Henoch-Schonlein Purpura

Henoch-Schonlein Purpura—MMj Symptom Relief

Henoch–Schönlein purpura (HSP, also known as anaphylactoid purpura, purpura rheumatica, and Schönlein–Henoch purpura  is a disease of the skin and other organs that most commonly affects children. In the skin, the disease causes palpable purpura (small hemorrhages);  often with joint and abdominal pain. With kidney involvement, there may be a loss of small amounts of blood and protein in the urine, but this usually goes unnoticed;  in a small proportion of cases, the kidney involvement proceeds to chronic kidney disease.  HSP is often preceded by an infection, such as pharyngitis.

HSP is a systemic vasculitis (inflammation of blood vessels) and is characterized by deposition of immune complexes containing the antibody IgA;  the exact cause for this phenomenon is presently unknown. It usually resolves within several weeks and requires no treatment apart from symptom control, but may relapse in a third of the cases and cause irreversible kidney damage in about one in a hundred cases.

In adults, kidney involvement progresses to end-stage renal disease (ESRD) more often.

In ESRD, some eventually need hemodialysis or equivalent renal replacement therapy (RRT).  If a kidney transplant is found for a patient on RRT, the disease will recur in the graft (transplanted kidney) in about 35 percent  of cases, and in 11 percent , the graft will fail completely (requiring resumption of the RRT and a further transplant).

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 80percent  and abdominal pain in 62 percent.   Some include gastrointestinal hemorrhage as a fourth criterion; this occurs in 33percent 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 percent 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 percent  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.

Symptoms:

  • Abdominal pain
  • Joint pain
  • Purple spots on the skin (purpura), usually over the buttocks, lower legs, and elbows
  • Bloody stools
  • Hives or angioedema
  • Nausea
  • Diarrhea
  • Painful menstruation
  • Vomiting

Recovery and recurrence

Overall prognosis is good in most patients, with one study showing recovery occurring in 94 percent and 89 percent  of children and adults, respectively (some having needed treatment).

In children under ten, the condition recurs in about a third of all cases and usually within the first four months after the initial attack.  Recurrence is more common in older children and adults

Tests may include:

  • Skin biopsy
  • Urinalysis

The disease usually gets better on its own without treatment.   In  most cases,  HSP lasts 4 to 6 weeks, with no long-term consequences.  Sometimes symptoms come and go during this time period.  About one in three people have more than one episode (recurrence) of HSP.  Recurrences usually occur within a few months and are usually less severe than the initial episode.  Even when it lasts longer than a few months, HSP can still resolve completely.  In a few cases however, it can lead to kidney damage and permanent kidney failure.  A person with severe kidney failure must receive a bloodcleansing treatment called dialysis or a kidney transplant if the damage is permanent.

Another rare complication of HSP is intussusception of the bowel, or intestine.  With this condition, a section of the bowel folds into itself  like a telescope.  The bowel may become blocked as a result.  Surgery may be needed to correct the problem.

The causes of HSP are not fully understood.  One theory is that it may develop as an immune response to an infection.  In other words, the body’s infection-fighting system, the immune system, continues to attack cells after the infecting organisms are gone.  For example, HSP may develop after a cold.  The cold germs cause your immune system to take action.  Once the immune cells have rid the body of the germ cells, they normally rest.  But with HSP, the immune cells continue to attack other cells in the body.  This theory is also based on the fact that, in many cases, HSP symptoms recur or worsen during upper respiratory infections.

HSP has also been associated with insect bites and exposure to cold weather.  Other cases have developed after a person received vaccination for typhoid, measles, cholera, hepatitis B, or yellow fever.  Some foods, drugs, or other chemical toxins may trigger HSP as well.  Often no cause can be found.

HSP has four main symptoms:

  • Rashes and bruising. Leaking blood vessels in the skin cause rashes that look like bruises or small red dots to develop on the legs, buttocks, and back of the arms. The rash may first look like hives, then change to look like bruises. Rarely, the rash may spread to the upper part of the body, but it is usually on the parts of the body that “hang down,” like the legs, buttocks, elbows, and even earlobes. The rash does not disappear or turn pale when you press on it.
  • Abdominal pain. About two-thirds of people with HSP experience pain in the stomach that may cause vomiting or blood in the stool. This pain and bleeding can vary from mild to severe.
  • Arthritis. About 80 percent of people with HSP have pain and swelling in their joints, usually in the knees and ankles, less frequently in the elbows and wrists. These joint symptoms have no long-lasting effects, although they can be very uncomfortable while they’re present.
  • Kidney involvement. Blood in the urine (hematuria) occurs in about 40 percent of people with HSP. Often the blood cannot be seen by the naked eye, but it can be measured with a laboratory test called a urinalysis. In most people the hematuria goes away without permanent kidney damage. Protein in the urine or development of high blood pressure (hypertension) suggests more severe kidney problems.

The causes of HSP are not fully understood.  One theory is that it may develop as an immune response to an infection. In other words, the body’s infection-fighting system, the immune system, continues to attack cells after the infecting organisms are gone.  For example, HSP may develop after a cold. The cold germs cause your immune system to take action.  Once the immune cells have rid the body of the germ cells, they normally rest.  But with HSP, the immune cells continue to attack other cells in the body.  This theory is also based on the fact that, in many cases, HSP symptoms recur or worsen during upper respiratory infections.

HSP has also been associated with insect bites and exposure to cold weather.  Other cases have developed after a person received vaccination for typhoid, measles, cholera, hepatitis B, or yellow fever.  Some foods, drugs, or other chemical toxins may trigger HSP as well. Often no cause can be found.

Conventional Treatment

Pain killers may be needed for the abdominal and joint pains.  It is uncertain as to whether HSP needs treatment beyond controlling the symptoms.  Most patients do not receive therapy because of the high spontaneous recovery rate.  Steroids are generally avoided.  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 is reduced.

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 oral steroids to a combination of intravenousmethylprednisolone (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.

Treatment:  Medical Marijuana

You will be treating the symptoms of this condition-not the disease .
Recommend using whole marijuana extracts or isolated cannabinoids.  Recommend a smoke free, under the tongue delivery system, or a vaporizer.  When marijuana is smoked, it is difficult to calculate dosage, difficult to understand which cannabinoids you are receiving and at what levels.  The AMA says, “Marijuana in smoked form is not suitable as a medicine.”

Symptoms include:

  • gastro-intestinal problems
  • inflammation of vessels
  • abdominal pain
  • vomiting
  • joint inflammation
  • joint pain
  • kidney disorder
  • insomnia

Corticosteroids (Prednisone) can be given short term.  Most people gain considerable weight using Prednisone.  This medication is known to have  many side effects.  Anti-inflammatory (OTC) drugs like Ibuprophen and aspirin can be taken.  These too have unwanted effects especially on the stomach.

Medical Marijuana can be taken to relieve all the symptoms above and with little to no side effects.  A cannabis extract salve can be rubbed into the joints to relieve painful joints.  A cannabis extract can be taken orally (under the tongue) to relieve the gastro-intestinal problems, abdominal pain.  The anti-emetic properties of cannabis will stop the vomiting.  Cannabis is very effective relieving sleep problems.  Cannabis relieves inflammation.

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References:



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2. NIH Publication No. 06–5860
September 2006Miller ML, Pachman LM. Vasculitis syndromes. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 166.
3. Primer on the Rheumatic Diseases. Springer, edited by John H. Klippel, et al., 2008.
Kelley's Textbook of Rheumatology. W B Saunders Co, edited by Shaun Ruddy, et al., 2000. American College of Rheumatology. "1990 criteria for the classification of Henoch-Schönlein purpura". Retrieved 2007-12-15.
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5. Murali NS, George R, John GT, et al. (2002). "Problems of classification of Henoch Schonlein purpura: an Indian perspective". Clin. Exp. Dermatol.27 (4): 260–3. doi:10.1046/j.1365-2230.2002.01063.x. PMID 12139664.
6. Ozen S, Ruperto N, Dillon MJ, et al. (July 2006). "EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides". Ann. Rheum. Dis. 65 (7): 936–41. doi:10.1136/ard.2005.046300. PMC 1798210. PMID 16322081.
7. Weiss PF, Feinstein JA, Luan X, Burnham JM, Feudtner C (2007). "Effects of corticosteroid on Henoch-Schönlein purpura: a systematic review".Pediatrics 120 (5): 1079–87. doi:10.1542/peds.2007-0667. PMID 17974