scholarly journals SEVERE GASTROINTESTINAL MANIFESTATIONS COMPLICATING HENOCH-SCHÖNLEIN PURPURA IN ADULT: A RARE CASE IN INDONESIA

2018 ◽  
Vol 11 (11) ◽  
pp. 11
Author(s):  
William Ray Cassidy ◽  
Ketut Suryana ◽  
Dewi Catur Wulandari
Keyword(s):  
Systemic Vasculitis ◽  
Renal Involvement ◽  
Immunoglobulin A ◽  
Henoch Schonlein Purpura ◽  
Gastrointestinal Manifestations ◽  
Patient Morbidity ◽  
Schonlein Purpura ◽  
Term Monitoring ◽  
Henoch Schönlein Purpura

Henoch-Schönlein purpura (HSP), also known as immunoglobulin A vasculitis, is a self-limiting, small vessel systemic vasculitis rarely found in adults characterized by palpable purpura, arthritis or arthralgia, gastrointestinal, and renal involvement. We report a case of a 31-year-old male with HSP complicated with severe gastrointestinal manifestations mimicking acute abdomen. He was successfully managed with conservative approach. Meticulous diagnosis, treatment, and long-term monitoring are important to reduce patient morbidity.

scholarly journals Case of Gut Necrosis in Adult-Onset Immunoglobulin A Vasculitis (Henoch-Schönlein Purpura)

2020 ◽  
Vol 8 ◽  
pp. 232470962092556 ◽  
Author(s):  
Amanda S. Weissman ◽  
Viral Sanjay Patel ◽  
Omar Mushfiq
Keyword(s):  
Abdominal Pain ◽  
Renal Disease ◽  
Renal Involvement ◽  
Immunoglobulin A ◽  
Glucocorticoid Therapy ◽  
Acute Glomerulonephritis ◽  
Henoch Schonlein Purpura ◽  
Schonlein Purpura ◽  
Immunoglobulin A Vasculitis ◽  
Henoch Schönlein Purpura

Immunoglobulin A vasculitis (IgAV), formerly known as Henoch-Schönlein purpura, is an immune-mediated small vessel vasculitis characterized by palpable purpura, arthralgia, abdominal pain, and renal disease. It is primarily a childhood disease and usually resolves spontaneously with supportive therapy. Treatment of IgAV in adults is controversial with no clearly established guidelines. We report a rare case of IgAV in an adult male who developed gut necrosis and perforation while receiving glucocorticoid therapy for treatment of acute glomerulonephritis. A 44-year-old male was admitted with joint pain, leg swelling, mild abdominal pain, and a diffuse rash. Laboratory values revealed acute kidney injury with significant proteinuria and hematuria. The patient was started on glucocorticoid therapy for suspected IgAV nephritis, which was confirmed by kidney biopsy. Several days later, he complained of worsening abdominal pain. Imaging demonstrated bowel ischemia and perforation requiring multiple abdominal surgeries. The patient was critically ill in the intensive care unit with worsening renal failure requiring dialysis. He was discharged a month later after gradual recovery with stable but moderately impaired kidney function. IgAV is less common in adults; however, the disease is more severe with a higher risk of long-term complications. Adult patients with renal involvement may benefit from glucocorticoid therapy in preventing progression to end-stage renal disease. However, glucocorticoids may mask the symptoms of abdominal complications like gut necrosis and perforation causing delay in diagnosis and treatment. Therefore, vigilance to detect early signs of gut ischemia is imperative when treating an adult case of IgAV nephritis with glucocorticoids.

scholarly journals Tubulointerstitial Infiltration of M2 Macrophages in Henoch-Schönlein Purpura Nephritis Indicates the Presence of Glomerular Crescents and Bad Clinical Parameters

2019 ◽  
Vol 2019 ◽  
pp. 1-10 ◽  
Author(s):  
Jisup Kim ◽  
Sung-Eun Choi ◽  
Keum Hwa Lee ◽  
Hyeon Joo Jeong ◽  
Jae Il Shin ◽  
...  
Keyword(s):  
Systemic Vasculitis ◽  
Renal Involvement ◽  
International Study ◽  
Clinical Parameters ◽  
M2 Macrophages ◽  
Henoch Schonlein Purpura ◽  
Severe Manifestation ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura ◽  
Purpura Nephritis

Henoch-Schönlein purpura (HSP) is the most common systemic vasculitis in children, and renal involvement (HSP nephritis, HSPN) is a severe manifestation. HSPN is histologically classified by the International Study of Kidney Disease in Children (ISKDC) based on mesangial hypercellularity and the extent of glomerular crescents. Macrophages, categorized as M1 or M2, frequently infiltrate in various glomerular and tubulointerstitial diseases and infiltration of specific subtypes is associated with disease progression. Therefore, to identify whether infiltration of M1 or M2 macrophages has clinical significance, we quantified the subtypes of macrophages in 49 HSPN specimens and correlated the counts with histologic features and clinical parameters. Higher tubulointerstitial M2 counts were associated with chronic renal failure (CRF), ISKDC classes III-IV, and crescents (P<0.001, 0.002, 0.001). Glomerular M2 counts were significantly related to ISKDC classes III-IV and crescents (area under curve, AUC 0.804, 0.833). Tubulointerstitial M2 counts were associated with CRF, ISKDC classes III-IV, and crescents (AUC 0.872, 0.778, 0.830). Tubulointerstitial M2 counts also revealed higher AUC than tubulointerstitial M1 counts for CRF (P=0.036) and ISKDC classes III-IV (P=0.047). Glomerular M2 counts revealed higher AUC than glomerular M1 counts for ISKDC classes III–IV (P=0.024). Tubulointerstitial M2 counts were the most powerful parameter for CRF (AUC 0.872) and revealed even higher AUC than ISKDC classification (AUC 0.716) with borderline significance (P=0.086) for CRF. In summary, tubulointerstitial M2 counts were a superior parameter to tubulointerstitial M1 counts and even to ISKDC classification indicating the presence of CRF.

scholarly journals Successful Outcome of a Corticodependent Henoch-Schönlein Purpura Adult with Rituximab

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Taylor Pindi Sala ◽  
Jean-Marie Michot ◽  
Renaud Snanoudj ◽  
Marion Dollat ◽  
Emmanuel Estève ◽  
...  
Keyword(s):  
Systemic Vasculitis ◽  
Transplant Rejection ◽  
Immunoglobulin A ◽  
Corticosteroid Treatment ◽  
Successful Outcome ◽  
Henoch Schonlein Purpura ◽  
Small Vessels ◽  
History Of ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Henoch-Schönlein purpura (HSP) is a systemic vasculitis involving small vessels with deposition of immunoglobulin A (IgA) complexes, usually affecting children. Compared with children, HSP in adults is more severe and frequently associated with cancer. We report the case of a 49-year-old woman with medical history of kidney transplantation for segmental glomerular hyalinosis. Eight years after the transplantation, while taking combined immunosuppressive therapy with tacrolimus and azathioprine indicated for the prevention against transplant rejection, she developed a Henoch-Schönlein purpura. Vasculitis involves skin and sciatic peroneal nerve and she received systemic corticosteroid treatment. Because of four relapses and corticosteroid dependence, the patient was treated with rituximab (two intravenous infusions of 1000 mg given two weeks apart). Successful outcome was observed along two years of follow-up. This new case of successful use of rituximab in HSP promotes more investigations of this treatment in clinical trials.

Henoch-Schönlein purpura presenting with multiplex gastrointestinal manifestations and massive nephrotic syndrome in adulthood — a case report

Open Medicine ◽  
2008 ◽  
Vol 3 (4) ◽  
pp. 521-524
Author(s):  
Farkas Klaudia ◽  
Molnár Tamás ◽  
Nagy Ferenc ◽  
Tiszlavicz László ◽  
Németh István ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Involvement ◽  
Gastrointestinal Symptoms ◽  
Small Vessel Vasculitis ◽  
Henoch Schonlein Purpura ◽  
Intravenous Cyclophosphamide ◽  
Rapid Improvement ◽  
Gastrointestinal Manifestations ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

AbstractHenoch-Schönlein purpura (HSP) is a systemic small vessel vasculitis mainly affecting children. We report a case of a 49-year-old woman with severe gastrointestinal and renal involvement of HSP. Endoscopy revealed more characteristic findings in the terminal ileum than in the gastric antrum. Histological examinations of the biopsy samples from the ileum, antrum, skin and kidney confirmed the diagnosis of HSP. Parenteral corticosteroid therapy led to a rapid improvement of the gastrointestinal symptoms, but because of the excessive proteinuria intravenous cyclophosphamide therapy had to be introduced.

scholarly journals Successful Treatment of Hemorrhagic Bullous Henoch-Schönlein Purpura with Oral Corticosteroid: A Case Report

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Celebi Kocaoglu ◽  
Ramazan Ozturk ◽  
Yasar Unlu ◽  
Fatma Tuncez Akyurek ◽  
Sukru Arslan
Keyword(s):  
Case Report ◽  
Gastrointestinal Tract ◽  
Female Patient ◽  
Oral Corticosteroid ◽  
Systemic Vasculitis ◽  
Immunoglobulin A ◽  
Henoch Schonlein Purpura ◽  
Hemorrhagic Bullae ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Henoch-Schönlein purpura (HSP) is a vasculitis of small-sized blood vessels, resulting from immunoglobulin-A-mediated inflammation. It is the most common acute systemic vasculitis in childhood and mainly affects skin, gastrointestinal tract, joints, and kidneys. The characteristic rash of HSP consists of palpable purpuric lesions 2 to 10 mm in diameter concentrating in the buttocks and lower extremities. The occurrence of hemorrhagic bullae in children with HSP is rarely encountered. This report describes a 4.5-year-old female patient with HSP associated with hemorrhagic bullous lesions.

P064 Henoch-Schonlein Purpura: experience of a general pediatric center

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
D J Belamri ◽  
I Boustil ◽  
S Zoubir ◽  
Bensoltane H Chériet ◽  
N Bouchair
Keyword(s):  
Renal Failure ◽  
Chronic Renal Failure ◽  
Renal Involvement ◽  
Winter Period ◽  
Henoch Schonlein Purpura ◽  
Severe Renal Failure ◽  
Benign Condition ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Abstract Background Henoch-Schonlein purpura (HSP) is the most classic dysimmune vasculitis in children, related to an abnormal response of an immature immune system following an external antigen attack. Digestive involvement in HSP conditions the immediate vital prognosis and severe renal failure conditions the long-term functional prognosis. The Objective is to identify the epidemiological, clinical, disease course and therapeutic particularities of patients with HSP with a review of the literature. Material and method The authors reported the results of a retrospective study of cases of patients with HSP hospitalized and treated in the 02 pediatric departments of the wilaya of Annaba in Algeria (Clinique Sainte Thérèse, Annaba hospital university center and EHS El Bouni) over a period of 02 years. Information was obtained from hospital records. Results 24 cases of HSP were identified with a sex ratio of 1.4 (14 boys and 10 girls) and a hospital incidence of 1.66%. The average age was 6.6 years. Half of patients was hospitalized during the autumn-winter period, 67% of whom had an ENT infection before their hospitalizations. 17% of cases have personal history of HSP (the oldest was 5 years ago and the most recent 10 days before hospitalization). 7 patients underwent surgeries prior to hospitalization. Skin lesions were found in all patients. Joint, digestive and renal involvement were recorded in 50%, 80% and 25% respectively. Gastrointestinal bleeding has been observed in one patient. Only 1 patient with renal impairment progressed to chronic renal failure and only 1 patient had confirmed hypertension. A rare association with viral hepatitis A has been noted in one patient. Conclusion HSP remains a benign condition, easy to recognize clinically. Its immediate prognosis depends on the digestive complications and its long-term on nephropathy which may expose to the risk of chronic renal failure.

scholarly journals 119 Location of skin lesions in Henoch-Schonlein purpura (HSP) and its association with renal involvement

2016 ◽  
Vol 136 (5) ◽  
pp. S22
Author(s):  
J. St. John ◽  
A. Garza-Mayers ◽  
P. Vedak ◽  
M. Hoang ◽  
S. Nigwekar ◽  
...  
Keyword(s):  
Renal Involvement ◽  
Skin Lesions ◽  
Henoch Schonlein Purpura ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Serum Amyloid A Levels Associated with Gastrointestinal Manifestations in Henoch-Schönlein Purpura

Inflammation ◽  
2012 ◽  
Vol 35 (4) ◽  
pp. 1251-1255 ◽  
Author(s):  
Xuelian He ◽  
Yulan Zhao ◽  
Yin Li ◽  
Shixiu Kang ◽  
Yan Ding ◽  
...  
Keyword(s):  
Serum Amyloid A ◽  
Serum Amyloid ◽  
Henoch Schonlein Purpura ◽  
Gastrointestinal Manifestations ◽  
Amyloid A ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura
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