [HSP] is the most common vasculitiis in children. It is characterized by non-thrombocytopenic purpura, arthritis and arthralgia, abdominal pain, gastrointestinal hemorrhage and glomerulonephritis. This study was conducted to determined epidemiological and clinical manifestations and laboratory findings of children who admitted with HSP at university hospitals of Ahvaz. Information about the epidemiological, clinical and paraclinical characteristics of patients were selected from hospital records of all 63 children with final diagnosis of HSP who were admitted in pediatric wards in university hospitals of Ahvaz during February 2001 to February 2008. Data analyzed by SPSS software. There were 63 children, 41[65%] males and 22 [35%] females [M/F ratio: 1.86/1]. The mean age of the patients was 6.4 [ +/- 3.15] years. Previous history of upper respiratory tract infections was found in 42.9% of the patients. In the review of clinical features, skin manifestations, gastrointestinal symptoms, arthritis/arthralgia and evidences of renal involvement were found in 100, 76, 66.7 and 39.6% of cases, respectively. The neurological signs were not found. Anemia, gastrointestinal bleeding, nephrotic syndrome and intussusception were found in 47.6, 19, 6.3 and 3.2% of cases, respectively. We found that relative to other studies, gastrointestinal manifestations in our patients were more common and more severe than arthicular manifestations. But, the final prognosis was good with no mortality

Púrpura de Schõnlein-Henoch de presentación en el adulto / Schõnlein-Henoch purpura in the adult

La púrpura de Schõnlein-Henoch es una vasculitis de pequeños vasos que afecta principalmente a niños, se presenta en adultos con poca frecuencia, en estos casos puede tener un curso desfavorable. Se presentó el caso de una paciente de 46 años de edad que evolucionó durante 4 meses con brotes de púrpura palpable y trastornos gastrointestinales, sin respuesta al tratamiento con prednisona oral. Se evidenció el valor de la inmunofluorescencia para confirmar el diagnóstico, así como la necesidad de indicar tratamiento inmunosupresor en algunos casos.

Schõnlein-Henoch purpura is a vasculitis affecting small vessels mainly in children. It is a rare disease in adults that may have an unfavourable course. Here is the case of a 46 years-old patient who evolved into palpable purpura rashes and gastrointestinal disorders for 4 months, without clear response to oral prednisone-based treatment. The value of immunofluorescence for confirming diagnosis as well as the need of immunosupressive therapy in some cases were evidenced.

Púrpura de Scholein-Henoch en el adulto / Scholein-Henoch purpura in an adult

La púrpura de Scholein-Henoch (PSH) es una vasculitis autolimitada que afecta a los pequeños vasos. Si bien su etiopatogenia es desconocida, se reconocen algunos factores desencadenantes. Es mediada por inmunocomplejos a predominio de IgA. Clínicamente se caracteriza por la tétrada consistente en púrpura palpable no trombocitopénica, artritis, nefritis y enteropatía. Aparece con mayor frecuencia en la primera y segunda década de la vida y es poco usual en adultos. El tratamiento se basa en medidas de sostén y en ocasiones corticoides, siendo fundamental el control de la función renal a largo plazo. Se presenta un caso y se realiza una revisión de la literatura sobre esta patología

Orquite da púrpura de Henoch-Schonlein / Orchitis of Henoch-Schonlein purpura

A púrpura de Henoch-Schonlein (PHS), também conhecida como púrpura anafilactóide ou púrpura reumática, é a vasculite mais frequente na faixa etária pediátrica. Geralmente, a doença é benigna, com a manifestação clínica característica - púrpura palpável não plaquetopênica, presente em todos os pacientes, e de localização simétrica em membros inferiores e nádegas...

Fasceitis necrotizante y púrpura fulminante por estreptococo del grupo A secundario a varicela en un caso pediátrico / Necrotizing fasciitis and purpura fulminans due to type a group streptoccocus secondary to chicken pox in a pediatric case

La Fasceitis Necrotizante (FN) y la Púrpura Fulminate (PF) son un grupo de patologías potencialmente incapacitantes y amenazadoras de la vida. Se presenta el caso de un preescolar masculino de 2 años de edad, con varicela, quien presentó signos de flogosis en la región lumbar, de rápida progresión a tórax con centro necrótico, concomitante con necrosis de párpado superior derecho y deterioro del estado general. En los paraclínicos presentó leucocitosis con neutrofilia, trombocitopenia y tiempos de coagulación prolongados. Se realizó necrectomía amplia e ingresó a la Unidad de Terapia Intensiva Pediátrica (UTIP), donde evolucionó a disfunción multiorgánica. A las 72 horas aparecen lesiones purpúricas en dedos de los pies y mano derecha, compatible con FP asociada a sepsis. La biopsia de piel reveló hallazgos compatibles con FN. Se hace una revisión de la FN y PF por Estreptococo del grupo A secundario a varicela

Púrpura de Schönlein-Henoch. Recidiva en el trasplante renal. Presentación de 1 caso / Schönlein-Henoch purpura. Recurrence in the kidney transplant. A case report

Se señaló que la afección renal marca el pronóstico en los pacientes con púrpura de Schönlein-Henoch, pero es rara en adultos y se reporta con poca frecuencia en los receptores de trasplante renal en estas edades. Se presentó el caso de una paciente con antecedentes de nefritis por púrpura de Schönlein-Henoch y pérdida de la función renal, con recidiva de dicha lesión en el riñón trasplantado, de una manera precoz e intensa. Se describió su evolución, hallazgos histológicos y biópsicos. Se comentaron algunas características de esta afección

Rapidly progressive antineutrophil cytoplasm antibodies associated with pulmonary-renal syndrome in a 10-year-old girl

CONTEXT: The term pulmonary-renal syndrome has been used frequently to describe the clinical manifestations of a great number of diseases in which pulmonary hemorrhage and glomerulonephritis coexist. The classic example of this type of vasculitis is Goodpasture´s syndrome, a term used to describe the association of pulmonary hemorrhage, glomerulonephritis and the presence of circulating antiglomerular basement membrane antibodies (anti-GBM). Among the several types of systemic vasculitides that can present clinical manifestations of the pulmonary-renal syndrome, we focus the discussion on two types more frequently associated with antineutrophil cytoplasm antibodies (ANCA), microscopic polyangiitis and Wegener´s granulomatosis, concerning a 10 year old girl with clinical signs and symptoms of pulmonary-renal syndrome, with positive ANCA and rapidly progressive evolution. CASE REPORT: We describe the case of a 10-year-old girl referred to our hospital for evaluation of profound anemia detected in a primary health center. Five days before entry she had experienced malaise, pallor and began to cough up blood-tinged sputum that was at first attributed to dental bleeding. She was admitted to the infirmary with hemoglobin = 4 mg/dL, hematocrit = 14 percent, platelets = 260,000, white blood cells = 8300, 74 percent segmented, 4 percent eosinophils, 19 percent lymphocytes and 3 percent monocytes. Radiographs of the chest revealed bilateral diffuse interstitial alveolar infiltrates. There was progressive worsening of cough and respiratory distress during the admission day, when she began to cough up large quantities of blood and hematuria was noted. There was rapid and progressive loss of renal function and massive lung hemorrhage. The antineutrophil cytoplasm antibody (ANCA) test with antigen specificity for myeloperoxidase (anti-MPO) was positive and the circulating anti-GBM showed an indeterminate result

Púrpura de Scholein-Henoch: evaluación de once pacientes pediátricos / Schoenlein-Henöch purpura: evaluation of eleven pediatric cases

Se presentan once pacientes afectados de síndrome de Schonlein-Henoch, lo que motivó a su internación en el Servicio de Pediatría de nuestro hospital. Las edades oscilaron entre 4 y 15 años, el período de estudio fue de 3 años (1994-1996). Se evalúan los factores etiológicos involucrados, las características clínicas, la evolución y el tratamiento