Henoch Schonlein Purpura (Anaphylactoid Purpura, HSP)

Article Author:
Porsha Roache-Robinson
Article Editor:
David Hotwagner
Updated:
12/26/2018 6:41:50 PM
PubMed Link:
Henoch Schonlein Purpura (Anaphylactoid Purpura, HSP)

Introduction

Henoch-Schonlein purpura is a vasculitis involving the small vessels of the joints, kidneys, gastrointestinal (GI) tract, and the skin. Henoch-Schonlein purpura can also involve the central nervous system (CNS) and the lungs; however, these findings are rare. It is an acute immunoglobulin A (IgA)-mediated disorder that is typically self-limited and managed with supportive care; however, serious complications, such as renal failure, may occur as a result of the disorder.

Henoch-Schonlein purpura is named after 2 German physicians, Dr. Johann Schonlein and his student Eduard Henoch. Schonlein identified the association of joint pain and purpura, and Henoch identified the GI and renal involvement. Although Henoch-Schonlein purpura is named after Henoch and Schonlein, an English physician named William Heberden was the first to describe the disorder in the early 1800s.[1]

Etiology

Environmental, genetic, and antigenic factors appear to contribute to the etiology of Henoch-Schonlein purpura. Many patients report a preceding infection. Upper respiratory tract infections are the most common; however, patients may also present with a previous GI or pharyngeal infection.

Group A Streptococcus has been found in cultures of greater than 30% of patients with Henoch-Schonlein nephritis.[2] Coxsackievirus, hepatitis A, hepatitis B, Mycoplasma, parvovirus B19, Campylobacter, Varicella, and adenoviruses have also been known to precede the development of Henoch-Schonlein purpura.[2]

Epidemiology

Henoch-Schonlein purpura is a very rare disorder that typically affects children; however, the disorder can also be seen in adults and adolescents. The majority of children present before the age of 10. It is often more severe and more likely to cause long-term renal disease in adults.[2] It is the most common vasculitis among children, affecting 10 to 20 children per 100,000 per year.[3] Henoch-Schonlein purpura occurs slightly more often in white males.

Pathophysiology

The pathophysiology of Henoch-Schonlein purpura is not fully understood; however, IgA plays a significant role. IgA-antibody immune complexes caused by antigenic exposure from an infection or medication, deposit in the small vessels (usually capillaries) of the skin, joints, kidneys, and GI tract. This results in an influx of inflammatory mediators such as prostaglandins. Complement C3 receptor lymphocytes may bind to immune complexes and deposit in the vessel walls- contributing to the hyper-inflammatory response. If the immune complexes are deposited in the intestinal wall, they may cause GI hemorrhage. Renal involvement of IgA-mediated immune complexes may result in mild proliferative or severe crescentic glomerulonephritis.[2] Immune complex deposits in the skin cause palpable purpura and petechiae.

History and Physical

The classic presentation of Henoch-Schonlein purpura includes palpable purpura, GI complaints, arthralgias, and renal involvement.

Patients may present with the following:

  • Rash
  • Joint pain
  • Abdominal pain
  • Vomiting
  • Subcutaneous edema
  • Rectal bleeding
  • Scrotal edema
  • Headache
  • Fever
  • Diarrhea
  • Hematemesis
  • Fatigue

Physical Examination

Skin

Skin involvement is present in all patients with Henoch-Schonlein purpura.[1] The rash associated with Henoch Schonlein purpura is non-pruritic, and it is characterized by palpable purpura and petechiae that most commonly affect the buttocks and lower extremities – particularly the extensor surfaces. Approximately one-third of patients experience the rash in the upper extremities and trunk.[3] The lesions have the potential to become bullous or necrotic. The lesions change from red to purple and then become rust-colored before they fade. These changes occur over approximately 10 days.[2]

GI

GI findings may occur before the rash in 10% to 40% of patients.[3] Patients may present with nausea and vomiting that is worse after meals. Potential life-threatening GI complications include intussusception, bowel perforation, bowel gangrene, and massive hemorrhage. Intussusception is the most common life-threatening gastrointestinal complication, affecting 3% to 4% of patients with Henoch Schonlein purpura.[1]

Renal

Renal symptoms typically occur within 1 to 3 months after the rash in 20% to 55% of children with Henoch-Schonlein purpura.[3] Renal manifestations include hematuria, proteinuria, nephrotic syndrome, nephritic syndrome, and renal failure. The most common renal manifestation is microscopic hematuria.[3] Severe proteinuria may present as nephrotic syndrome, and patients with persistent proteinuria are at high risk of developing progressive glomerulonephritis. Patients may also develop ureteric obstructions. Approximately 50% of patients develop renal manifestations, with less 1% progressing to end-stage renal failure.[4] Death from Henoch-Schonlein purpura is rare; however, renal disease is the most common cause of death in patients with the disorder.[2]

Joints

Approximately 15% of patients with Henoch-Schonlein purpura present with arthritis as the initial symptom, and overall arthralgia or arthritis occurs in 75% of children with the disorder.[3] Patients often present with painful swollen joints that most commonly involve the knees, ankles, hands, and feet. The arthralgias are typically transient and non-destructive.

CNS

CNS involvement is rare; however, when present, patients may present with headaches, dizziness, ataxia, seizures, irritability, mononeuropathy, intracranial hemorrhage, or acute motor-sensory axonal neuropathy.[3]

Evaluation

The diagnosis of Henoch-Schonlein purpura is made based on the presence of petechiae (without thrombocytopenia) or palpable purpura that predominantly affects the lower limbs plus at least one of the following four characteristics[1]:

  1. Abdominal pain
  2. Arthralgia or arthritis
  3. Renal involvement (proteinuria, red blood cell casts, or hematuria)
  4. Proliferative glomerulonephritis or leukocytoclastic vasculitis with predominant deposition of IgA on histology

CLinicians should order a urinalysis to identify hematuria, proteinuria, or red blood cell casts. If the urine dipstick is positive for protein, a 24-hour collection must be obtained to quantify the protein excretion [1]

The measurement of serum IgA levels is non-diagnostic. There are no definitive tests for the diagnosis of Henoch-Schonlein purpura.

Treatment / Management

Symptomatic and supportive care are the foundations of treatment for patients with Henoch-Schonlein purpura unless there is renal involvement. Acetaminophen and nonsteroidal anti-inflammatory drugs may be used for joint pain and fever; however, nonsteroidal anti-inflammatory medications should most certainly be avoided if there is GI or renal involvement.[5]

Supportive and symptomatic care may include:

  • Rehydration with intravenous (IV) fluids
  • Pain management
  • Wound care for ulcerative skin lesions

The management of Henoch-Schonlein purpura nephritis may include the following:

  • Corticosteroids
  • Plasma exchange
  • Immunosuppressants
  • Angiotensin-converting enzyme inhibitors.

Early oral prednisone treatment is useful for the management of renal, joint, and GI manifestations. Prednisone does not prevent renal disease; however, it reduces the risk of developing a persistent, renal disease in children.[2] According to randomized control trials performed by Ronkainen et al. and Jauhola et al., evidence suggests that prednisone reduces the duration and severity of abdominal pain during the first 2 weeks of treatment.[5]

Differential Diagnosis

  • IgA nephropathy
  • Acute renal failure
  • Acute glomerulonephritis
  • Idiopathic thrombocytopenic purpura
  • Disseminated intravascular coagulation
  • Thrombotic thrombocytopenic purpura
  • Hemolytic uremic syndrome
  • Meningococcal meningitis
  • Hypersensitivity vasculitis
  • Systemic lupus erythematosus
  • Polyarteritis nodosa
  • Bacterial endocarditis
  • Inflammatory bowel disease
  • Wegener granulomatosis
  • Rocky Mountain spotted fever

Prognosis

Henoch-Schonlein purpura is typically a self-limited illness that demonstrates an excellent prognosis in patients without renal involvement. The majority of patients fully recover in 4 weeks.[2] Henoch-Schonlein Purpura recurs in approximately one-third of patients within 4 to 6 months after the initial onset.

The long-term morbidity of Henoch-Schonlein purpura is dependent on the extent of renal involvement. Approximately 1% of patients with Henoch-Schonlein purpura will develop end-stage renal disease (ESRD) and require renal transplantation.[5]

Complications

Henoch-Schonlein purpura involves multiple organ systems, and the potential complications are relatively extensive. Potential complications include:

  • Renal Failure
  • Proteinuria
  • Hematuria
  • Nephrotic syndrome
  • Intussusception
  • Gastrointestinal bleeding
  • Bowel infarction
  • Bowel perforation
  • CNS bleeding
  • Seizures
  • Neuropathy
  • Pleural effusion
  • Pulmonary hemorrhage
  • Testicular torsion

Consultations

Patients who present with nephritic syndrome, nephrotic syndrome, hematuria, or rapidly worsening proteinuria should be referred to a pediatric nephrologist on an urgent basis.[5]

Deterrence and Patient Education

  • Symptoms are likely to resolve within weeks; however, symptoms may recur.
  • Severe renal involvement is rare.
  • If there is evidence of severe renal involvement, patients will require aggressive treatment and management by a nephrologist.

Enhancing Healthcare Team Outcomes

Henoch-Schonlein purpura is typically a self-limiting illness; however, patients may develop life-threatening complications such as intussusception, massive GI hemorrhage, and renal failure. An interprofessional approach is necessary for the adequate diagnosis and management of the illness. Patients may present with non-specific symptoms such as malaise, upper respiratory symptoms or arthralgias before the development of the characteristic rash. This may result in delayed diagnosis. Nurse practitioners, physician assistants, and physicians may see patients during different stages of the disease course; therefore, it is important for medical staff to communicate and be aware of the potential complications.

A surgical team and radiologist may be required for the diagnosis and management of intussusception.

An important aspect of the disease process is adequate to follow up with frequent urinalyses to screen for potential renal involvement. Patients who are treated with corticosteroids may require assistance from pharmacists with regards to adequate therapeutic dosing and tapering. Patients with severe renal disease will need to see a nephrology team comprised of medical assistants, nurses, physicians, and possibly a transplant team.


References

[1] Trnka P, Henoch-Schönlein purpura in children. Journal of paediatrics and child health. 2013 Dec     [PubMed PMID: 24134307]
[2] Reamy BV,Williams PM,Lindsay TJ, Henoch-Schönlein purpura. American family physician. 2009 Oct 1     [PubMed PMID: 19817340]
[3] Hetland LE,Susrud KS,Lindahl KH,Bygum A, Henoch-Schönlein Purpura: A Literature Review. Acta dermato-venereologica. 2017 Nov 15     [PubMed PMID: 28654132]
[4] Guo D,Lam JM, Henoch-Schönlein purpura. CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne. 2016 Oct 18     [PubMed PMID: 27431304]
[5] Bluman J,Goldman RD, Henoch-Schönlein purpura in children: limited benefit of corticosteroids. Canadian family physician Medecin de famille canadien. 2014 Nov     [PubMed PMID: 25551129]