Continuing Education Activity

Henoch-Schönlein purpura is an IgA-mediated immune vasculitis involving the small vessels of the joints, kidneys, gastrointestinal (GI) tract, skin, and less commonly, the central nervous system and lungs. Patients with this disorder typically present with gastrointestinal complaints, palpable purpura, arthralgias, and renal involvement. This activity reviews the evaluation and management of Henoch-Schönlein purpura and explains the role of the interprofessional team in improving care for patients with this condition.

Objectives:

• Identify the etiology of Henoch-Schönlein purpura.
• Describe the role of IgA in the pathophysiology of Henoch-Schönlein purpura.
• Outline the management of Henoch-Schönlein purpura.
• Review the importance of collaboration and communication among the interprofessional team members to be aware of the possible complications and improve outcomes for patients affected by Henoch-Schönlein purpura.

Introduction

Henoch-Schönlein purpura is a vasculitis involving the small vessels of the joints, kidneys, gastrointestinal tract, and skin. Henoch-Schönlein 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-Schönlein purpura is named after two German physicians, Dr. Johann Schönlein and his student Eduard Henoch. Schönlein identified the association of joint pain and purpura, and Henoch identified gastrointestinal and renal involvement. Although Henoch-Schönlein purpura is named after Henoch and Schönlein, 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-Schönlein purpura. Many patients report a preceding infection. Upper respiratory tract infections are the most common; however, patients may also present with a previous gastrointestinal or pharyngeal infection.

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

Epidemiology

Henoch-Schönlein purpura is a 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-Schönlein purpura occurs slightly more often in White males.

Pathophysiology

The pathophysiology of Henoch-Schönlein 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 gastrointestinal 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 gastrointestinal 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-Schönlein purpura includes palpable purpura, gastrointestinal 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-Schönlein purpura.[1] The rash associated with Henoch Schönlein 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 ten days.[2]

Gastrointestinal

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 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 Schönlein purpura.[1]

Renal

Renal symptoms typically occur within 1 to 3 months after the rash in 20% to 55% of children with Henoch-Schönlein 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 than 1% progressing to end-stage renal failure.[4] Death from Henoch-Schönlein 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-Schönlein 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.

Central nervous system

Central nervous system 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-Schönlein 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-Schönlein purpura.

Treatment / Management

Symptomatic and supportive care are the foundations of treatment for patients with Henoch-Schönlein 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 gastrointestinal 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-Schönlein 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 gastrointestinal 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 two weeks of treatment.[5]

Differential Diagnosis

The differential diagnosis of Henoch Schönlein purpura include the following:

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

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

Complications

Henoch-Schönlein 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 urgently referred to a pediatric nephrologist.[5]

Deterrence and Patient Education

Patients should be educated that the symptoms are likely to resolve within weeks; however, symptoms may recur. Although severe renal involvement is rare, if there is evidence of severe renal involvement, patients require aggressive treatment and management by a nephrologist.

Enhancing Healthcare Team Outcomes

Henoch-Schönlein purpura is typically a self-limiting illness; however, patients may develop life-threatening complications such as intussusception, massive gastrointestinal 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 a delayed diagnosis. Nurse practitioners, physician assistants, and physicians may see patients during different stages of the disease; 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 regard 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.