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Henoch-schoenlein Purpura

Disease Details

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Description: Henoch-Schönlein purpura (HSP) is a disease characterized by inflammation of small blood vessels, causing a rash, joint pain, abdominal pain, and kidney involvement.
Type: Henoch-Schönlein purpura (HSP) is a type of small vessel vasculitis. It is not typically considered a genetic disorder, as its exact cause is not well understood and it generally occurs sporadically. Most cases are thought to be triggered by infections, medications, or other environmental factors. There is no established pattern of genetic transmission for HSP.
Signs And Symptoms: Purpura, arthritis, and abdominal pain are known as the "classic triad" of Henoch–Schönlein purpura. Purpura occur in all cases, joint pains and arthritis in 80%, and abdominal pain in 62%. Some include gastrointestinal hemorrhage as a fourth criterion; this occurs in 33% of cases, sometimes, but not necessarily always, due to intussusception. The purpura typically appear on the legs and buttocks, but may also be seen on the arms, face and trunk. The abdominal pain is colicky in character, and may be accompanied by nausea, vomiting, constipation or diarrhea. There may be blood or mucus in the stools. The joints involved tend to be the ankles, knees, and elbows, but arthritis in the hands and feet is possible; the arthritis is nonerosive and hence causes no permanent deformity. Forty percent have evidence of kidney involvement, mainly in the form of hematuria (blood in the urine), but only a quarter will have this in sufficient quantities to be noticeable without laboratory tests. Problems in other organs, such as the central nervous system (brain and spinal cord) and lungs may occur, but is much less common than in the skin, bowel and kidneys.Of the 40% of patients who develop kidney involvement, almost all have evidence (visible or on urinalysis) of blood in the urine. More than half also have proteinuria (protein in the urine), which in one eighth is severe enough to cause nephrotic syndrome (generalised swelling due to low protein content of the blood). While abnormalities on urinalysis may continue for a long time, only 1% of all HSP patients develop chronic kidney disease. Hypertension (high blood pressure) may occur. Protein loss and high blood pressure, as well as the features on biopsy of the kidney if performed, may predict progression to advanced kidney disease. Adults are more likely than children to develop advanced kidney disease.
Prognosis: Overall prognosis is good in most patients, with one study showing recovery occurring in 94% and 89% of children and adults, respectively (some having needed treatment). In children under ten, the condition recurs in about a third of all cases, usually within the four months of the initial attack. Recurrence is more common in older children and adults.
Onset: Henoch-Schönlein purpura (HSP) typically has an onset that occurs most frequently in children, usually between the ages of 3 and 15. It often follows an upper respiratory infection. The symptoms can start suddenly and may progress over a period of days or weeks.
Prevalence: Henoch-Schönlein purpura (HSP) primarily affects children, with an estimated incidence of 10-20 cases per 100,000 children per year. It is less common in adults.
Epidemiology: HSP occurs more often in children than in adults, and usually follows an upper respiratory tract infection. Half of affected patients are below the age of six, and 90% are under ten. It occurs about twice as often in boys as in girls. The incidence of HSP in children is about 20 per 100,000 children per year, making it the most common vasculitis in children.Cases of HSP may occur anytime throughout the year, but some studies have found that fewer cases occur during the summer months.
Intractability: Henoch-Schönlein purpura (HSP) is generally not considered intractable. Most cases resolve on their own, especially in children, with supportive care. However, some individuals may experience more severe complications, such as kidney involvement, which might require more intensive treatment. Relapses can occur, but they are typically manageable with appropriate medical care.
Disease Severity: Henoch-Schönlein purpura (HSP) is generally a self-limiting disease with a good prognosis in most cases. The severity can vary:

- Mild cases: Characterized by skin purpura, mild joint pain, and abdominal discomfort.
- Moderate to severe cases: Involves significant gastrointestinal symptoms, more intense joint pain, and renal involvement (glomerulonephritis).

Nephritis is the most concerning complication, potentially leading to long-term kidney damage if severe. Monitoring and treatment are essential, particularly in cases with renal involvement.
Healthcare Professionals: Disease Ontology ID - DOID:11123
Pathophysiology: Henoch–Schönlein purpura is a small-vessel vasculitis in which complexes of immunoglobulin A (IgA) and complement component 3 (C3) are deposited on arterioles, capillaries, and venules (hence it is a type III hypersensitivity reaction). The activation of the alternative complement pathway results in the deposition of IgA aggregates or IgA complexes in target organs (with deposition of C3). This leads to the production of inflammatory mediators, including vascular prostaglandins like prostacyclin, which may play a key role in the development of IgAV and its organ-specific clinical manifestations. As with IgA nephropathy, serum levels of IgA are high in HSP and there are identical findings on renal biopsy; however, IgA nephropathy has a predilection for young adults while HSP is more predominant among children. Further, IgA nephropathy typically only affects the kidneys while HSP is a systemic disease. HSP involves the skin and connective tissues, scrotum, joints, gastrointestinal tract and kidneys. The genetic basis remains unclear except for involvement of the human leukocyte antigen region of the genome.
It is hypothesized to involve autoimmunity triggered by infections. Streptococcus strains and Parainfluenza virus are the most commonly associated pathogens, and in children Human Parvovirus B19 is a frequent viral trigger
Carrier Status: Henoch-Schönlein purpura (HSP) is not a condition that has a carrier status because it is not a hereditary or genetic disease. Instead, HSP is a form of vasculitis, which involves inflammation of small blood vessels and is often triggered by an infection or an abnormal immune response.
Mechanism: Henoch-Schönlein purpura (HSP), also known as IgA vasculitis, involves inflammation of small blood vessels, typically due to the deposition of IgA1-containing immune complexes. This inflammatory response leads to symptoms such as purpura (small blood spots under the skin), arthritis, gastrointestinal issues, and kidney involvement.

Molecular Mechanisms:
1. **IgA1 Immune Complex Formation**: HSP is characterized by the deposition of IgA1-containing immune complexes in the walls of small blood vessels.
2. **Glycosylation Abnormalities**: Research suggests that aberrant glycosylation of the hinge region of IgA1 molecules may enhance immune complex formation and deposition.
3. **Complement Activation**: The deposition of IgA1 immune complexes can activate the complement system, particularly the alternative pathway, contributing to inflammation and tissue injury.
4. **Cytokine Release**: The inflammatory process is further driven by the release of various cytokines and mediators, such as interleukins and tumor necrosis factor-alpha (TNF-α), which can amplify vascular damage and enhance leukocyte recruitment.

Understanding these molecular mechanisms helps in developing targeted therapies to better manage HSP.
Treatment: As of 2017, the optimal way to treat Henoch–Schönlein purpura remains controversial. Analgesics may be needed for the abdominal and joint pains. Wound care is warranted if skin death and ulcerations occur. It is uncertain as to whether HSP needs treatment beyond controlling the symptoms. Most people do not receive therapy because of the high spontaneous recovery rate. Experts disagree on whether to routinely use corticosteroids as treatment for HSP. However, if they are given early in the disease episode, the duration of symptoms may be shortened, and abdominal pain can improve significantly. Moreover, the chance of severe kidney problems may be reduced. A systematic review did not find any evidence that steroid treatment (prednisone) is effective at decreasing the likelihood of developing long-term kidney disease.Evidence of worsening kidney damage would normally prompt a kidney biopsy. Treatment may be indicated on the basis of the appearance of the biopsy sample; various treatments may be used, ranging from steroids by mouth to a combination of intravenous methylprednisolone (steroid), cyclophosphamide and dipyridamole followed by prednisone. Other regimens include steroids/azathioprine, and steroids/cyclophosphamide (with or without heparin and warfarin). Intravenous immunoglobulin (IVIG) is occasionally used.There is no good evidence that treating children who have HSP with antiplatelet agent prevents persistent kidney disease. There is also no evidence that treating children or adults with cyclophosphamide prevents severe kidney disease. Heparin treatment is not justified.
Compassionate Use Treatment: Henoch-Schönlein Purpura (HSP), also known as IgA vasculitis, is typically a self-limiting disease that often resolves on its own. However, more severe cases may require treatment beyond standard medical care. Compassionate use treatments, off-label or experimental treatments include:

1. **Immunosuppressive Agents**: Although primarily used for other conditions, medications like cyclophosphamide or azathioprine might be used off-label to manage severe or refractory cases.

2. **Biologics**: Rituximab, primarily used for certain autoimmune diseases and cancers, is occasionally considered off-label for severe HSP cases unresponsive to conventional therapies.

3. **Plasma Exchange (Plasmapheresis)**: An experimental approach for severe kidney involvement, removing antibodies from the blood to reduce inflammation.

They are typically considered when standard treatments such as corticosteroids and NSAIDs fail to control symptoms, especially in severe renal involvement.
Lifestyle Recommendations: Lifestyle recommendations for Henoch-Schönlein purpura (HSP) include:

1. **Rest and Avoid Overexertion:** Adequate rest is crucial to help reduce symptoms and aid recovery.
2. **Hydration:** Drink plenty of fluids to help with kidney function, especially if there are signs of kidney involvement.
3. **Healthy Diet:** Maintain a balanced diet. Avoid foods that might exacerbate gastrointestinal symptoms.
4. **Pain Management:** Over-the-counter pain relievers, like acetaminophen, can help manage pain. Avoid NSAIDs if there is kidney involvement.
5. **Skin Care:** Keep the skin moisturized to avoid additional irritation. Protect any purpura (skin lesions) from becoming infected.
6. **Regular Follow-ups:** Ensure regular medical check-ups to monitor kidney function and other potential complications.
7. **Avoid Immune System Stressors:** Avoid exposure to infections which might trigger or worsen symptoms.
8. **School/Work Adjustments:** School or work activities may need to be adjusted to accommodate periods of fatigue and other symptoms.

Always consult with a healthcare professional for personalized advice and before making any significant lifestyle changes.
Medication: Henoch-Schönlein purpura (HSP) is primarily managed through supportive care, as the condition often resolves on its own. Medications generally used include:

1. **Analgesics**: For pain relief, such as acetaminophen.
2. **Nonsteroidal anti-inflammatory drugs (NSAIDs)**: Such as ibuprofen, for joint and abdominal pain.
3. **Corticosteroids**: Such as prednisone, may be used for severe abdominal pain, kidney involvement, or other serious symptoms.
4. **Immunosuppressive agents**: In rare, severe cases with significant kidney involvement, drugs like cyclophosphamide may be considered.

Always consult a healthcare provider for individual assessment and treatment recommendations.
Repurposable Drugs: Repurposable drugs for Henoch-Schönlein purpura (HSP) are not widely established, but some corticosteroids and immunosuppressive agents may provide symptomatic relief and address severe complications. Prednisone is commonly used to reduce inflammation and manage symptoms, though its efficacy in altering the disease course is debated. Additionally, drugs like cyclophosphamide or azathioprine could be considered in severe cases with kidney involvement or other significant complications. Always consult a healthcare provider for personalized medical advice and treatment options.
Metabolites: Henoch-Schönlein purpura (HSP), also known as IgA vasculitis, involves the deposition of IgA immune complexes in the small blood vessels. Though specific metabolite profiling for HSP isn't well established, some studies have pointed towards changes in various biochemical pathways. Elevated levels of certain markers like creatinine, indicative of renal involvement, and increased inflammatory cytokines may be observed. Further detailed metabolomic studies are required to pinpoint specific metabolites associated with HSP.
Nutraceuticals: There is currently no well-established evidence to support the use of nutraceuticals specifically for Henoch-Schönlein purpura (HSP). HSP, also known as IgA vasculitis, is typically managed with supportive care, including hydration, rest, and pain relief. In severe cases, corticosteroids or other immunosuppressive treatments may be used. Always consult healthcare professionals for management and treatment options tailored to individual cases.
Peptides: Henoch-Schönlein Purpura (HSP) does not primarily involve peptides in its pathology. It is an immunoglobulin A (IgA)-mediated small vessel vasculitis. Understanding of peptides or specific nanotechnological approaches in the treatment or diagnosis of HSP is not prominent in current literature. The primary approach to managing HSP involves supportive care and, in some cases, corticosteroids for severe symptoms.