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Results for “IgA Vasculitis”

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At least 19 recordsLinked to original sources
PubMed2026

IgA Vasculitis with necrotizing arteritis: a multicenter retrospective study from the French Vasculitis Study Group and systematic review of the literature.

IgA vasculitis (IgAV) primarily affects small vessels, but rare cases with necrotizing arteritis (NA) raise questions about overlap with polyarteritis nodosa (PAN). To characterize IgAV with necrotizing arteritis (IgAV-NA) and compare its phenotype with classical IgAV and PAN. We performed a multicenter retrospective study combined with a systematic literature review (1990-2025). Patients fulfilled EULAR/PRINTO/PRES IgAV criteria, had pathological or imaging evidence of NA in small or medium arteries, and were ANCA-negative. Thirty patients were included (7 from databases, 23 from the literature). NA was confirmed by biopsy (n = 16) or vascular imaging (n = 14). Clinical features, treatments, remission, and mortality were compared with 257 adult IgAV and 196 PAN patients. Median age was 54.5 years. IgAV-NA was characterized by severe manifestations, including gastrointestinal bleeding, perforation, surgical abdomen, neuropathy, pancreatitis, and livedo. Compared with classical IgAV, IgAV-NA showed significantly higher rates of multi-organ involvement and mortality. Compared with PAN, IgAV-NA shared vascular complications but had less fever and neuropathy. Despite arterial involvement, patients did not fulfil PAN criteria. IgAV-NA represents a rare, severe IgAV phenotype with life-threatening complications rather than an IgAV-PAN overlap. Severe or atypical IgAV presentations should prompt vascular imaging and intensified immunosuppression.

Humans↗
PubMed1992

Urticarial vasculitis, IgA deficiency and C1 esterase inhibitor deficiency in the presence of an IgG monoclonal gammopathy--a case report.

A female patient, currently aged 40, has attended our Lupus Clinic for 8 years with a clinical picture of urticarial vasculitis, joint pains, severe myalgias and abdominal pain which has occurred in bouts at irregular intervals. She has been found to have consistently reduced C4 levels, C1-esterase inhibitor deficiency and a persistent monoclonal band in the gamma region on electrophoresis. Her serum IgA level has been continuously low. Her symptoms have failed to respond to antihistamines and a variety of other measures but have been partially alleviated by immunosuppressive therapy ('pulsed' cyclophosphamide). We believe that this is the first patient described showing this combination of clinical and laboratory features.

Adult↗
PubMed1993

Cutaneous vasculitis and IgA glomerulonephritis in ankylosing spondylitis.

Two patients with ankylosing spondylitis were found to have IgA nephropathy and leucocytoclastic cutaneous vasculitis. Immunofluorescence showed perivascular deposition of IgA in the skin of one patient and in the mesangium of both patients. Such an association has been reported only once before. This supports the concept of abnormal IgA immune stimulation in the pathogenesis of ankylosing spondylitis.

Glomerulonephritis, IGA↗
PubMed2001

Leukocytoclastic vasculitis with IgA deposits in angioimmunoblastic T cell lymphoma.

Angioimmunoblastic T cell lymphoma (AILD) is a type of peripheral T cell lymphoma associated with fever and generalized lymphadenopathy. Cutaneous manifestations are seen in approximately 40% of the patients. We report herein a Japanese male patient with AILD associated with generalized purpura. The histology of the purpura included leukocytoclastic vasculitis with IgA deposits, which is rare in this disease. Using in situ hybridization and PCR methods, we showed that the involved lymph node was positive for Epstein-Barr virus and that the purpura was negative.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2005

Cutaneous IgA-associated vasculitis induced by alcohol.

A patient with elevated levels of serum IgA developed purpuric lesions histologically resembling Henoch-Schönlein purpura brought on by consuming alcohol. Alcohol challenge with 5 units of alcohol reproduced the lesions, with a rapid rise of circulating CD4+ and CD8+ T cells followed by a fall of serum IgA and C3 concentration. The skin lesions and serum abnormalities resolved spontaneously within 6 weeks of the alcohol challenge.

Adult↗
PubMed1994

[Schoenlein-Henoch syndrome in adults--current aspects].

Schoenlein-Henoch purpura is characterized clinically by palpable purpura, mainly on the legs, with varying degrees of gastrointestinal, articular and renal involvement. Renal involvement is often more severe in the adult than in children and it often determines the outcome of the disease. Schoenlein-Henoch purpura is also characterized by an IgA-vasculitis, which may be triggered by several non-specific antigenic factors. Its pathogenesis is still unclear, but a dysfunction of the IgA immune system has been demonstrated. We discuss the anatomo-clinical, therapeutic and etiopathogenic aspects of this syndrome according to today's knowledge.

Combined Modality Therapy↗
PubMed1999

Henoch-Schönlein nephritis in children and adults. Morphological features and clinicopathological correlations.

Rheumatoid purpura or Henoch-Schönlein syndrome is an IgA vasculitis affecting small vessels. The acute disease progresses by successive flare-ups of limited duration. Long-term prognosis depends mainly on the degree of initial renal damage. A review of the literature shows that renal involvement occurs in about 33% of children and 63% of adults with rheumatoid purpura. The most typical manifestation is segmentary focal glomerulonephritis, always associated with granulous IgA deposits in the mesangium. When renal signs are severe enough to warrant renal biopsy (generally at urine protein > 1 g/d and/or organic renal failure) the risk of developing chronic renal failure is 18% in children and 28% in adults. The best prognostic features are histological. The percentage of crescents, the presence of interstitial fibrosis and the presence of dense sub-epithelial deposits are correlated with risk of chronic renal failure. This risk is high (47%) in children with crescents in more than half the glomeruli. In adults, the percentage of crescents associated with unfavorable course appears to be lower than 50%. Predictions are only valid if no further renal flare-up occurs. In addition, histology cannot precisely predict the course of persistent renal sequelae. The severity of sequelae determines the risk and the rapidity of developing chronic renal failure. It is thus recommended to follow patients with Henoch-Schönlein nephritis for long periods.

Acute Disease↗
PubMed1992

[Schoenlein-Henoch syndrome in adults and skin infection].

Schoenlein-Henoch purpura is a rare disease in adults clinically characterized by palpable purpura, mainly on the legs, with varying degrees of gastrointestinal, articular and renal involvement. This syndrome is also characterized by IgA-vasculitis and its pathogenesis is still unclear, although it appears frequently after a viral or bacterial infection especially in the ORL sphere. We report here the cases of three adult patients who developed Schoenlein-Henoch purpura shortly after a bacterial infection of the skin, notably by Staphylococcus aureus.

Aged↗
PubMed1981

Radioimmunoassay profile of antiglobulins in connective tissue diseases: elevated level of IgA antiglobulin in systemic sicca syndrome.

A microplate radioimmunoassay for IgG, IgA and IgM antiglobulins reactive with rabbit IgG was performed on sera from 69 patients with various connective tissue diseases. IgM antiglobulins were detected in rheumatoid arthritis, systemic sicca syndrome and some patients with systemic vasculitis. IgA antiglobulins were found in seven of 10 patients with systemic sicca syndrome and only five of 59 patients with other connective tissue diseases. There was no correlation between the levels of IgM and IgA antiglobulins in the systemic sicca syndrome or rheumatoid arthritis (r = -0.21 and 0.2 respectively). IgG antiglobulins were not detected in any serum which lacked IgM or IgA antiglobulins. IgG isolated by DEAE columns showed antiglobulin activity in six of 15 rheumatoid arthritis and two of 10 systemic sicca syndrome sera tested. Antiglobulin analysis of sera fractionated by sucrose density ultracentrifugation at neutral and acid pH enabled the size of each class of antiglobulin to be determined. In certain sera, antiglobulin activity extended into the denser region of the gradient at pH 7.2 suggesting that the antiglobulins were complexed.

Adult↗
PubMed1991

Infantile acute hemorrhagic edema of the skin: study of ten cases.

Infantile acute hemorrhagic edema of the skin is not included as a separate entity in the current English-language literature as it is in continental Europe. Therefore we have attempted to clarify the nosologic position of acute hemorrhagic edema among cutaneous vasculitides in children, on the basis of our experience in 10 cases. Our study confirms that acute hemorrhagic edema affects infants between 4 and 24 months of age. The two main features are an ecchymotic purpura, often in a cockade pattern, and an inflammatory edema of the limbs and face. Visceral involvement is uncommon. Spontaneous and complete resolution occurs within 1 to 3 weeks; one to four attacks may occur. Histopathologic examination demonstrates a leukocytoclastic vasculitis. Perivascular IgA deposits can occasionally be found. Besides typical acute hemorrhagic edema, some cases in 2- to 4-year-old children appear to overlap with Schönlein-Henoch purpura. We suggest that typical acute hemorrhagic edema should be regarded as a separate clinical entity. This allows an appropriate prognosis to be made for this generally benign disease of infants.

Acute Disease↗
PubMed2005

Henoch-Schönlein purpura presenting as terminal ileitis and complicated by thrombotic microangiopathy.

We report here on a 40-year-old woman with abdominal pain, low-grade fever, and diarrhea in whom the cutaneous features of Henoch-Schönlein purpura (HSP) appeared only a few days after acute abdominal symptoms. Endoscopy showed terminal ileitis, and histopathological examination of a biopsy of the ileum showed a picture of IgA vasculitis. The clinical course was further complicated by the development of microangiopathic hemolytic anemia, thrombocytopenia, and severe renal failure.

Journal Article↗