Pathogenesis of
pulmonary vasculitis.Semin
Respir Crit Care Med. 2004 Oct;25(5):465-74.
Vasculitis is
inflammation of blood vessels and can affect any type of vessel in any
organ. Pulmonary vasculitis usually is a component of a systemic small
vessel vasculitis. Three major forms of small vessel vasculitis that
often affect the lungs are Wegener's granulomatosis, microscopic
polyangiitis, and Churg-Strauss syndrome. These forms of vasculitis are
strongly associated with antineutrophil cytoplasmic autoantibodies (ANCA)
directed against enzymes contained in the primary granules of
neutrophils and peroxidase-positive lysosomes of monocytes. This review
discusses the evidence for a pathogenic role of ANCA. In vitro, ANCAs
can activate cytokine-primed neutrophils and monocytes resulting in
oxygen radical formation and release of lysosomal enzymes. In vivo,
antimyeloperoxidase ANCA has been shown to induce crescentic
glomerulonephritis and systemic vasculitis. Overall, the available data
suggest that ANCA are indeed a pathogenic factor in the development of
small-vessel vasculitis. Antiglomerular basement membrane (anti-GBM)
disease also causes pulmonary vasculitis through immune attack on
alveolar capillaries and glomerulonephritis through antibody mediated
injury to glomerular capillaries. Thus, there is evidence that
antibodies are important pathogenic factors in both ANCA disease and
anti-GBM disease, however, there are also indications that T cells may
play important pathogenic roles in both categories of disease as well.
Wegener's
granulomatosis (WG), microscopic polyangiitis (MPA) and Churg- Strauss
syndrome (CSS) are small to medium -vessel vasculitides that, because of
their frequent association with antineutrophil cytoplasmic antibodies (ANCA),
are usually referred to as ANCA-associated systemic vasculitides (AASV).
These diseases are
challenging to diagnose and to treat. The diagnosis of AASV is made on the
basis of clinical findings, biopsy of the involved organ and the presence
of ANCA in the serum.
Lung disease is a
very common and important clinical feature of AASV.
Vasculitis
in the lung usually involves the small vessels -arteries, capillaries and venules.
Distinguishing the ANCA-associated vasculitides from
other forms of vasculitis or nonvasculitic processes (such as infection) can
be particularly difficult.
ANCA are
associated with small sized vessel vasculitis ; one subtype is an
antibody against myeloperoxidase (MPO), which stains in a perinuclear
pattern (P-ANCA) indirect immunofluorescence (IIF) using a neutrophil
substrate, and the other subtype is an antibody against
proteinase-3(PR-3), which stains in a diffuse granular cytoplasmic
pattern ANCA by IIF. PR-3 ANCA is more specific in Wegener's
granulomatosis than the other primary vasculitides. MPO-ANCA can
be found in microscopic polyangiitis (MPA), Churg Strauss
Syndromes(CSS)
Pulmonary vasculitis.Proc
Am Thorac Soc. 2006;3(1):48-57.
Pulmonary
vasculitis describes a number of distinct disorders that are
pathologically characterized by the destruction of blood vessels. The
clinical manifestations of each disorder are defined by the size, type,
and location of the affected vasculature. The clinical approach to these
disorders rests upon an astute clinician considering the diagnosis and
identifying the specific patterns of clinical, radiologic, laboratory,
and pathologic abnormalities. Lung involvement is most commonly seen
with the primary, idiopathic, small-vessel, or antineutrophil
cytoplasmic antibody-associated vasculitides; Wegener's granulomatosis,
microscopic polyangiitis, and Churg-Strauss syndrome. However, primary,
idiopathic medium and large-vessel vasculitis, primary immune
complex-mediated vasculitis, and secondary vasculitis are all capable of
presenting with lung involvement. In this article, we focus on the more
common, antineutrophil cytoplasmic antibody-associated disorder,
vasculitides.
ANCA are
associated with small sized vessel vasculitis; one subtype is an
antibody against myeloperoxidase(MPO), which stains in a perinuclear
pattern(P-ANCA) indirect immunofluorescence(IIF) using a neutrophil
substrate, and the other subtype is an antibody against
proteinase-3(PR-3), which stains in a diffuse granular cytoplasmic
pattern ANCA by IIF. PR-3 ANCA is more specific in Wegener's
granulomatosis(WG) than the other primary vasculitides. MPO-ANCA can
be found in microscopic polyangiitis (MPA), Churg Strauss
Syndromes(CSS), drug-induced vasculitis, and environmental
factor-induced such as silicosis vasculitis more frequently than WG.
The value of the IIF test for ANCA detection can be greatly increased
by the addition of a standardized antigen-specific ELISA. The
intra-assay and inter-assay CV of the MPO and PR-3 ELISA were 6.6 to
4.8%, respectively. Close ANCA titer correlation was shown between
MPO-ANCA ELISA and the activity of ANCA associated vasculitis. Renal
manifestations and pulmonary manifestations are observed in 70-90% of
AAV as the initial manifestation. The changes in titers of ANCA seem
to reflect disease activity in 60-70% of AAV patients. A combination
of steroids and immunosuppressive drugs is effective in relieving the
clinical symptoms of AAV.
Update in the
diagnosis and management of pulmonary vasculitis.Chest.
2006 Feb;129(2):452-65.
The term
vasculitis encompasses a number of distinct clinicopathologic disease
entities, each of which is characterized pathologically by cellular
inflammation and destruction of the blood vessel wall, and clinically by
the types and locations of the affected vessels. While multiple
classification schemes have been proposed to categorize and simplify the
approach to these diseases, ultimately their diagnosis rests on the
identification of particular patterns of clinical, radiologic,
laboratory, and pathologic features. While lung involvement is most
commonly seen with the primary idiopathic, small-vessel or
antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides of
Wegener granulomatosis, microscopic polyangiitis, and Churg-Strauss
syndrome, one should remember that medium-vessel vasculitis (ie, classic
polyarteritis nodosa), large-vessel vasculitis (ie, Takayasu arteritis),
primary immune complex-mediated vasculitis (ie, Goodpasture syndrome),
and secondary vasculitis (ie, systemic lupus erythematosus) can all
affect the lung. However, for the purpose of this review, we will focus
on the ANCA-associated vasculitides.
Capillaritis with
severe pulmonary hemorrhage may be seen in all three diseases or as isolated
single organ disease.
Of the other causes of small vessel
vasculitis of the lungs SLE is the most frequent. Alveolar hemorrhage may be
the presenting manifestations and is usually associated with
glomerulonephritis. Immune complexes are present in the lungs with a
granular distribution.
Less commonly, pulmonary capillaritis has
been reported in patients with polymyositis and occasionally in the other
collagen vascular diseases.
Pulmonary disease is uncommon in
Henoch-Schonlein purpura and in the vasculitis of cryoglobulinanemia.
In Goodpasture’s syndrome primary
capillaritis is associated with linear deposition of IgG and complement
along the basement membrane.
The
dual
circulation of the lung may be one reason why the response to the systemic vasculitic disorders is unique, providing a spectrum of diseases
different to that seen in other organs. Eg. The classical lesions
of polyarteritis nodosa, which is a disease of systemic muscular arteritis,
are very rarely seen in the lungs; whereas certain diseases such as
Wegener’s granulomatosis typically involve the lower respiratory tract.
Recent classifications separate
the types of vasculitis according to vessel size and type of immune response
involved.
With rare exceptions, those that involve the lung cause disease of
the small vessels - small muscular arteries, arterioles, capillaries, and venules. Some types of small vessel vasculitis in the lung are also frequently
associated with glomerulonephritis, producing the "pulmonary-renal"
syndromes.
Very rarely the
elastic pulmonary arteries may be involved by giant cell arteritis or
Takayasu arteritis.
In addition to small vessel disease, Behcet’s syndrome
typically produces aneurysms of the large elastic pulmonary arteries.
The interpretation of biopsy material requires detailed information of the
clinical, radiological and serological findings.
Respiratory system involvement in ANCA-associated systemic vasculitides.Sarcoidosis
Vasc Diffuse Lung Dis. 2005 Dec;22 Suppl
1:S40-8.
BACKGROUND AND AIM
OF THE WORK: The respiratory system may be involved in all systemic
vasculitides (SV), although with a variable frequency. Lung disease is a
very common and important feature of the antineutrophil cytoplasmic
antibodies (ANCA)-associated SV (AASV), such as Wegener's granulomatosis
(WG), Churg-Strauss syndrome (CSS), and microscopic polyangiitis (MPA).
The aim of the work is to review the clinical findings, as well as the
radiological and pathological features of respiratory system involvement
in AASV. METHODS: A detailed search via the PubMed index from the
National Library of Medicine, covering the period from 1980 to December
2004, was accomplished. RESULTS: In WG, almost all patients have either
upper airway or lower respiratory tract disease. Solitary or multiple
nodules and masses are the most common findings on chest radiograph.
Asthma is a main symptom of CSS, often preceded by allergic rhinitis,
frequently complicated by nasal polyposis and sinusitis. Pulmonary
transient and patchy alveolar infiltrates are the most common
radiographic findings. In MPA, diffuse alveolar haemorrhage (DAH) due to
alveolar capillaritis is the most frequent manifestation of the
respiratory involvement, clinically expressing with haemoptysis,
respiratory distress and anaemia. CONCLUSIONS: The involvement of the
respiratory system is a very common and important feature of AASV. There
is substantial overlap in many of the clinical pulmonary features of
AASV. In some cases, distinguishing between these diseases on the basis
of the clinical features alone is difficult and pathological assessment
is needed.
Respiratory system involvement in antineutrophil cytoplasmic-associated
systemic vasculitides: clinical, pathological, radiological and
therapeutic considerations.Drugs
R D. 2007;8(1):25-42.
Wegener's
granulomatosis (WG), microscopic polyangiitis (MPA) and Churg- Strauss
syndrome (CSS) are small-vessel vasculitides that, because of their
frequent association with antineutrophil cytoplasmic antibodies (ANCA),
are usually referred to as ANCA-associated systemic vasculitides (AASV).
The diagnosis of AASV is made on the basis of clinical findings, biopsy
of an involved organ and the presence of ANCA in the serum. Lung disease
is a very common and important clinical feature of AASV. In WG, almost
all patients have either upper airway or lower respiratory tract
disease. Solitary or multiple nodules, frequently cavitated, and masses
are the most common findings on chest radiography. Asthma is a cardinal
symptom of CSS, often preceded by allergic rhinitis. Pulmonary transient
and patchy alveolar infiltrates are the most common radiographic
findings. In MPA, diffuse alveolar haemorrhage as a result of alveolar
capillaritis is the most frequent manifestation of respiratory
involvement, and is clinically expressed as haemoptysis, respiratory
distress and anaemia. However, diffuse alveolar haemorrhage may also be
subclinical and should be suspected when a chest radiograph demonstrates
new unexplained bilateral alveolar infiltrates in the context of falling
haemoglobin levels. Normal and high-resolution CT have a higher
sensitivity than chest radiography for demonstrating airway, parenchymal
and pleural lesions. However, many of these radiological findings are
nonspecific and, therefore, their interpretation must take into account
all clinical, laboratory and pathological data. Therapy of AASV is
commonly divided into two phases: an initial 'remission induction'
phase, in which more intensive immunosuppressant therapy is used to
control disease activity, and a 'maintenance' phase, which uses less
intensive therapy, for maintaining disease remission while lowering the
risk of adverse effects of immunosuppressant drugs. In patients with
AASV refractory to standard therapy with corticosteroids and oral
cyclophosphamide, new therapeutic options are now available. Recurrence
of pulmonary symptoms suggesting a flare indicates the need for a
careful search for an opportunistic lung infection or iatrogenic
pulmonary complications. In conclusion, involvement of the respiratory
system is a very common and important organ manifestation of AASV.
Respiratory system involvement comprises a wide spectrum of clinical
features and radiological findings, and because of its frequency and
prognostic significance, a complete assessment of the respiratory system
should be included in the work-up of all patients with AASV.
Respiratory
system involvement in systemic vasculitides.Clin
Exp Rheumatol. 2006 Mar-Apr;24(2 Suppl
41):S48-59.
The respiratory
system may be involved in all systemic vasculitides (SV), although with
a variable frequency. Lung disease is a very common and important
feature of the antineutrophil cytoplasmic antibodies (ANCA)-associated
SV (AASV), such as Wegener's granulomatosis (WG), Churg-Strauss syndrome
(CSS), and microscopic polyangiitis (MPA). In WG, almost all patients
have either upper airway or lower respiratory tract disease. Solitary or
multiple nodules and masses are the most common findings on chest
radiograph. Asthma is a cardinal symptom of CSS, often preceded by
allergic rhinitis, frequently complicated by nasal polyposis and
sinusitis. Pulmonary transient and patchy alveolar infiltrates are the
most common radiographic findings. In MPA, diffuse alveolar hemorrhage (DAH)
due to alveolar capillaritis is the most frequent manifestation of the
respiratory involvement, clinically expressing with hemoptysis,
respiratory distress and anemia. However, DAH may be subclinical and has
to be suspected when chest radiograph demonstrates new unexplained
bilateral alveolar infiltrates, in the face of falling hemoglobin
levels. In giant cell arteritis, the most frequent respiratory symptom
is cough, usually non-productive, persistent, and responsive to
corticosteroids. In Takayasu arteritis, pulmonary involvement is
frequently subclinical and detectable by non-invasive techniques.
Pulmonary involvement is rare in polyarteritis nodosa, Kawasaki disease,
Henoch-Schonlein purpura and cryoglobulinemic vasculitis.In conclusion,
the involvement of the respiratory system is a very common and important
feature of AASV, whereas is less frequent in other SV. It comprises a
wide spectrum of clinical features and radiological findings, and may
have a prognostic significance. The assessment of the respiratory system
should be included in the work-up of all patients with SV, especially of
those with AASV.
Immune
mediated intra-alveolar haemorrhage in the adult.Rev
Mal Respir. 2006 Feb;23(1 Suppl):3S61-73.
INTRODUCTION:
The diagnosis of diffuse intra-alveolar haemorrhage (DAH) is suggested
by the combination of haemoptysis, anaemia and pulmonary infiltrates.
Broncho-alveolar lavage produces macroscopically haemorrhagic fluid
and/or haemosiderin laden macrophages. The diagnostic approach should
allow distinction between immune mediated and other causes on account of
the therapeutic implications. BACKGROUND: The main immunological causes
are small and medium vessel vasculitis (Wegener's granulomatosis,
microscopic polyangeitis), lupus and Goodpasture's syndrome. Other
immune disorders are only rarely involved. The association of DAH with
an acute glomerulonephritis, indicating the pulmonary-renal syndrome,
extra-thoracic involvement and immunological abnormalities suggest an
immune aetiology. Immunosuppressant treatment should be started as soon
as possible with corticosteroids often combined with intravenous
cyclophosphamide. Plasmapharesis is indicated for Goodpasture's syndrome
and poorly responding lupus. Aggravating factors such as hypervolaemia
and disorders of haemostasis should be searched for and treated.
Hospital mortality is close to 20%. VIEWPOINT AND CONCLUSION: Immune
mediated DAH is a disorder whose rarity justifies the establishment of a
national registry with the aim of developing standardised diagnostic and
therapeutic strategies.
Update in the
diagnosis and management of pulmonary vasculitis.Chest.
2006 Feb;129(2):452-65.
The term
vasculitis encompasses a number of distinct clinicopathologic disease
entities, each of which is characterized pathologically by cellular
inflammation and destruction of the blood vessel wall, and clinically by
the types and locations of the affected vessels. While multiple
classification schemes have been proposed to categorize and simplify the
approach to these diseases, ultimately their diagnosis rests on the
identification of particular patterns of clinical, radiologic,
laboratory, and pathologic features. While lung involvement is most
commonly seen with the primary idiopathic, small-vessel or
antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides of
Wegener granulomatosis, microscopic polyangiitis, and Churg-Strauss
syndrome, one should remember that medium-vessel vasculitis (ie, classic
polyarteritis nodosa), large-vessel vasculitis (ie, Takayasu arteritis),
primary immune complex-mediated vasculitis (ie, Goodpasture syndrome),
and secondary vasculitis (ie, systemic lupus erythematosus) can all
affect the lung. However, for the purpose of this review, we will focus
on the ANCA-associated vasculitides.
Diagnosis and
treatment of primary small vessel vasculitis with involvement of lungs.
Zhonghua Jie He He Hu Xi Za Zhi.
1999 Jun;22(6):347-50.
OBJECTIVE: To
investigate the clinical characteristics of primary small vessel
vasculitis with involvement of lungs. METHODS: 13 cases of primary small
vessel vasculitis with involvement of lungs from 1993 to 1998 were
analyzed retrospectively. RESULTS: Among 13 cases 7 were microscopic
polyangiitis (MPA) and 6 were Wegener granulomatosis (WG). The ages of
onset were from 17 to 68 years old with average age 48.8 years old.
69%(9/13) were ANCA positive, among them 100%(7/7) MPA were ANCA
positive (6/7 were P-ANCA positive, 1/7 was C-ANCA positive), while
33%(2/6) WG were ANCA positive (one case of P-ANCA and another case of
C-ANCA was positive respectively). The major symptoms of respiratory
system included hemoptasis 69% (9/13), dyspnea 23%(3/13), dry cough
15%(2/13) and chest pain 15%(2/13). The chest x-rays showed multiple
patchy shadows in both lungs were mainly found in MPA (3/7) and single
or multiple masses or nodular shadows were mainly found in WG (5/6) with
or without cavity formation. The appearance of lungs in MPA 71% (5/7)
had been explained as "pulmonary infection" and that of WG had been
explained as "primary lung cancer or metastatic carcinoma". Symptoms of
respiratory system might occur before (3/5) or after (2/5) occurrence of
acute renal failure. The treatments with corticosteroid and CTX were
effective in these cases, in particularly, pulmonary lesions improved
obviously. CONCLUSIONS: It is very difficult to make diagnosis of
primary small vessel vasculitis with involvement of lungs and this
should be paid more attention. ANCA detection is very useful in
diagnosis of MPA. Corticosteroid and CTX are most effective in treating
these diseases.
Pulmonary vasculitis.Semin
Respir Crit Care Med. 2004 Oct;25(5):483-9.
This review
summarizes the radiological manifestations of the vasculitides of proven
or presumed immunologic origin in which the inflammatory reaction is
directed primarily against the vessel wall. These include Wegener's
granulomatosis, Churg-Strauss syndrome, Takayasu's arteritis, Behcet's
syndrome, Goodpasture's syndrome, and microscopic polyangiitis. Chest
radiography is used routinely in the initial evaluation and follow-up of
these patients. The radiographic findings however are nonspecific and
need to be interpreted together with the clinical findings. Computed
tomography (CT) plays an increasingly important role in the assessment
of patients with vasculitis and, in the proper clinical context, allows
a confident diagnosis of some of these entities. Magnetic resonance
imaging and positron emission tomography play a limited role. The
characteristic imaging manifestations of the various vasculitides are
reviewed and illustrated.
Pathology of pulmonary vasculitis.Semin
Respir Crit Care Med. 2004
Oct;25(5):475-82.
There are three
major vasculitis syndromes that affect the lung: Wegener's
granulomatosis (WG), Churg-Strauss syndrome (CSS), and microscopic
polyangiitis (MPA). The pathology of pulmonary vasculitis is complicated
because it requires correlation with clinical, laboratory, and
radiological features; there is overlap in some histological features
among the vasculitis syndromes; biopsies early in the course of disease
or after therapy may show atypical or incomplete histological features;
the differential diagnosis is complex and includes infection that should
not be treated with corticosteriods or immunosupressive agents; and few
pathologists have much experience with these cases. Major histological
features of necrosis, granulomatous inflammation, and vasculitis
characterize WG. The inflammatory consolidation consists of a mixture of
neutrophils, lymphocytes, plasma cells, macrophages, giant cells, and
eosinophils. Necrosis may take the form of neutrophil microabscesses or
geographic necrosis. Granulomas may take several forms, including
scattered or loose clusters of giant cells, palisading histiocytes or
giant cells lining the border of geographic necrosis or microabscesses,
and palisading microgranulomas. Sarcoidal granulomas are very rare. CSS
may show eosinophilic pneumonia, allergic granulomas, and eosinophilic
vasculitis. Asthmatic bronchitis may also be present. Biopsies from CSS
patients are rare because this syndrome is usually diagnosed clinically.
Microscopic polyangiitis demonstrates neutrophilic capillaritis and
diffuse alveolar hemorrhage.
Does infection play
a role in the pathogenesis of pulmonary vasculitis?Semin
Respir Infect. 2003 Mar;18(1):17-22
The pulmonary vasculitides are a heterogeneous group of systemic
inflammatory diseases of unknown etiology with potential for significant
morbidity. The syndromes with particular predilection for the
respiratory tract are Wegener's granulomatosis, microscopic polyangiitis,
and Churg-Strauss syndrome. The discovery of antineutrophil cytoplasmic
antibodies (ANCA) in these disorders has facilitated their diagnosis and
contributed to the understanding of their pathogenesis. Clinical studies
and some animal models suggest a disease-modifying role for
antimicrobial therapy in ANCA-associated vasculitis. Nasal colonization
with Staphylococcal aureus is an independent risk factor for relapse of
Wegener's granulomatosis. This evidence suggests infectious pathogens as
potential triggers of a cascade of events that result in vascular
inflammation. Multiple laboratory studies have contributed to a coherent
and plausible theory about the pathogenesis of ANCA-associated
vasculitis in which infection plays a critical role. In susceptible
individuals immune tolerance may break down and ANCA production
resulting from molecular mimicry ensues. In addition, bacterial
superantigens may serve as potent stimulators of the immune system. In
this context, ANCA directed against proteinase 3 or myeloperoxidase may
interact with their target antigens expressed on the surface of
activated neutrophils, leading to an enhanced and perpetuated
inflammation of vessels. Despite significant advances, the precise
connection between infections and pulmonary vasculitis remains poorly
understood, and further studies into the pathogenesis of these diseases
are needed.
New perspectives in
pulmonary angiitis. From pulmonary angiitis and granulomatosis to ANCA
associated vasculitis. Sarcoidosis
Vasc Diffuse Lung Dis. 2000
Mar;17(1):33-52.
Traditionally
clinical and histopathological features were mainly relied on for
classification of vasculitis and granulomatosis of the lung. These can
be complemented by immunodiagnostic features which contribute to the
classification as well as to the understanding of the pathogenesis of
these disorders. Previously five conditions were classified together
under the heading "pulmonary angiitis and granulomatosis" (PA & G),
mainly on the basis of histological similarities. These conditions have
in common a granulomatous histopathology together with necrosis of
varying degree, pulmonary vasculitis and occasionally systemic
vasculitis. The introduction of novel immunodiagnostic methods led to
different approaches of classification, specifically a separation
between a group of disorders associated with antinuclear antibodies
(ANA), referred to as collagen vascular diseases, and a group of
systemic autoimmune diseases unrelated to ANA, referred to as primary
systemic vasculitides. Among the latter, Wegener's granulomatosis (WG),
Churg-Strauss syndrome (CSS) and microscopic polyangiitis (MPA) are
associated with a group of autoantibodies (antineutrophil cytoplasmic
antibodies--ANCA) which separate them from other members of the PA & G
group. The granulomatous and vasculitic disorders WG and CSS together
with the non-granulomatous small-vessel vasculitis MPA now form a new
group of diseases ('ANCA-associated vasculitides') which have many
clinical, serological and immunohistochemical features in common.
Collagen vascular diseases (CVD) are serologically characterized by
distinct subspecificities of antinuclear antibodies (ANA), sometimes
pronounced hypergammaglobulinaemia, complement consumption and immune
deposits (antigen-antibody-complement complexes) which are common in
situ in immune-complex vasculitis. In this article newer aspects of the
clinical course, the immunodiagnostic procedure, and the
immunopathogenesis of the relatively large group of pulmonary angiitis
will be described.
Cavitating lung lesions
in the course of ANCA-associated vasculitis: differential diagnostic
aspects. Aktuelle Radiol. 1998
May;8(3):114-8.
Antineutrophil
cytoplasmatic antibodies (ANCA)-associated vasculitides (Wegener's
granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome) show
quite variable courses. Clinical features of the full blown generalized
systemic vasculitis are usually found in the respiratory tract and the
kidney. Pulmonary involvement of Wegener's granulomatosis shows commonly
nodules and cavitations but also diffuse alveolar hemorrhage. We report
the case of a 57 year-old man suffering from dyspnea, thoracal pain,
arthralgia, purpura, scleritis and tinitus. Specimen of the kidney
showed segmental glomerulosclerosis and tubulointerstitial nephritis.
Because of the presence of cANCA Wegener's disease was assumed.
Pulmonary infiltrates developed under immunosuppressive treatment with
cyclophosphamid. As differential diagnosis of the pulmonary infiltrates,
we considered invasive pulmonary aspergillosis as well as infiltrates
due to Wegener's granulomatosis. In spite of maximal therapeutic
management of patient died of respiratory and cardiovascular failure.
The findings at autopsy showed distinct invasive pulmonary aspergillosis
and perifocal hemorrhage.
The spectrum of pulmonary
vasculitis.Monaldi
Arch Chest Dis. 1996 Feb;51(1):35-8.
Wegener's
granulomatosis and Churg-Strauss syndrome are the predominant pulmonary
vasculitides. Next in frequency are the various diffuse alveolar
haemorrhage syndromes, which may be related to the antineutrophil
cytoplasmic autoantibody (ANCA)-associated diseases, such as Wegener's
granulomatosis and Churg-Strauss syndrome, or may be a part of a
collagen vascular disease, such as lupus erythematosus, or associated
with antiglomerular basement membrane antibody (AGBM) and fall within
the definition of Goodpasture's syndrome. Whereas Behclet's disease and
Takayasu's arteritis have major pulmonary manifestations, they are rare
diseases. Entities previously confused with pulmonary vasculitis include
lymphomatoid granulomatosis or polymorphic reticulosis, and benign
lymphocytic angiitis and granulomatosis, which are probably in the
spectrum of T-cell lymphomas. Necrotizing sarcoid and sarcoidosis can
involve blood vessels, but do not follow a typical course associated
with the traditional concept of vasculitis.
The granulomatous
vasculitides frequently involve the lung. These syndromes include
Wegener's granulomatosis, allergic angiitis and granulomatosis, and the
polyangiitis overlap syndrome. Although not a true systemic vasculitis,
necrotizing sarcoid granulomatosis also represents a type of pulmonary
vasculitis. It is clear that many infectious agents can cause a picture
in the lung that can be confused with granulomatous vasculitis and that
an infectious process must be ruled out before a diagnosis of pulmonary
vasculitis can be established. Pulmonary vasculitis can be associated
with the hypersensitivity vasculitides, and pulmonary hemorrhage can be
secondary to pulmonary capillaritis. Therapy of the hypersensitivity
vasculitides consists of removing the offending antigen and instituting
a limited course of corticosteroids. If the vasculitis is secondary to
an underlying disease, such as lymphoma, therapy should be directed at
the primary disease. Combination therapy with cyclophosphamide and
corticosteroids is effective in the systemic vasculitides and the 5-yr
survival rate is approximately 90%.