Vasculitis

Lia Trapaidze
 Vasculitis
• DEFINITION AND PATHOGENESIS
• A clinicopathologic process characterized by inflammation of and
damage to blood vessels, compromise of vessel lumen, and
resulting ischemia.
• Clinical manifestations depend on size and location of affected
vessel.
• Most vasculitic syndromes appear to be mediated by
immunemechanisms.
• May be primary or sole manifestation of a disease or secondary to
another disease process.
• Unique vasculitic syndromes can differ greatly with regards to
clinical features, disease severity, histology, and treatment.
 PRIMARY VASCULITIS
SYNDROMES
• Churg-Strauss Syndrome
• Granulomatous vasculitis of multiple organ systems,
particularly the lung; characterized by asthma,
peripheral eosinophilia, eosinophilic tissue infiltration;
glomerulonephritis can occur.
• Polyarteritis Nodosa (PAN)
• Medium-sized muscular arteries involved; frequently
associated with arteriographic aneurysms; commonly
affects renal arteries, liver, GI tract, peripheral nerves,
skin, heart; can be associated with hepatitis B.
 PRIMARY VASCULITIS
SYNDROMES
• Microscopic Polyangiitis
• Small-vessel vasculitis that can affect the glomerulus and
lungs; mediumsized vessels also may be affected.
• Giant Cell Arteritis
• Inflammation of medium- and large-sized arteries;
primarily involves temporal artery but systemic and large
vessel involvement may occur; symptoms include
headache, jaw/tongue claudication, scalp tenderness,
fever, musculoskeletal symptoms (polymyalgia rheumatica);
sudden blindness from involvement of optic vessels is a
dreaded complication.
 PRIMARY VASCULITIS
SYNDROMES
• Takayasu’s Arteritis
• Vasculitis of the large arteries with strong predilection
for aortic arch and its branches; most common in
young women; presents with inflammatory or ischemic
symptoms in arms and neck, systemic inflammatory
symptoms, aortic regurgitation.
• Henoch-Schönlein Purpura
• Characterized by involvement of skin, GI tract, kidneys;
more common in children; may recur after initial
remission.
 PRIMARY VASCULITIS
SYNDROMES
• Cryoglobulinemic Vasculitis
• Majority of cases are associated with hepatitis C where
an aberrant immune response leads to formation of
cryoglobulin; characterized by cutaneous vasculitis,
arthritis, peripheral neuropathy, and glomerulonephritis.
• Idiopathic Cutaneous Vasculitis
• Cutaneous vasculitis is defined broadly as inflammation
of the blood vessels of the dermis; due to underlying
disease in >70% of cases (see “Secondary Vasculitis
Syndromes,” below) with 30% occurring idiopathically.
 PRIMARY VASCULITIS
SYNDROMES
• Miscellaneous Vasculitic Syndromes
• Kawasaki disease (mucocutaneous lymph
node syndrome)
• Isolated vasculitis of the central nervous
system
• Behçet’s syndrome
• Cogan’s syndrome
• Polyangiitis overlap syndrome
 SECONDARY VASCULITIS SYNDROMES

• Drug-induced vasculitis
• Serum sickness
• Vasculitis associated with infection,
malignancy, rheumatic disease
 Vasculitis
• EVALUATION
• Thorough Hx and physical exam—special reference to
ischemic manifestations and systemic inflammatory
signs/symptoms.
• Laboratories—important in assessing organ
involvement: CBC with differential, ESR, renal function
tests, UA.
• Should also be obtained to rule out other diseases:
ANA, rheumatoid factor, anti-GBM, hepatitis B/C
serologies, HIV.
 Vasculitis
• EVALUATION
• Antineutrophil cytoplasmic autoantibodies (ANCA)—associated with
granulomatosis with polyangiitis (Wegener’s), microscopic polyangiitis, and
some pts with Churg-Strauss syndrome; presence of ANCA is adjunctive and
should not be used in place of biopsy as a means of diagnosis or to guide
treatment decisions.
• Radiographs—CXR should be performed even in the absence of symptoms.
• Diagnosis—can usually be made only by arteriogram or biopsy of affected
organ(s).
• DIFFERENTIAL DIAGNOSIS
• Guided by organ manifestations. In many instances includes infections and
neoplasms, which must be ruled out prior to beginning immunosuppressive
therapy. Consideration must also be given for diseases that can mimic
vasculitis
 CONDITIONS THAT CAN MIMIC VASCULITIS

• Infectious diseases
• Bacterial endocarditis
• Disseminated gonococcal infection
• Pulmonary histoplasmosis
• Coccidioidomycosis
• Syphilis
• Lyme disease
• Rocky Mountain spotted fever
• Whipple’s disease
• Coagulopathies/thrombotic microangiopathies
• Antiphospholipid antibody syndrome
• Thrombotic thrombocytopenic purpura
 CONDITIONS THAT CAN MIMIC VASCULITIS

• Neoplasms
• Atrial myxoma
• Lymphoma
• Carcinomatosis
• Drug toxicity
• Cocaine
• Amphetamines
• Ergot alkaloids
• Methysergide
• Arsenic
• Sarcoidosis
• Atheroembolic disease
• Anti–glomerular basement membrane antibody disease (Goodpasture’s
syndrome)
• Amyloidosis
• Migraine
 Vasculitis
• TREATMENT
• Therapy is based on the specific vasculitic syndrome
and the severity of its manifestations.
• Immunosuppressive therapy should be avoided in
disease that rarely results in irreversible organ
system dysfunction or that usually does not respond
to such agents (e.g., isolated cutaneous vasculitis).
• Antiviral agents play an important role in treating
vasculitis occurring with hepatitis B or C.
 Vasculitis
• TREATMENT
• Glucocorticoids alone may control giant cell
arteritis and Takayasu’s arteritis.
• Therapy that combines glucocorticoids with
another immunosuppressive agent is
particularly important in syndromes with life-
threatening organ system involvement,
especially active glomerulonephritis.
 Vasculitis
• TREATMENT
• Frequently used agents:
• Prednisone
• Cyclophosphamide
• Rituximab
• Methotrexate
• Azathioprine
• Mycophenolate mofetil
• Plasmapheresis may have an adjunctive role in rapidly
progressive glomerulonephritis.
 Granulomatosis with Polyangiitis (GPA)
(Wegener's Granulomatosis)

• Granulomatosis with polyangiitis is characterized by necrotizing

granulomatous inflammation, small- and medium-sized vessel
vasculitis, and focal necrotizing glomerulonephritis, often with crescent
formation.
• Typically, the upper and lower respiratory tract and the kidneys are
affected, but any organ may be.
• Symptoms vary depending on the organs and systems affected. Patients
may present with upper and lower respiratory tract symptoms (eg,
recurrent nasal discharge or epistaxis, cough), followed by hypertension
and edema, or with symptoms reflecting multiorgan involvement.
• Diagnosis usually requires biopsy.
• Treatment is with corticosteroids plus an immunosuppressant.
Remission is usually possible, although relapses are common.
 Granulomatosis with Polyangiitis (GPA)
(Wegener's Granulomatosis)

• Granulomatosis with polyangiitis (GPA) occurs

in about 1/25,000 people; it is most common
among whites but can occur in all ethnic
groups and at any age. Mean age at onset is
40.
• The cause of GPA is unknown, although
immunologic mechanisms play a role. Most
patients with active generalized disease have
antineutrophil cytoplasmic antibodies (ANCA).
 Pathophysiology

• Characteristically, granulomas form with histiocytic

epithelioid cells and often with giant cells. Plasma cells,
lymphocytes, neutrophils, and eosinophils are present.
• Inflammation affects tissues as well as vessels; vasculitis may
be a small or large component of the disease.
• Micronecrosis, usually with neutrophils (microabscesses),
occurs early. Micronecrosis progresses to macronecrosis.
• A central area of necrosis (called geographic necrosis) is
rimmed by lymphocytes, plasma cells, macrophages, and
giant cells. A zone of fibroblastic proliferation with palisading
histiocytes may surround the area.
 Pathophysiology

• Nonspecific chronic inflammation and tissue necrosis occur

in the nose.
• The lungs are most likely to display the full spectrum of
histopathologic abnormalities, but diagnostic features are
not typically identified on the small tissue samples
obtained by transbronchial biopsy.
• In the kidneys, the most common finding is a pauci-
immune crescentic focal glomerulonephritis with necrosis
and thrombosis of individual loops or larger segments of
the glomerulus. Vasculitic lesions and disseminated
granulomas occur only occasionally.
 Histologic pattern

• May not correlate with the

clinical presentation
• Various histological types of
glomerulonephritis
Crescentic GN
 Symptoms and Signs

• Onset of granulomatosis with polyangiitis may be insidious or

acute; the full spectrum of the disease may take years to evolve.
• Some patients present initially with upper and lower respiratory
tract symptoms; at some point later, the kidneys are affected.
• In other patients, onset of systemic manifestations is relatively
acute; several organs and systems, such as the upper respiratory
tract, peripheral nervous system (causing multiple
mononeuropathy [mononeuritis multiplex]), kidneys (causing
glomerulonephritis), and lower respiratory tract (causing
hemorrhage,
• lung nodules, cavities, or a combination), are simultaneously
affected.
 Symptoms and Signs

• Upper respiratory tract: 

• Sinus pain, serosanguineous or purulent discharge, and
epistaxis may occur.
• The mucosa appears granular (like cobblestones) and is
friable; ulcers, thick dark crusts, and septal perforation are
common.
• Nasal chondritis can occur with swelling, pain, and collapse of
the nasal bridge (saddle nose).
• Patients may report recurrent sinusitis that has responded
inadequately to multiple antibiotic regimens and has required
one or more sinus operations before diagnosis.
• Secondary infections (eg, due to Staphylococcus aureus) may
develop. Subglottic stenosis may develop, causing symptoms
such as pain in the larynx, hoarseness, dyspnea, wheezing,
and stridor.
 Symptoms and Signs

• Ears: 
• Otitis, sensorineural hearing loss, vertigo, and chondritis may
occur. The middle ear, inner ear, and mastoids are often affected.
• Eyes: 
• Eyes may appear red and swollen. Nasolacrimal duct
inflammation and obstruction, conjunctivitis, scleritis, uveitis, or
retinal vasculitis may also occur. Inflammatory infiltrates in the
retro-orbital space (orbital pseudotumor) can cause proptosis,
compression of the optic nerve, and blindness. Extension into the
extraocular muscles leads to diplopia. If serious eye symptoms
develop, evaluation and treatment are required immediately to
prevent permanent vision loss.
 Symptoms and Signs

• Lower respiratory tract: 

• Respiratory manifestations are common. Inflammation of the
major bronchi and branches can cause localized wheezing,
postobstructive pneumonia, and atelectasis. Single or multiple
pulmonary nodules, with or without cavitation, and parenchymal
infiltrates, sometimes cause symptoms, such as chest pain,
shortness of breath, and productive cough. Dyspnea with bilateral
infiltrates, with or without hemoptysis, may indicate alveolar
hemorrhage and must be evaluated immediately.
• Heart: Coronary artery disease may occur but rarely.
• Musculoskeletal system: Patients frequently present with
myalgias, arthralgias, or nonerosive inflammatory arthritis.
 Symptoms and Signs

• Skin: Palpable purpura, tender subcutaneous nodules, papules, livedo

reticularis, or ulcers may develop.
• Nervous system: Vasculitis may cause ischemic peripheral neuropathy, brain
lesions, or extension of lesions from contiguous sites. Lesions that originate in
the sinuses or middle ear may extend directly to the retropharyngeal area
and base of the skull, leading to cranial neuropathy, proptosis, diabetes
insipidus, or meningitis.
• Kidneys: Symptoms and signs of glomerulonephritis develop. Urinary
sediment is frequently abnormal, and serum creatinine may increase rapidly.
Edema and hypertension may result. Rapidly progressive
glomerulonephritis, which is life threatening, can develop.
• Venous system: Deep venous thrombosis can affect the lower extremities
mostly when granulomatosis with polyangiitis is active.
• Other organs: Occasionally, an inflammatory mass occurs in the breasts,
kidneys, prostate, or other organs.
Granulomatosis with Polyangiitis (GPA)
 Diagnosis

• Routine laboratory tests, including urinalysis

• Tests for antineutrophil cytoplasmic antibodies
• Biopsy for definitive diagnosis
• Granulomatosis with polyangiitis should be suspected
in patients with chronic, unexplained respiratory
symptoms and signs (including otitis media in adults),
particularly if manifestations in other organ systems,
especially the kidneys, also suggest the disorder.
Routine laboratory tests are done, but ANCA testing
and biopsy yield the most specific findings.
 Diagnosis

• Routine laboratory tests include:

• ESR,
• C-reactive protein,
• CBC with differential,
• Serum albumin and total protein,
• Serum creatinine,
• Urinalysis,
• 24-hour urine protein,
• Chest x-ray.
• Chest CT without contrast is nearly always necessary because the chest x-ray
may miss nodules, masses, and/or cavitary lesions caused by granulomatosis
with polyangiitis.
• In most patients with active disease, ESR and C-reactive protein are elevated,
and serum albumin and total protein are decreased; anemia, thrombocytosis,
and mild-to-moderate eosinophilia are detected.
• Dysmorphic RBCs and RBC casts, detected during urinalysis, indicate glomerular
involvement. Proteinuria may be detected. Serum creatinine may be increased.
 Diagnosis

• Serologic testing to detect antineutrophil

cytoplasmic antibodies (ANCA) is followed by
enzyme-linked immunosorbent assay (ELISA) to
check for specific antibodies.
• Most patients with active disease have
cytoplasmic ANCA (c-ANCA), with antibodies
against proteinase-3 (PR3); these findings plus
characteristic clinical findings suggest GPA.
 Diagnosis

• Some patients with other disorders (eg, bacterial endocarditis,

cocaine abuse, systemic lupus erythematosus, amebiasis,
tuberculosis) test positive for ANCA.
• Because tests for rare diseases are likely to be falsely positive
when ordered for the general population and the positive
predictive value of a positive ANCA test is around 50%,
• ANCA testing should be reserved for patients in whom the
pretest probability for GPA or another ANCA-associated
vasculitis is at least moderately high (eg, patients with alveolar
hemorrhage, glomerulonephritis, or multiple mononeuropathy
plus other features of microscopic polyangiitis or GPA).
 Diagnosis

• A positive ANCA test does not rule out mycobacterial and

fungal infections; thus, patients with positive ANCA results and
cavitary lung lesions still require bronchoscopy and adequate
cultures and other tests for tuberculosis and fungal infections.
• ANCA testing (titre) should not be used to guide subsequent
treatment.
• During apparent remission, ANCA may increase or ANCA test
results may change from negative to positive.
• In some of these patients, symptoms do not recur; in others,
symptoms recur or worsen soon after the test is done or during
the next few weeks, months, or sometimes years.
 Diagnosis

• Biopsy should be done if possible to confirm the diagnosis of GPA.

• Clinically abnormal sites may be biopsied first.
• Biopsy of affected lung tissue is most likely to reveal characteristic findings;
open thoracotomy provides the best access.
• Biopsies of lung or sinus tissue are cultured to exclude infection.
• Renal biopsy that shows pauci-immune necrotizing focal crescentic or
noncrescentic glomerulonephritis strongly supports the diagnosis.
• Biopsy results of various tissues may also provide histologic information
that can help guide treatment (eg, renal fibrosis).
• Differential diagnosis includes other vasculitic disorders that affect small-
and medium-sized vessels.
• Infections, especially those due to slow-growing fungi or acid-fast
organisms, should be ruled out by staining and culture of the sampled
tissues.
 Treatment

• To induce remission in life- or organ-threatening GPA, high-dose

corticosteroids plus either cyclophosphamide or rituximab
• To induce remission in less severe GPA, corticosteroids and
either methotrexate or rituximab
• To maintain remission, rituximab alone or another drug such
as methotrexate, azathioprine, or mycophenolate mofetil
(rituximab plus another of these drugs, sometimes with a low
dose of a corticosteroid, if patients have multiple relapses or
GPA is difficult to control)
• Kidney transplantation if necessary.
• Treatment of granulomatosis with polyangiitis depends on the
severity of disease. A multidisciplinary approach is required for
multiorgan disease, often including a rheumatologist,
otorhinolaryngologist, pulmonologist, and nephrologist.
 Treatment

• Patients who have severe life-threatening or organ-threatening

manifestations (eg, alveolar hemorrhage, rapidly progressive
glomerulonephritis, multiple mononeuropathy with motor
involvement) require immediate hospital admission for treatment
to induce remission. These patients require high-dose
corticosteroids and cyclophosphamide or rituximab.
• Efficacies of rituximab and cyclophosphamide appear to be similar
for inducing and maintaining remission.
• Although the evidence supporting use of plasma exchange is
weaker than that for the other interventions, plasma exchange can
be added to the standard treatment regimen in patients with
severe acute renal insufficiency (particularly if the anti–glomerular
basement membrane antibody test is not known to be negative,
so that rapidly progressive glomerulonephritis has not been
excluded) or alveolar hemorrhage.
 Treatment

• Rituximab seems to be particularly helpful in patients with

recurrent disease. In one study that included patients with GPA
and other ANCA-associated vasculitides, major relapses
occurred in only 5% of patients treated with rituximab but
occurred in 29% of patients treated with azathioprine.
• Whether rituximab should be given alone or in combination with
another drug and the dose and frequency of rituximab are not
entirely clear. However, in one retrospective study, relapse rates
were lower when rituximab was combined
with methotrexate, azathioprine, or mycophenolate mofetil
than when rituximab was used alone.
• The optimal dosage of rituximab for maintenance therapy has
not been established. A corticosteroid, given at a low dose, is
often used to help maintain remission.
 Treatment

• For less severe disease, corticosteroids

and methotrexate are used to induce remission. 
• Rituximab may be used instead of methotrexate.
• For upper respiratory tract
manifestations, rituximab appears to maintain
remission better
than cyclophosphamide, methotrexate, or azathioprine.
• Corticosteroids are tapered to as low a dose as possible
or discontinued.
• Irrigation of sinuses with saline, with or
without mupirocin 2% nasal ointment, helps minimize
crusting and secondary staphylococcal infections.
 Treatment

• Treatment of subglottic stenosis is difficult. Systemic

immunosuppressants may not be effective. Intralesional
injection of long-acting corticosteroids, with gentle
progressive dilation, markedly improves outcome and
limits the need for tracheostomy.
• Patients should be taught about the disorder so that
relapses can be detected early. Patients should learn how
to test their urine for blood and protein and be instructed
to notify their physician of any sign of hematuria.
• Kidney transplantation has been successful; the risk of
relapse after transplantation is reduced compared with
maintenance dialysis treatment (possibly due in part to
use of immunosuppressants to prevent rejection).
 Goodpasture's Syndrome;
Anti-GBM Antibody Disease
• Goodpasture syndrome, a subtype of pulmonary-renal syndrome, is
an autoimmune syndrome of alveolar
hemorrhage and glomerulonephritis caused by circulating anti-
glomerular basement membrane (anti-GBM) antibodies.
• Goodpasture syndrome most often develops in genetically
susceptible people who smoke cigarettes, but hydrocarbon exposure
and viral respiratory infections are additional possible triggers.
• Symptoms are dyspnea, cough, fatigue, hemoptysis, and hematuria.
• Goodpasture syndrome is suspected in patients with hemoptysis or
hematuria and is confirmed by the presence of anti-GBM antibodies
in the blood or in a renal biopsy specimen.
• Prognosis is good when treatment is begun before onset of
respiratory or renal failure. Treatment includes plasma exchange,
corticosteroids, and immunosuppressants, such
as cyclophosphamide.
 Pathophysiology

• Goodpasture syndrome is the combination of

glomerulonephritis with alveolar hemorrhage and anti-
GBM antibodies. Goodpasture syndrome most often
manifests as diffuse alveolar hemorrhage
and glomerulonephritis together but can occasionally
cause glomerulonephritis (10 to 20%) or pulmonary
disease (10%) alone.
• Men are affected more often than women.
• Anti-GBM antibodies are directed against the
noncollagenous (NC-1) domain of the alpha3 chain of
type IV collagen, which occurs in highest concentration
in the basement membranes of renal and pulmonary
capillaries.
 Pathophysiology

• Environmental exposures—cigarette smoking, viral

URI, and hydrocarbon solvent inhalation most
commonly and pneumonia less commonly—
expose alveolar capillary antigens to circulating
antibody in genetically susceptible people, most
notably those with HLA-DRw15, -DR4, and -DRB1
alleles.
• Circulating anti-GBM antibodies bind to basement
membranes, fix complement, and trigger a cell-
mediated inflammatory response, causing
glomerulonephritis, pulmonary capillaritis, or both.
 Symptoms and Signs

• Hemoptysis is the most prominent symptom; however,

hemoptysis may not occur in patients with alveolar
hemorrhage, and patients may present with only chest x-
ray infiltrates or with infiltrates and respiratory distress,
respiratory failure, or both.
• Other common symptoms include:
• Dyspnea
• Cough
• Fatigue
• Fever
• Weight loss
• Hematuria
 Symptoms and Signs

• Up to 40% of patients have gross hematuria,

although pulmonary hemorrhage may
precede renal manifestations by weeks to
years.
• Signs vary over time and range from clear
lungs on auscultation to crackles and rhonchi.
• Some patients have peripheral edema due to
renal failure and pallor due to anemia.
 Diagnosis

• Serum anti-GBM antibody tests

• Sometimes renal biopsy
• Patients are tested for serum anti-GBM antibodies by
indirect immunofluorescence testing or, when
available, direct enzyme-linked immunosorbent assay
(ELISA) with recombinant or human NC-1 alpha3.
• Presence of these antibodies confirms the diagnosis.
• Antineutrophil cytoplasmic antibodies (ANCA) testing
is positive (in a peripheral pattern) in only 25% of
patients with Goodpasture syndrome.
 Diagnosis

• If anti-GBM antibodies are absent and patients have evidence of

glomerulonephritis (hematuria, proteinuria, red cell casts detected
with urinalysis, renal insufficiency, or a combination of these
findings), renal biopsy is indicated to confirm the diagnosis.
• A rapidly progressive focal segmental necrotizing
glomerulonephritis with crescent formation is found in biopsy
specimens in patients with Goodpasture syndrome and all other
causes of pulmonary-renal syndrome.
• Immunofluorescence staining of renal or lung tissue classically
shows linear IgG deposition along the glomerular or alveolar
capillaries. IgG deposition also occurs in the kidneys of patients
with diabetes or with fibrillary glomerulonephritis (a rare disorder
causing the pulmonary-renal syndrome), but GBM binding of
antibodies in these disorders is nonspecific and does not occur in
linear patterns.
 Treatment

• Plasma exchange
• Corticosteroids and cyclophosphamide
• Immediate survival in patients with pulmonary
hemorrhage and respiratory failure is linked to
airway control; endotracheal
intubation and mechanical ventilation are
recommended for patients with borderline
ABGs and impending respiratory failure.
• Patients with significant renal impairment may
require dialysis or kidney transplantation.
 Treatment

• Treatment is daily or every-other-day plasma exchange for 2

to 3 wk using 4-L exchanges to remove anti-GBM antibodies,
combined with a corticosteroid (usually methylprednisolone 1
g IV over 20 min once/day or every other day for 3 doses
followed by prednisone (1 mg/kg po once/day for 3 wk, then
titrated down to 20 mg po once/day for 6 to 12 mo)
and cyclophosphamide (2 mg/kg po or IV once/day for 6 to 12
mo) to prevent formation of new antibodies.
• Therapy can be tapered when pulmonary and renal function
stop improving.
• Rituximab could be used in some patients who have severe
adverse effects due to cyclophosphamide or
refuse cyclophosphamide as treatment, but it has not been
studied in patients with Goodpasture syndrome.
Idiopathic pulmonary hemosiderosis
• Idiopathic pulmonary hemosiderosis is a rare disease
that causes recurrent diffuse alveolar hemorrhage
 with no detectable underlying disorder; it occurs
mainly in children < 10 years. In patients with
idiopathic pulmonary hemosiderosis, repeated
alveolar bleeding can eventually result in pulmonary
hemosiderosis and fibrosis.
• The disease is thought to be due to a defect in the
alveolar capillary endothelium, possibly due to
autoimmune injury. Many affected patients have 
celiac disease.
Idiopathic pulmonary hemosiderosis
• Symptoms and Signs
• Symptoms and signs of idiopathic pulmonary
hemosiderosis in children include recurrent episodes
of dyspnea and cough, particularly nonproductive
cough initially. Hemoptysis occurs later.
• Children with idiopathic pulmonary hemosiderosis
may present with only failure to thrive and 
iron deficiency anemia.
• The most common symptoms in adults are
exertional dyspnea and fatigue due to pulmonary
hemorrhage and iron deficiency anemia.
Idiopathic pulmonary hemosiderosis
• Diagnosis
• Bronchoalveolar lavage
• Diagnosis of idiopathic pulmonary hemosiderosis
involves demonstration of a combination of
characteristic clinical findings, iron deficiency
anemia, and hemosiderin-laden macrophages in
bronchoalveolar lavage (BAL) fluid or lung biopsy
specimens plus no evidence of small-vessel
vasculitis (pulmonary capillaritis) or another
explanatory diagnosis; it is confirmed by lung
biopsy if other findings are inconclusive.
Idiopathic pulmonary hemosiderosis
• Treatment
• Corticosteroids
• Corticosteroids may reduce the morbidity and
mortality of acute episodes of alveolar
bleeding and may control the disease
progression of pulmonary fibrosis.
• Some patients may require additional
immunosuppressive drugs.
• Patients with celiac disease should be on a
gluten-free diet.