History

Fact Explanation
Anorexia Anorexia is a presenting complaint which occurs during the prodromal period. [7]
Fever Some affected patients can be febrile. Low grade fever is common. [8]
Rash After the prodrome, patients develop skin rash, which is predominantly distributed over the legs. [2]
Abdominal pain Involvement of the small vessels of the intestines can produce intestinal ischemia and abdominal pain. Around 85% of the patients develop gastrointestinal symptoms. Patients develop colicky abdominal pain with intussusception. Pain over the right lower quadrant is indicative of acute appendicitis, which is a recognized complication of the disease. [1,2,3,4]
Vomiting 30% of the patients present with vomiting. [8]
Joint pain Polyarthralgia is a common presentation of AP. Knees, elbows and ankles are commonly involved. The triad of symptoms suggestive of AP includes abdominal pain, palpable purpura and arthritis. [1,2,3,4]
Scrotal edema Scrotal swelling and orchitis develop in about 35% of boys with AP. [8]
Bleeding per-rectum Bleeding per-rectum is a complication of gastrointestinal tract involvement. Some children may rarely develop hematochezia (passage of fresh blood per-rectum). [5]
Hematemesis Hematemesis is another gastrointestinal complication of AP. Rarely some may develop massive upper gastrointestinal haemorrhage. [3]
Hematuria Hematuria is another presenting complain of AP. [8]
Recent exposure to an allergen Although the exact allergen is not known, AP can develop after being exposed to some foods, infections, drugs, vaccinations (meningitis C), and insect bites. Infections that may lead to the development of AP include group A streptococci, mycoplasma, Epstein-Barr, and Varicella virus. [1,4,9]
Chest pain Patients with AP may rarely develop myocardial infarction secondary to involvement of coronary arteries. Children may complain of acute left sided chest pain, which radiates to the left arm and jaw. [4]
Neurological presentations Headache and behavioral changes are the common presenting complains that are due to vasculitis of the central nervous system. Rarely children can develop seizures, hemiplegia due to intracranial hemorrhage and encephalopathy. [6]
References
  1. CHEN O, ZHU X, REN P, WANG Y, SUN R, WEI D. Henoch Schonlein Purpura in children: clinical analysis of 120 cases Afr Health Sci [online] 2013 Mar, 13(1):94-99 [viewed 08 September 2014] Available from: doi:10.4314/ahs.v13i1.26
  2. CHEN T, LU YH, WANG WJ, BIAN CY, CHENG XY, SU Y, ZHOU PM. Elevated Urinary Levels of Cystatin C and Neutrophil Gelatinase-Associated Lipocalin in Henoch-Sch?nlein Purpura Patients with Renal Involvement PLoS One [online] , 9(6):e101026 [viewed 09 September 2014] Available from: doi:10.1371/journal.pone.0101026
  3. SEMEENA N, ADLEKHA S. Henoch-Sch?nlein Purpura Associated with Gangrenous Appendicitis: A Case Report Malays J Med Sci [online] 2014 Mar, 21(2):71-73 [viewed 09 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4028575
  4. HASIJA N, TAXAK S, BHARDWAJ M, VASHIST K. Anesthetic management of a patient with Henoch-Schonlein purpura for drainage of cervical lymphadenitis: A case report Saudi J Anaesth [online] 2014, 8(2):282-283 [viewed 09 September 2014] Available from: doi:10.4103/1658-354X.130750
  5. NAGAMORI T, OKA H, KOYANO S, TAKAHASHI H, OKI J, SATO Y, MURONO K, ISEKI K, TAKEGUCHI R, TAKEDA T, SATO M, SUGAI R, KITAMURA H, KAJINO H, MIURA Y, ISHIOKA T, AZUMA H. Construction of a scoring system for predicting the risk of severe gastrointestinal involvement in Henoch-Sch?nlein Purpura Springerplus [online] :171 [viewed 09 September 2014] Available from: doi:10.1186/2193-1801-3-171
  6. BAKKALOGLU S. A.. Cerebral vasculitis in Henoch-Schonlein purpura. [online] 2000 February, 15(2):246-248 [viewed 09 September 2014] Available from: doi:10.1093/ndt/15.2.246
  7. HAMEED S, DUA S, TAYLOR HW. Henoch-Schonlein Purpura with Ischaemic Bowel Ann R Coll Surg Engl [online] 2008 Oct, 90(7):W16-W17 [viewed 09 September 2014] Available from: doi:10.1308/147870808X303155
  8. REAMY BV, WILLIAMS PM, LINDSAY TJ. Henoch-Schönlein purpura. Am Fam Physician [online] 2009 Oct 1, 80(7):697-704 [viewed 09 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/19817340
  9. COURTNEY P. A.. Henoch-Schonlein purpura following meningitis C vaccination. [online] 2001 March, 40(3):345-346 [viewed 09 September 2014] Available from: doi:10.1093/rheumatology/40.3.345

Examination

Fact Explanation
Fever Low grade fever can be detected in some patients. [5]
Rash Palpable purpura is commonly seen over the legs, buttocks and the extensor surfaces of the body. Characteristically the rash is distributed symmetrically. [1,3,4]
Scrotal edema Some affected boys can develop orchitis and scrotal swelling. [5]
Examination of the joints The affected joints are swollen and tender. Knees, elbows and ankles are the commonest joints that are involved. [4]
Blood pressure Measurement of blood pressure is mandatory in children with AP as elevated blood pressure indicates renal involvement, which is the most common and serious complication of AP. Blood pressure can be reduced in children with massive gastrointestinal tract hemorrhage and nephrotic syndrome. [2,3]
Hepatosplenomegaly Hepatosplenomegaly can be detected in very few children with AP. [4]
References
  1. CHEN O, ZHU X, REN P, WANG Y, SUN R, WEI D. Henoch Schonlein Purpura in children: clinical analysis of 120 cases Afr Health Sci [online] 2013 Mar, 13(1):94-99 [viewed 08 September 2014] Available from: doi:10.4314/ahs.v13i1.26
  2. CHEN T, LU YH, WANG WJ, BIAN CY, CHENG XY, SU Y, ZHOU PM. Elevated Urinary Levels of Cystatin C and Neutrophil Gelatinase-Associated Lipocalin in Henoch-Sch?nlein Purpura Patients with Renal Involvement PLoS One [online] , 9(6):e101026 [viewed 09 September 2014] Available from: doi:10.1371/journal.pone.0101026
  3. SEMEENA N, ADLEKHA S. Henoch-Sch?nlein Purpura Associated with Gangrenous Appendicitis: A Case Report Malays J Med Sci [online] 2014 Mar, 21(2):71-73 [viewed 09 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4028575
  4. HASIJA N, TAXAK S, BHARDWAJ M, VASHIST K. Anesthetic management of a patient with Henoch-Schonlein purpura for drainage of cervical lymphadenitis: A case report Saudi J Anaesth [online] 2014, 8(2):282-283 [viewed 09 September 2014] Available from: doi:10.4103/1658-354X.130750
  5. REAMY BV, WILLIAMS PM, LINDSAY TJ. Henoch-Schönlein purpura. Am Fam Physician [online] 2009 Oct 1, 80(7):697-704 [viewed 09 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/19817340

Differential Diagnoses

Fact Explanation
Acute abdomen Patients with AP and acute abdomen can present with abdominal pain. Appendicitis, intussusceprtion, bowel infarction, should be suspected in children presenting with acute abdominal pain. [2]
Essential mixed cryoglobulinemia Purpura, weakness and arthralgia are the common complains of cryoglobulinemia. Leukocytoclastic vasculitis affects small and medium sized vessels. [3]
Acute glomerulonephritis In acure glomerulonephritis patients develop renal failure, hematuria and or proteinuria. Renal failure occurs due to immune mediated damage. [5]
Acute renal failure Acute renal failure is acute and reversible deterioration in renal function. Patients commonly present with oliguria. [4]
Bacterial endocarditis Patients with endocarditis present with prolonged and low grade fever. Microscopic hematuria can be a clinical feature of renal involvement. [2]
Polyarteritis nodosa Polyarteritis nodosa is a rare vasculitic disease of the childhood. Mild fever, muscle and joint involvement is common. [1,6]
Hypersensitivity vasculitis Hypersensitivity vasculitis can occur commonly after being exposed to a triggering antigen. Affected patients develop palpable purpura, fever, malaise, myalgia, and anorexia. [2]
References
  1. BAKKALOGLU S. A.. Cerebral vasculitis in Henoch-Schonlein purpura. [online] 2000 February, 15(2):246-248 [viewed 09 September 2014] Available from: doi:10.1093/ndt/15.2.246
  2. REAMY BV, WILLIAMS PM, LINDSAY TJ. Henoch-Schönlein purpura. Am Fam Physician [online] 2009 Oct 1, 80(7):697-704 [viewed 09 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/19817340
  3. FERRI CLODOVEO. Mixed cryoglobulinemia. Array [online] 2008 December [viewed 09 September 2014] Available from: doi:10.1186/1750-1172-3-25
  4. NEEDHAM E. Management of acute renal failure. Am Fam Physician [online] 2005 Nov 1, 72(9):1739-46 [viewed 09 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/16300036
  5. VINEN C S. Acute glomerulonephritis. [online] 2003 April, 79(930):206-213 [viewed 09 September 2014] Available from: doi:10.1136/pmj.79.930.206
  6. BANSAL NINA-KAREN, HOUGHTON KRISTIN MICHELLE. Cutaneous Polyarteritis Nodosa in Childhood: A Case Report and Review of the Literature. Arthritis [online] 2010 December, 2010:1-7 [viewed 09 September 2014] Available from: doi:10.1155/2010/687547

Investigations - for Diagnosis

Fact Explanation
Full blood count Presence of purpura in the absence of thrombocytopenia is the hallmark of diagnosing AP. Some children develop leukocytosis. [1,4,7]
Urine full report UFR can be normal during the early disease. Hematuria is common and some patients can develop proteinuria. Both indicate the presence of renal involvement. [3]
Erythrocyte sedimentation rate (ESR) ESR can be mildly elevated. [4]
Stool occult blood test Stool occult blood can be detected in the presence of occult gastrointestinal bleeding. [10]
Plasma D-dimer Hyperfibrinolysis can be seen in patients with AP especially in the presence of nephritis. Fibrinolysis will further damage the kidneys and worsen nephritis. [2,7]
Antistreptolysin O (ASO) If post streptococcal AP is suspected ASO can be assessed. [11]
Complement levels C3 and C4 are decreased in some affected children. This leads to abnormal activation of B lymphocytes and excessive secretion of IgA. [2,7]
Immunoglobulin Both IgG and IgA are elevated. [2,7]
Biopsy According to the diagnostic guidelines of American College of Rheumatology, presence of granulocytes in the walls of small arterioles or venules, palpable purpura and gastrointestinal complains is diagnostic of AP. [5]
Renal biopsy Although not routinely indicated renal biopsy is indicated in children with AP and nephritis. [6]
Video capsule endoscopy Video capsule endoscopy is helpful in evaluating gastrointestinal tract lesions. [8]
MRI MRI of the head is highly sensitive in detecting cerebral vasculitis. [9]
References
  1. CHEN O, ZHU X, REN P, WANG Y, SUN R, WEI D. Henoch Schonlein Purpura in children: clinical analysis of 120 cases Afr Health Sci [online] 2013 Mar, 13(1):94-99 [viewed 08 September 2014] Available from: doi:10.4314/ahs.v13i1.26
  2. DING D, YAN H, ZHEN X. Effects of Chinese herbs in children with Henoch-Schonlein purpura nephritis: a randomized controlled trial. J Tradit Chin Med [online] 2014 Feb, 34(1):15-22 [viewed 09 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/25102685
  3. CHEN T, LU YH, WANG WJ, BIAN CY, CHENG XY, SU Y, ZHOU PM. Elevated Urinary Levels of Cystatin C and Neutrophil Gelatinase-Associated Lipocalin in Henoch-Sch?nlein Purpura Patients with Renal Involvement PLoS One [online] , 9(6):e101026 [viewed 09 September 2014] Available from: doi:10.1371/journal.pone.0101026
  4. SEMEENA N, ADLEKHA S. Henoch-Sch?nlein Purpura Associated with Gangrenous Appendicitis: A Case Report Malays J Med Sci [online] 2014 Mar, 21(2):71-73 [viewed 09 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4028575
  5. HASIJA N, TAXAK S, BHARDWAJ M, VASHIST K. Anesthetic management of a patient with Henoch-Schonlein purpura for drainage of cervical lymphadenitis: A case report Saudi J Anaesth [online] 2014, 8(2):282-283 [viewed 09 September 2014] Available from: doi:10.4103/1658-354X.130750
  6. HADIDI R, HADIDI M, ALDABBAS M. Spectrum of biopsy-proven kidney disease in children at a Jordanian Hospital. Saudi J Kidney Dis Transpl [online] 2014 May, 25(3):680-3 [viewed 09 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/24821178
  7. NAGAMORI T, OKA H, KOYANO S, TAKAHASHI H, OKI J, SATO Y, MURONO K, ISEKI K, TAKEGUCHI R, TAKEDA T, SATO M, SUGAI R, KITAMURA H, KAJINO H, MIURA Y, ISHIOKA T, AZUMA H. Construction of a scoring system for predicting the risk of severe gastrointestinal involvement in Henoch-Sch?nlein Purpura Springerplus [online] :171 [viewed 09 September 2014] Available from: doi:10.1186/2193-1801-3-171
  8. LI M, OMI T, MATANO Y, FUJIMORI S, KAWANA S. The diagnostic usefulness of video capsule endoscopy in adolescent immunoglobulin a vasculitis (henoch-schönlein purpura). J Nippon Med Sch [online] 2014, 81(2):114-7 [viewed 09 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/24805100
  9. BAKKALOGLU S. A.. Cerebral vasculitis in Henoch-Schonlein purpura. [online] 2000 February, 15(2):246-248 [viewed 09 September 2014] Available from: doi:10.1093/ndt/15.2.246
  10. HAMZAOUI AMIRA, MELKI WISSEM, HARZALLAH OLFA, NJIM LEILA, KLII RIM, MAHJOUB SILVIA. Gastrointestinal involvement revealing Henoch Schonlein purpura in adults: Report of three cases and review of the literature. Array [online] 2011 December [viewed 09 September 2014] Available from: doi:10.1186/1755-7682-4-31
  11. REAMY BV, WILLIAMS PM, LINDSAY TJ. Henoch-Schönlein purpura. Am Fam Physician [online] 2009 Oct 1, 80(7):697-704 [viewed 09 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/19817340

Investigations - Fitness for Management

Fact Explanation
Full blood count If the child is awaiting surgery for the treatment of gastrointestinal complications of AP assessment and correction of hemoglobin is indicated prior to surgery. [1]
Renal function test Similarly fitness for general anesthesia is assessed with renal function test, which includes serum electrolytes and serum creatinine. [1]
References
  1. REAMY BV, WILLIAMS PM, LINDSAY TJ. Henoch-Schönlein purpura. Am Fam Physician [online] 2009 Oct 1, 80(7):697-704 [viewed 09 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/19817340

Investigations - Followup

Fact Explanation
Renal function test 20- 60% of affected children may develop nephritis due to deposition of IgA. These children have higher mortality and morbidity. Altered serum sodium and potassium levels and elevated serum creatinine enable early detection of nephritis. Nephritis may progress to nephropathy in very few children. 50% of patients with mixed nephritic- nephrotic syndrome may develop chronic renal failure. [1,2,3]
Urine full report (UFR) UFR is indicated in patient followup up to about six months for the detection of nephritis. [3]
Biomarkers Urinary levels of cystatin C (Cys C) and neutrophil gelatinase-associated lipocalin (NGAL) are elevated in children with HSP. These are useful in early detection of chronic renal involvement as serum creatinine is elevated relatively late in the disease process. [3]
References
  1. DING D, YAN H, ZHEN X. Effects of Chinese herbs in children with Henoch-Schonlein purpura nephritis: a randomized controlled trial. J Tradit Chin Med [online] 2014 Feb, 34(1):15-22 [viewed 09 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/25102685
  2. NIKIBAKHSH AA, MAHMOODZADEH H, KARAMYYAR M, HEJAZI S, NOROOZI M, MACOOIE AA. Treatment of severe henoch-schonlein purpura nephritis with mycophenolate mofetil. Saudi J Kidney Dis Transpl [online] 2014 Jul-Aug, 25(4):858-63 [viewed 09 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/24969202
  3. CHEN T, LU YH, WANG WJ, BIAN CY, CHENG XY, SU Y, ZHOU PM. Elevated Urinary Levels of Cystatin C and Neutrophil Gelatinase-Associated Lipocalin in Henoch-Sch?nlein Purpura Patients with Renal Involvement PLoS One [online] , 9(6):e101026 [viewed 09 September 2014] Available from: doi:10.1371/journal.pone.0101026

Investigations - Screening/Staging

Fact Explanation
Renal biopsy Renal biopsy is indicated to stage the renal involvement in children with AP. [1]
References
  1. HADIDI R, HADIDI M, ALDABBAS M. Spectrum of biopsy-proven kidney disease in children at a Jordanian Hospital. Saudi J Kidney Dis Transpl [online] 2014 May, 25(3):680-3 [viewed 09 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/24821178

Management - General Measures

Fact Explanation
Hydration Patients with gastrointestinal tract bleeding may require initial assessment of airway, breathing and circulation. Fluid replacement should be started with intravenous crystalloids followed by colloids and cross-matched blood if needed.
Analgesics Simple analgesics or non-steroidal anti-inflammatory drugs are indicated for the treatment of arthritis. [1]
References
  1. HASIJA N, TAXAK S, BHARDWAJ M, VASHIST K. Anesthetic management of a patient with Henoch-Schonlein purpura for drainage of cervical lymphadenitis: A case report Saudi J Anaesth [online] 2014, 8(2):282-283 [viewed 09 September 2014] Available from: doi:10.4103/1658-354X.130750

Management - Specific Treatments

Fact Explanation
Conservative management AP is often self-limiting and do not usually require specific treatment. [1]
Corticosteroids Corticosteroids are indicated for children with severe renal and central nervous system involvement, such as rapidly progressive glomerulonephritis (RPGN). [1,2,4]
Immunosuppressors Immunesuppressors such as cyclophosphamide, cyclosporine, mycophenolate mofetil and azathioprine are used in the treatment of severe nephritis. [1,2]
IV immunoglobulin G (IVIg) High-dose IVIg is indicated for the treatment of severe renal involvement. [2]
Plasmapheresis Plasmapheresis is indicated for children with severe nephritis and central nervous system involvement. [2,3]
Surgery Children who develop intussusception, intestinal perforation, bowel necrosis, and massive gastrointestinal bleeding require surgical intervention. [5]
Kidney transplantation In end-stage renal failure kidney transplantation is indicated. [6]
References
  1. CHEN O, ZHU X, REN P, WANG Y, SUN R, WEI D. Henoch Schonlein Purpura in children: clinical analysis of 120 cases Afr Health Sci [online] 2013 Mar, 13(1):94-99 [viewed 08 September 2014] Available from: doi:10.4314/ahs.v13i1.26
  2. NIKIBAKHSH AA, MAHMOODZADEH H, KARAMYYAR M, HEJAZI S, NOROOZI M, MACOOIE AA. Treatment of severe henoch-schonlein purpura nephritis with mycophenolate mofetil. Saudi J Kidney Dis Transpl [online] 2014 Jul-Aug, 25(4):858-63 [viewed 09 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/24969202
  3. HASIJA N, TAXAK S, BHARDWAJ M, VASHIST K. Anesthetic management of a patient with Henoch-Schonlein purpura for drainage of cervical lymphadenitis: A case report Saudi J Anaesth [online] 2014, 8(2):282-283 [viewed 09 September 2014] Available from: doi:10.4103/1658-354X.130750
  4. BAKKALOGLU S. A.. Cerebral vasculitis in Henoch-Schonlein purpura. [online] 2000 February, 15(2):246-248 [viewed 09 September 2014] Available from: doi:10.1093/ndt/15.2.246
  5. SEMEENA N, ADLEKHA S. Henoch-Sch?nlein Purpura Associated with Gangrenous Appendicitis: A Case Report Malays J Med Sci [online] 2014 Mar, 21(2):71-73 [viewed 09 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4028575
  6. DAVIN J.-C.. Henoch-Schonlein Purpura Nephritis: Pathophysiology, Treatment, and Future Strategy. Clinical Journal of the American Society of Nephrology [online] December, 6(3):679-689 [viewed 10 September 2014] Available from: doi:10.2215/​CJN.06710810