Diffuse alveolar hemorrhage

What it is

From: Orphanet

A rare clinical situation for which there is a European and/or American orphan designation. Characteristics include diffuse bleeding into the alveolar spaces that originate from the pulmonary microvasculature, including the alveolar capillaries, arterioles and venules. Patients present with cough, dyspnea, chest pain, fever, anemia and hemoptysis.

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.

Pulmonary infiltrates · Very frequent (99-80%)

Anemia · Frequent (79-30%)

A reduction in erythrocytes volume or hemoglobin concentration.

Autoimmunity · Frequent (79-30%)

The occurrence of an immune reaction against the organism's own cells or tissues.

Cough · Frequent (79-30%)

A sudden, audible expulsion of air from the lungs through a partially closed glottis, preceded by inhalation.

Elevated erythrocyte sedimentation rate · Frequent (79-30%)

An increased erythrocyte sedimentation rate (ESR). The ESR is a test that measures the distance that erythrocytes have fallen after one hour in a vertical column of anticoagulated blood under the influence of gravity. The ESR is a nonspecific finding. An elevation may indicate inflammation or may be caused by any condition that elevates fibrinogen.

Fever · Frequent (79-30%)

Body temperature elevated above the normal range.

Ground-glass opacification on pulmonary HRCT · Frequent (79-30%)

On chest radiographs, ground-glass opacity appears as an area of hazy increased lung opacity, usually extensive, within which margins of pulmonary vessels may be indistinct. On CT scans, it appears as hazy increased opacity of lung, with preservation of bronchial and vascular margins. It is caused by partial filling of airspaces, interstitial thickening (due to fluid, cells, and/or fibrosis), partial collapse of alveoli, increased capillary blood volume, or a combination of these, the common factor being the partial displacement of air. Ground-glass opacity is less opaque than consolidation, in which bronchovascular margins are obscured.

Hemoptysis · Frequent (79-30%)

Coughing up (expectoration) of blood or blood-streaked sputum from the larynx, trachea, bronchi, or lungs.

Weight loss · Frequent (79-30%)

Reduction of total body weight.

Abnormality of head or neck · Occasional (29-5%)

An abnormality of head and neck.

Abnormality of the nervous system · Occasional (29-5%)

An abnormality of the nervous system.

Abnormality of the skeletal system · Occasional (29-5%)

An abnormality of the skeletal system.

Abnormality of the skin · Occasional (29-5%)

An abnormality of the skin.

Antineutrophil antibody positivity · Occasional (29-5%)

The presence of autoantibodies in the serum that react against neutrophils.

Other findings in the same source

From: Orphanet

Additional reported features include Antinuclear antibody positivity (Occasional (29-5%)); Antiphospholipid antibody positivity (Occasional (29-5%)); Chest pain (Occasional (29-5%)); Decreased circulating complement C3 concentration (Occasional (29-5%)); Decreased circulating complement C4 concentration (Occasional (29-5%)); Dyspnea (Occasional (29-5%)); Elevated circulating creatinine concentration (Occasional (29-5%)); Hematuria (Occasional (29-5%)); Hypoxemia (Occasional (29-5%)); Increased DLCO (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; Infancy

Inheritance in the source

From: Orphanet

Not applicable

Frequency and the population described

From: Orphanet

Point prevalence: 1-9 / 100 000; Europe; Value and class.

Which doctor should you see?

The suggested department for discussing Diffuse alveolar hemorrhage is Pulmonology, with a pulmonologist / chest physician 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.

• What is the likely explanation for the breathing symptoms?
• Would a breathing test or another investigation change management?
• If symptoms worsen, what written action plan should be followed?

Treatment discussions and follow-up

The material gathered for this draft does not provide a complete condition-specific treatment pathway for Diffuse alveolar hemorrhage. 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.