Pauci-immune glomerulonephritis

What it is

From: Orphanet

A rare small vessel vasculitis associated with rapidly progressive glomerulonephritis (GN) and clinically characterized by renal manifestations such as urinary abnormalities (hematuria and/or proteinuria) and hypertension leading to renal failure within days or weeks, and distinguished by the absence of immune deposits on immunofluorescent microscopy. The disease can occur as a renal-limited disease or as a component of systemic necrotizing small-vessel vasculitis.

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.

Cytoplasmic antineutrophil antibody positivity · Very frequent (99-80%)

The presence of autoantibodies in the serum that react against proteins predominantly expressed in cytoplasmic granules of neutrophils.

Decreased glomerular filtration rate · Very frequent (99-80%)

An abnormal reduction in the volume of fluid filtered out of plasma through glomerular capillary walls into Bowman's capsules per unit of time.

Elevated circulating creatinine concentration · Very frequent (99-80%)

An increased amount of creatinine in the blood.

Glomerulonephritis · Obligate (100%)

Inflammation of the renal glomeruli.

Microscopic hematuria · Very frequent (99-80%)

Microscopic hematuria detected by dipstick or microscopic examination of the urine.

Proteinuria · Very frequent (99-80%)

Increased levels of protein in the urine.

Renal insufficiency · Very frequent (99-80%)

A reduction in the level of performance of the kidneys in areas of function comprising the concentration of urine, removal of wastes, the maintenance of electrolyte balance, homeostasis of blood pressure, and calcium metabolism.

Small vessel vasculitis · Very frequent (99-80%)

A type of vasculitis (inflammation of blood vessel walls) that affects blood vessels that are smaller than arteries, i.e., arterioles, venules, and capilllaries.

Abnormality of the respiratory system · Frequent (79-30%)

An abnormality of the respiratory system, which include the airways, lungs, and the respiratory muscles.

Abnormality of the skin · Frequent (79-30%)

An abnormality of the skin.

Crescentic glomerulonephritis · Frequent (79-30%)

A type of extracapillary glomerulonephritis characterized by the formation of crescent-like cellular proliferation.

Fever · Frequent (79-30%)

Body temperature elevated above the normal range.

Glomerulosclerosis · Frequent (79-30%)

Accumulation of scar tissue within the glomerulus.

Abdominal pain · Occasional (29-5%)

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

Other findings in the same source

From: Orphanet

Additional reported features include Abnormality of the eye (Occasional (29-5%)); Abnormality of the upper respiratory tract (Occasional (29-5%)); Acute kidney injury (Occasional (29-5%)); Antinuclear antibody positivity (Occasional (29-5%)); Arteritis (Occasional (29-5%)); Arthralgia (Occasional (29-5%)); Cough (Occasional (29-5%)); Dyspnea (Occasional (29-5%)); Hoarse voice (Occasional (29-5%)); Macroscopic hematuria (Occasional (29-5%)). This is a selected summary, not a complete description of the condition.

When it may begin

From: Orphanet

Adolescent; Adult; Childhood; Elderly

Inheritance in the source

From: Orphanet

Not applicable

Frequency and the population described

From: Orphanet

Point prevalence: Unknown; Worldwide; Class only.

Which doctor should you see?

The suggested department for discussing Pauci-immune glomerulonephritis is Nephrology, with a nephrologist 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.

• How is kidney function being assessed over time?
• Are any current medicines or supplements relevant to kidney safety?
• Is there an individual recommendation about fluids, diet or blood pressure?

Treatment discussions and follow-up

The material gathered for this draft does not provide a complete condition-specific treatment pathway for Pauci-immune glomerulonephritis. 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.