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Isolated pulmonary capillaritis and diffuse alveolar hemorrhage in
rheumatoid arthritis and mixed connective tissue disease.Chest.
1998 Jun;113(6):1609-15.
STUDY OBJECTIVES:
To demonstrate that pulmonary capillaritis and diffuse alveolar
hemorrhage (DAH) occur and are isolated to the lung and therefore not
part of systemic vasculitis at the time of the DAH episode in
rheumatoid arthritis (RA) and mixed connective tissue disease (MCTD).
DESIGN: Lung biopsy specimens from patients with DAH were reviewed and
those with the histologic features of pulmonary capillaritis were
identified. SETTING: The patients were selected from seven Denver-area
general hospitals. PATIENTS: Fifty-eight patients with biopsy specimen
proved pulmonary capillaritis (1991 to 1997) were identified and
classified according to disease. Three patients met the American
Rheumatism Association criteria for RA and one patient fulfilled
clinical and serologic criteria for MCTD. INTERVENTIONS: All clinical,
laboratory, and radiographic data on initial presentation and at
follow-up periods were extracted from the charts of the four study
patients. Histologic slides were reviewed and immunofluorescent
studies of lung tissue were performed. MEASUREMENTS AND RESULTS: All
four patients had a connective tissue disease diagnosis prior to the
DAH episode. Symptoms referable to pulmonary capillaritis were of
short duration (2 to 14 days) and there was no clinical or serologic
evidence for an accompanying systemic vasculitis, in particular
glomeronephritis. Three patients, two with RA and one with MCTD,
demonstrated pulmonary immune complex deposition. Three resolved their
illness following IV methylprednisilone and cyclophosphamide therapy.
One RA patient died following a myocardial infarction. In the three
survivors, no further episodes of DAH have occurred after a mean of 24
months (range, 10 to 48 months). CONCLUSIONS: To our knowledge, these
are the first cases of DAH due to pulmonary capillaritis documented to
complicate RA and MCTD. The capillaritis was not part of a systemic
vasculitis at the time of the DAH episode, but rather represented an
isolated small-vessel vasculitis of the lungs in this group of
patients. Immune complex deposition may be involved in the
pathogenesis.
Pulmonary
capillaritis in children: a review of eight cases with comparison to
other alveolar hemorrhage syndromes.J
Pediatr. 2005 Mar;146(3):376-81.
OBJECTIVE: To
review clinical, laboratory, and outcome characteristics of children
diagnosed with pulmonary capillaritis (PC), a small-vessel vasculitis,
presenting as diffuse alveolar hemorrhage (DAH), and to compare these
findings with those for children with other alveolar hemorrhage
syndromes. STUDY DESIGN: A retrospective chart review of patients who
underwent a lung biopsy because of a clinical suggestion of pulmonary
hemorrhage. RESULTS: PC was identified in 8 of 23 patients. In these
patients, cough, crackles, and hypoxia were common. Alveolar
infiltrates on radiography and anemia were present in 7 of 8 cases.
Serologic evidence of a systemic vasculitis was present in 50% of
patients. High-dose corticosteroids proved effective in controlling
alveolar hemorrhage in all cases. There were no presenting signs or
symptoms that could differentiate patients with PC from those with
non-immune-mediated alveolar hemorrhage. In general, patients with PC
had a lower hematocrit and higher erythrocyte sedimentation rate (ESR).
CONCLUSION: Children presenting with lower respiratory tract symptoms,
chest x-ray abnormalities, and anemia should undergo evaluation for
PC, as early initiation of immunosuppression can be lifesaving and
organ sparing. No clinical signs to differentiate immune and
non-immune-mediated alveolar hemorrhage were evident in this study.
Pulmonary
capillaritis.Semin
Respir Crit Care Med. 2004
Oct;25(5):547-55.
Pulmonary
capillaritis is defined as a histopathologic pattern of alveolar wall
inflammation that leads to the disruption of the integrity of
alveolar-capillary basement membranes and flooding of the alveoli with
blood. The clinical presentation is that of diffuse alveolar
hemorrhage (DAH). Pulmonary capillaritis is usually the consequence of
an underlying immune-mediated process that is systemic in nature.
Rarely, pulmonary capillaritis occurs in isolation. This article
outlines a systematic approach to the management of patients
presenting with DAH, and provides an overview of specific causes of
pulmonary capillaritis.
Pulmonary
capillaritis.Curr
Opin Pulm Med. 2000 Sep;6(5):430-5.
Vasculitis,
inflammation, and necrosis of blood vessels can involve any size or
type of vessel in the pulmonary vasculature, including the
capillaries, so-called capillaritis. Although pulmonary capillaritis
is a histopathologic diagnosis that is not pathognomonic of a specific
disorder, it usually signals the presence of an underlying systemic
vasculitis or collagen vascular disease. Patients with pulmonary
capillaritis usually present with bilateral infiltrates on chest
radiographs and can be acutely ill with diffuse alveolar hemorrhage
that may be life threatening. Therapy depends on diagnosis of the
underlying disease that gave rise to the capillaritis. Since many of
the disorders leading to capillaritis are treated by immunosuppression
with corticosteroids and cyclophosphamide or azathioprine, infection
must be excluded early in the course of therapy.
Diffuse alveolar
hemorrhage with underlying isolated, pauciimmune pulmonary
capillaritis.Am
J Respir Crit Care Med. 1997 Mar;155(3):1101-9.
Diffuse
alveolar hemorrhage (DAH) resulting from pulmonary capillaritis
typically accompanies the systemic vasculitides and collagen vascular
diseases. Isolated pulmonary capillaritis and DAH without systemic
disease occurs in patients with antineutrophil cytoplasmic antibodies.
However, isolated pulmonary capillaritis and DAH is not described for
patients without clinical or serologic evidence for an underlying
systemic disease. To describe such patients, we undertook a
retrospective chart review of 29 patients with DAH and biopsy-proven
pulmonary capillaritis from seven Denver hospitals. Eight (28%) were
diagnosed with isolated pulmonary capillaritis without clinical,
serologic, or histologic evidence of an associated illness. Their
median age was 30 yr. No association with occupational or drug
exposures was identified. All had lower respiratory tract symptoms;
seven had upper respiratory tract symptoms. None demonstrated systemic
disease or evidence of glomerulonephritis. All were antineutrophil
cytoplasmic antibody negative. Other serologies were not significant
where measured. Direct immunofluorescence studies of lung tissue were
negative in five. Six presented with acute respiratory failure, four
requiring mechanical ventilation. One died during initial
hospitalization; seven survived. Median follow-up is 43 mo (7 to 73
mo). Five remain in remission. Two experienced multiple recurrences of
DAH but without development of systemic disease while on therapy.
Herein we characterize DAH and isolated pulmonary capillaritis in the
absence of clinical, serologic, or histologic evidence indicating an
accompanying systemic illness. The prognosis for this group appears
favorable.
Pulmonary
capillaritis and alveolar hemorrhage. Update on diagnosis and
management.Chest.
1996 Nov;110(5):1305-16.
Pulmonary vascular inflammatory disorders may involve all components
of the pulmonary vasculature, including capillaries. The principal
histopathologic features of pulmonary capillaritis include capillary
wall necrosis with infiltration by neutrophils, interstitial
erythrocytes, and/or hemosiderin, and interalveolar septal capillary
occlusion by fibrin thrombi. Immune complex deposition is variably
present. Patients often present clinically with diffuse alveolar
hemorrhage, which is characterized by dyspnea and hemoptysis; diffuse,
bilateral, alveolar infiltrates on chest radiograph; and anemia.
Pulmonary capillaritis has been reported with variable frequency and
severity as a manifestation of Wegener's granulomatosis, microscopic
polyarteritis, systemic lupus erythematosus, Goodpasture's syndrome,
idiopathic pulmonary renal syndrome, Behçet's syndrome,
Henoch-Schönlein purpura, IgA nephropathy, antiphospholipid syndrome,
progressive systemic sclerosis, and diphenylhydantoin use. In addition
to history, physical examination, and routine laboratory studies,
certain ancillary laboratory tests, such as antineutrophil cytoplasmic
antibodies, antinuclear antibodies, and antiglomerular basement
membrane antibodies, may help diagnose an underlying disease.
Diagnosis of pulmonary capillaritis can be made by fiberoptic
bronchoscopy with transbronchial biopsy, but thoracoscopic biopsy is
often employed. Since many disorders can result in pulmonary
capillaritis with diffuse alveolar hemorrhage, it is crucial for
clinicians and pathologists to work together when attempting to
identify an underlying disease. Therapy depends on the disorder that
gave rise to the pulmonary capillaritis and usually includes
corticosteroids and cyclophosphamide or azathioprine. Since most
diseases that result in pulmonary capillaritis are treated with
immunosuppression, infection must be excluded aggressively.
Pulmonary
capillaritis and hemorrhage in patients with systemic vasculitis.
Arch Pathol Lab Med. 1985
May;109(5):413-8.
Thirteen
patients with prominent pulmonary signs and symptoms had pulmonary
capillaritis and extensive hemorrhage demonstrated by open-lung biopsy
or autopsy. Vasculitis was demonstrated in other organs before or
after the lung biopsy or at autopsy. Although there were several
suspected causes for the pulmonary capillaritis and different final
clinicopathologic diagnoses, the histopathologic features in the lung
were similar in all cases and distinctive enough to separate
capillaritis from other causes of hemorrhagic lung. All patients were
treated with prednisone or cyclophosphamide, or both. Six patients
died of their vasculitis, five in respiratory failure and one in renal
failure. None of the seven survivors had a clinical recrudescence of
pulmonary hemorrhage. By extrapolation from these 13 cases, one may
histopathologically recognize pulmonary capillaritis when it causes
hemorrhagic lung in a patient without clinically evident
extrapulmonary vasculitis. One can then proceed to investigate the
patient and possibly determine the nature of the vasculitis.
Pulmonary
capillaritis. The association with progressive irreversible airflow
limitation and hyperinflation.Am
Rev Respir Dis. 1993 Aug; 148(2) :
507-11.
We report two
patients with systemic necrotizing vasculitis (microscopic
polyarteritis) and associated recurrent pulmonary capillaritis, in
whom progressive irreversible airway dysfunction began approximately
10 yr after disease onset. Their course was characterized by repeated
episodes of diffuse alveolar hemorrhage, glomerulonephritis, palpable
purpura, and splinter hemorrhages. The lung revealed intraalveolar
hemorrhage, neutrophilic infiltration and cellular fragmentation,
fibrinoid necrosis of the alveolar interstitium, and parenchymal
hemosiderin deposits. No medium-sized vessel involvement,
granulomatous inflammation, or bronchiolar obliteration were seen.
Renal biopsies revealed focal segmental necrotizing glomerulonephritis,
and a cutaneous biopsy in one case showed a leukocytoclastic
vasculitis. Immunofluorescent studies of lung and kidney showed
minimal or no immunoreactivity. The clinical course and serologic
tests did not support another systemic vasculitis, connective tissue
disease, or antiglomerular basement membrane antibody disease. The
acute episodes responded to antiinflammatory and immunosuppressive
therapy. Symptoms, serial pulmonary function tests, and chest imaging
documented the development of a progressive irreversible obstructive
airway disease. No other predisposing factors were identified. These
cases demonstrate the unexpected appearance of an irreversible
obstructive airway disease with lung parenchymal hyperinflation after
systemic necrotizing vasculitis associated with recurrent pulmonary
capillaritis and diffuse alveolar hemorrhage.
Antineutrophil
cytoplasmic autoantibody-associated alveolar capillaritis in patients
presenting with pulmonary hemorrhage.Arch
Pathol Lab Med. 1994 May;118(5):517-22.
The
objective of this study was to determine the significance of the
antineutrophil cytoplasmic autoantibodies (ANCAs) from the
clinicopathologic viewpoint of pulmonary hemorrhage occurring as a
prominent event of disease. Forty-three consecutive patients with both
pulmonary hemorrhage as a prominent clinical manifestation and a
positive test for antineutrophil cytoplasmic autoantibodies were
studied. Thirty-six patients underwent open lung biopsy, including
histologic, tissue immunofluorescence, and microbiologic studies.
Immunoassays were performed to investigate the antigenic specificities
of antineutrophil cytoplasmic autoantibodies in the patients studied.
All patients with lung biopsy confirmation had pauci-immune
hemorrhagic alveolar capillaritis as the main morphologic substrate.
In addition, renal involvement in the form of pauci-immune crescentic
glomerulonephritis was a common finding. Serum samples from the 43
study patients contained antibodies that were monospecific for
proteinase 3 (n = 13) or myeloperoxidase (n = 30). In our study,
whereas anti-proteinase 3 antibodies were mainly detected in patients
with alveolar capillaritis and a well-established diagnosis of
Wegener's granulomatosis, antimyeloperoxidase antibodies were
principally found in those patients who had alveolar capillaritis and
polyarteritis nodosa not only as a primary finding but also
accompanying other diseases. However, a significant number of patients
with alveolar capillaritis and antimyeloperoxidase antibodies showed
no evidence of polyarteritis nodosa (idiopathic pulmonary-renal
syndrome and isolated forms of pulmonary hemorrhage). We conclude that
in patients presenting with pulmonary hemorrhage as a prominent event
of disease, antineutrophil cytoplasmic autoantibodies are a new clue
strongly supportive of a pulmonary capillary vasculitis, irrespective
of the primary underlying disease. Moreover, the antigenic subtype of
antineutrophil cytoplasmic autoantibodies helps in recognizing the
type of vasculitic disorder involved.
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