Immunoglobulin A vasculitis

What it is

From: Orphanet

A rare, small-vessel vasculitis characterized by skin purpura, arthritis, abdominal and/or renal involvement, IgA tissue deposits (arterioles, capillaries, and venules) and circulating IgA immune complexes.

Reported clinical features and what the terms mean

The following findings are associated with this condition in Orphanet. They are not a checklist for diagnosing yourself, and they do not all occur in every affected person. Some are examination, imaging or laboratory findings that cannot be recognised at home.

The frequency labels describe how often a finding was reported among people with the condition in the source. They do not give the chance that a person with that symptom has the condition. Definitions below reproduce HPO terminology; they explain the term, not the likely severity in an individual.

Abdominal pain · Very frequent (99-80%)

An unpleasant sensation characterized by physical discomfort (such as pricking, throbbing, or aching) and perceived to originate in the abdomen.

Arthralgia · Very frequent (99-80%)

Joint pain.

Bruising susceptibility · Very frequent (99-80%)

An ecchymosis (bruise) refers to the skin discoloration caused by the escape of blood into the tissues from ruptured blood vessels. This term refers to an abnormally increased susceptibility to bruising. The corresponding phenotypic abnormality is generally elicited on medical history as a report of frequent ecchymoses or bruising without adequate trauma.

Hematuria · Very frequent (99-80%)

The presence of blood in the urine. Hematuria may be gross hematuria (visible to the naked eye) or microscopic hematuria (detected by dipstick or microscopic examination of the urine).

Nausea and vomiting · Very frequent (99-80%)

Nausea is a commonly encountered symptom that has been defined as an unpleasant painless subjective feeling that one will imminently vomit. Vomiting has been defined as the forceful expulsion of the contents of the stomach, duodenum, or jejunum through the oral cavity. While nausea and vomiting are often thought to exist on a temporal continuum, this is not always the case. There are situations when severe nausea may be present without emesis and less frequently, when emesis may be present without preceding nausea.

Purpura · Very frequent (99-80%)

Purpura (from Latin: purpura, meaning purple) is the appearance of red or purple discolorations on the skin that do not blanch on applying pressure. They are caused by bleeding underneath the skin. This term refers to an abnormally increased susceptibility to developing purpura. Purpura are larger than petechiae.

Pustule · Very frequent (99-80%)

A small elevation of the skin containing cloudy or purulent material usually consisting of necrotic inflammatory cells.

Skin rash · Very frequent (99-80%)

A red eruption of the skin.

Vasculitis · Very frequent (99-80%)

Inflammation of blood vessel.

Gastrointestinal infarctions · Very frequent (99-80%)

Anorexia · Frequent (79-30%)

Lack of desire to eat (loss of appetite).

Arthritis · Frequent (79-30%)

Inflammation of a joint.

Erythema · Frequent (79-30%)

Redness of the skin, caused by hyperemia of the capillaries in the lower layers of the skin.

Fever · Frequent (79-30%)

Body temperature elevated above the normal range.

Other findings in the same source

From: Orphanet

Additional reported features include Infectious encephalitis (Frequent (79-30%)); Migraine (Frequent (79-30%)); Myalgia (Frequent (79-30%)); Orchitis (Frequent (79-30%)); Skin ulcer (Frequent (79-30%)); Vascular skin abnormality (Frequent (79-30%)); Angioedema (Occasional (29-5%)); Edema (Occasional (29-5%)); Episcleritis (Occasional (29-5%)); Gastrointestinal hemorrhage (Occasional (29-5%)). This is a selected summary, not a complete description of the condition.

When it may begin

From: Orphanet

Childhood

Inheritance in the source

From: Orphanet

Not applicable

Frequency and the population described

From: Orphanet

Annual incidence: 6-9 / 10 000; Korea, Republic of; Value and class. Annual incidence: 1-5 / 10 000; France; Value and class. Annual incidence: 1-9 / 100 000; Croatia; Value and class. Annual incidence: 1-5 / 10 000; United Kingdom; Value and class.

Which doctor should you see?

The suggested department for discussing Immunoglobulin A vasculitis is Rheumatology, with a rheumatologist as the relevant type of clinician. General physician / Family Medicine; paediatrician for children. Referral depends on symptoms.

This is an editorial referral starting point. The appropriate clinic depends on the person’s age, symptoms, previous diagnosis and local services. The first clinician can decide whether another specialty or a team is needed; a department label does not confirm the diagnosis.

How to prepare for an assessment

Bring a short timeline of the main symptoms: when they first appeared, whether they are constant or episodic, what seems to change them, and how they affect daily activities. Include previous reports, discharge summaries, current medicines and supplements, allergies, and any relevant family history. A dated record is more useful than trying to match every feature in an online article.

Ask the clinician what is already established and what remains uncertain. If a test is suggested, ask what question it answers, what its limitations are and how the result would change the next step. The information here is not an instruction to arrange every possible test. In children, bring growth, developmental and school information if it is relevant to the concern.

• Are the findings inflammatory, structural or due to another mechanism?
• Is there evidence that other organs need assessment?
• How will function and any treatment-related risks be monitored?

Treatment discussions and follow-up

The material gathered for this draft does not provide a complete condition-specific treatment pathway for Immunoglobulin A vasculitis. That gap does not mean that treatment is unavailable. A clinician needs to establish the diagnosis and review current guidance before recommending medicines, procedures, rehabilitation or other support.

Before leaving the appointment, clarify the next review date, who will communicate results, and whom to contact if the situation changes. Discuss difficulties with sleep, work, school, mobility, eating or emotional wellbeing when these are relevant. Practical support may require coordination between the treating clinician and other services.

The collected references do not establish a complete prevention or long-term outlook section for this entry. Missing information should not be interpreted as proof that prevention is impossible or that a particular outcome is inevitable. Ask what is known for the exact subtype, stage and personal circumstances, and which uncertainties remain.

When to seek emergency help

Severe breathing difficulty, collapse, new stroke-like symptoms, a seizure that is prolonged or repeated without recovery, uncontrolled major bleeding, or an immediate risk of self-harm require emergency help. In India, call 112 or reach the nearest emergency department. This is a general, non-exhaustive warning list; it is not a condition-specific triage tool.