Pulmonary Hemorrhage in Henoch-Schönlein Purpura: Case Report and Systematic Review of The English Literature
Pulmonary Hemorrhage in Henoch-Schönlein Purpura: Case Report and Systematic Review of The English Literature
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Table 1 Continued
References No. (If Series)
Age/
Sex
Clinical Features
Kidney
biopsy IF
Diagnosis of DAH
Time to
Lung
Involvement
Treatment
Prior to DAH
Regimen After DAH
Outcome Skin
Arthritis/
Arthralgia
NephritisCr
(mg/dl) GI Others
Drop
in
Hb Hemoptysis Radiology BALF Others MP CYC
Yokose et
al.
1 77/M Hematuria;
RF; Cr
6.5
No Fibrinoid
necrosis?
MPN
MPGN
IgA
C3
Alveolar; large
PE
NA Skin Bx LCV IgA,
autopsy lung
LCV, DAH IgA
2 d None Pulse MP 30
mg/kg/d
x? doses
None Died at 51 d
Wright et
al.
1 14/F Hematuria;
RF; Cr
2.2
Melena Lung biopsy
LCV, DAH
IgM,
C3,
IgA
DPGN;
IgA,
IgM,
C3
Alveolar NA Skin Bx LCV; IgA 3 wk Pulse MP Repeat pulse
MP 30
mg/kg/d
3
doses,
then oral
2 mg/kg/
d
None Alive 7 mo;
duodenal
ulcer
Mallat et
al.
1 29/M Nephrotic
syndrome;
ESRD on
dialysis
No Diagnosed
HSP 11 yr;
recurrent
bouts 4
No Reticulonodular Yes IgA C3 skin Bx
LCV of normal
skin
11 yr None Pulse MP
3 doses
None Alive no
recurrence;
ESRD
Reznik et
al.
1 14/F RF; CR NA Pain No MPGN;
IgA,
C3
Alveolar NA Lung biopsy LCV,
DAH; IgA NA
1 wk Recurrence
of DAH 3
yr later;
none
Pulse MP
? doses
Pulse CYC? dose,
PE? sessions
Alive; normal
Carter et
al.
1 15/M Nephritis;
Cr 1.3
Pain/
blood
None;
intercostal
tube
drainage
No Alveolar;
massive
transudative
PE
NA No skin, renal, or
lung biopsy done
1 wk None Pulse MP
500 mg/
d 4,
then 300
mg
4 d, then
160 mg
1 d
None Alive 18 mo;
recurrence
of PH
References No. (If Series)
Age/
Sex
Clinical Features
Kidney Biopsy
IF
Diagnosis of DAH
Time to
Lung
Involvement
Treatment
Prior to DAH
Regimen After DAH
Outcome Skin Arthritis Nephritis GI Others
Drop
in
Hb Hemoptysis Radiology BALF Others MP CYC
Paller et
al.
2 12/M Hematuria;
RF; Cr
1.7
No Epistaxis;
stroke,
seizures
CGN; IgA _ Alveolar NA Skin Bx LCV
IgA
1.5 wk Prednisolone
2 mg/kg/
d
Prednisolone
2 mg/kg/
d
Pulse CYC 750
mg/m
2
, then 3
mo
Alive at 2 yr;
CYC
anaphylaxis
0.4/F Hematuria;
Cr NA
No None NA Alveolar NA Skin Bx LCV IgA
NA; autopsy
lung Bx
DAH; no
LCV
64 wk None None None Multiple
recurrences;
died
Vats et al. 1 7/M Not at
onset;
day
4
Hematuria;
Cr 0.5
Colicky
pain;
bleed
Sinus
involvement;
surgery
MPGNIgA,
C3
Alveolar;
pleural
effusions
NA Skin Bx: IgA-
LCV; sinus,
jejunum
LCV-
1 wk None Pulse MP 30
mg/kg/d
3
doses,
then 2
mg/kg/d
None Alive;
hematuria
6 wk
Besbas et
al.
1/550 (0.18%) 6/M Hematuria;
RF Cr 2.8
No None DPGN; IgA,
C3
Alveolar NA None 1 wk Pulse MP 30
mg/kg/d
3
doses
Pulse MP 15
mg/kg/d
2, then
2 mg/kg/
d
Pulse CYC 250
mg/d, then
oral 2 mg/kg/d
? months
Alive 18
mo,
neutropenia
Al-Harbi et
al.
1 9/F Nephrotic
syndrome;
RF Cr 5.4
Pain;
bleed
None No biopsy Alveolar NA None 1.5 wk Prednisolone
30 mg/d
Pulse MP 30
mg/kg/d
3
doses,
then oral
6 mo
Worsening on MP;
CYC pulse 750
mg/m
2
, then
750 mg/m
2
6
Alive at 1 yr
Teixeira et
al.
1 45/M Nephritis; Cr
1.0
Pain Occult alveolar
hemorrhage;
previous
pulmonary
tuberculosis
FPGN IgA, C3
mesangium
Normal Yes Skin Bx normal;
no lung Bx
3 mo Occult DAH;
none
Prednisone 1
mg/kg/d
None Alive at 1 yr
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Table 1 Continued
References No. (If Series)
Age/
Sex
Clinical Features
Kidney Biopsy
IF
Diagnosis of DAH
Time to
Lung
Involvement
Treatment
Prior to DAH
Regimen After DAH
Outcome Skin Arthritis Nephritis GI Others
Drop
in
Hb Hemoptysis Radiology BALF Others MP CYC
Nadrous
et al.
2/124 (1.6%) 20/M RF Cr 2.0 No None Not done Alveolar NA Skin Bx: IgA
deposition
2 wk Parenteral
prednisolone?
dose
Pulse MP
500 mg/
d 2;
then 60
mg/d 10
d
None Alive
76/F RF Cr 1.2 Pain Systemic
recurrences
multiple;
2nd
episode of
DAH
Not done Alveolar NA Skin Bx: IgA
deposition
18 yr One prior
DAH on
3 mg/d
prednisone
Parenteral
prednisolone
60
mg/d
2; then
30 mg/d
None Recurrence of
PH; alive
Kalyoncu
et al.
1 13/M Not at
onset;
nephritis
later Cr
3.6
Severe
pain
Rheumatic (?)
carditis 6-yr
autopsy,
DAH IgA-
brinoid
necrosis
Not done Alveolar
opacities
NA Skin biopsy LCV
IgA C3
autopsy
lungs LCV;
IgA NA
3 Prednisolone
2 mg/kg
Pulse MP 30
mg/kg/d
CYC 2 mg/kg/d
26 d
Died at 4 wk;
massive
PH
References
No./
Series
Age/
Sex
Clinical Features
Kidney
Biopsy IF
Diagnosis of DAH Time to
Lung
Involvement
(wk)
Treatment
Prior to DAH
Regimen After DAH
Outcome Skin
Arthritis
Arthralgia
Nephritis Cr
mg/dl GI Others HbDrop Hemoptysis Radiology BALF Others MP CYC
Tiryaki et al. 1 78/F Hematuria,
proteinuria
Cr 0.7
No None MPGN IgA
NA
Alveolar
opacities
Yes Skin biopsy:
LCV Ig A
Na
12 Pulse MP
?Doses,
then 48
mg/d
Pulse MP
1.5 g
3, then 1
mg/kg/d
3 mo
None Alive; tapered
3 mo
Usui et al. 1 69/M ESRD; Cr
8.73
Gastroenteropathy ESRD 18 yr Hyalinized
glomeruli;
IgA NA
GGO/
consolidation
NA IgA C3 skin
biopsy;
autopsy-
LCV, DAH
IgA
2 100 mg
prednisolone
Pulse MP
3, then
125 mg/
d;
recurrence
26th day
MP 3
None Died at 5 wk
Matsubayashi
et al.
1 6/M Hematuria;
Cr 0.3
Protein-losing
enteropathy,
colonic blebs,
hemorrhage
None MPGNIg A GGO/
consolidation
NA No 2.5 Prednisolone 2
mg/kg/d
Pulse MP
?doses
Cyclosporine
A;
abdominal
recurrence
MP 2
Mizoribine?
duration
Renal
recurrence;
alive-
mizoribine
started
Touissant et
al.
1 54/M Nephrotic
syndrome;
Cr 3.9
Intestinal
obstruction
Trans-bronchial
Bx LCV;
alveolar
hemorrhage
IgA NA
FPGN with
mesangial
IgA, C3
Alveolar
opacities;
GGO on CT
Yes; also
on
biopsy
Skin Bx: LCV
IgA NA
3 Parenteral MP Pulse MP
3
CYC 2 mg/
kg/d 4
mo; MMF
later
Alive; dialysis
independent
at 3 yr
Oto et al. 1 33/F No No Kabuki
syndrome
No biopsy GGO/
consolidation
NA Skin biopsy
LVC IgA
1.5 None Prednisolone
20 mg
None Alive at 2 mo
Goel et al. 1 72/M Nephritis; Cr
1.6
No Multiple
comorbidities
MPGN Ig
A
GGO/nodular
inltrates
Yes IgA renal/
skin
biopsy; no
lung
biopsy
2 Prednisolone
40 mg/d
PE-1 session;
pulse MP
?doses
CYC 175 mg/
d 3 mo;
AZA until
6 mo
Alive at 6 mo;
Cr 1.6 mg/
dl
Nagasaka et
al.
1 74/M Hematuria;
Cr 2.4
Duodenal ulcers Intracranial
bleed; MPO-
ANCA
CGN; IgA,
C3
Consolidation/
pleural
effusion
No IgA skin
biopsy
autopsy:
DAH, no
LCV
8 Pulse MP 3 None None Died 4 wk
later
The numerator is the number of cases in males and the denominator is the total number of cases reported.
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tion (3,5,6,10-13,15-20,25,28,29) for a mean duration
of 26 4.8 days (n 10). Continuation of oral steroids,
often at higher doses, was associated with a mortality rate
of 27. 2% (n 3/11) (3,5,6,11). Pulse methylpred-
nisolone, at a varying number of boluses, was adminis-
tered after the occurrence of DAH in 11 patients (mor-
tality 27.2%, n 3/11). Pulse methylprednisolone along
with cyclophosphamide (11,17,19,20,23), pulse cyclo-
phosphamide followed by azathioprine (7,8,29), or my-
cophenolate mofetil (27) and cyclosporine A (26) were
administered in 11 patients and had the least mortality
rate observed (9%, n 1/11). Untreated DAH had a
uniformly fatal outcome (n 3/3, 100%) (6,17,30). Ste-
roids and immunosuppression were administered for a
median duration of 9 months (range, 10 days to 36
months) and 4.5 months (2.5 to 36 months), respectively.
The total duration of reported follow-up (mean, SE)
was 1.6 (0.5) months (n 19). Recurrence of vasculitis
was seen in 27.7%, including 4 patients (11.1%) with
recurrent DAH (7,16,22,25). Morbidity due to DAH
was also common and persistent urinary abnormalities
(8,9,11,18) (12%), chronic renal failure (9%) (14,25),
Table 2 Summary Findings of All Reported Cases of Henoch-Schnlein Purpura with Diffuse Alveolar Hemorrhage in the
English Literature
Parameter Value
Age (median, range) 16.5 (4 mo to 78 yr), N 32
Kidney biopsy (50%, N 18/36) MPGN (44.4%, N 8/18), FSGN (16.7%, N 3/18), DPGN (5.5%,
N 1/18), CGN (27.8%, N 5/18)
Hyalinized glomeruli (5.5%, N 1/18)
Kidney biopsy immunouorescence
(44.4%, N 16/36)
IgA, C3 positive (93.75%, N 15/16), IgA, C3 negative (6.3%,
N 1/16)
Time to lung involvement from onset
of illness (mean, range)
3 wk (2 d to 18 yr) (N 29)
Diagnosis of diffuse alveolar
hemorrhage
Drop in hemoglobin (74%, N 27), hemoptysis (75%, N 36), chest
radiology nding (94.4%, N 36), bronchoalveolar lavage (16.7%,
N 36)
Chest radiology ndings Consolidation/ground glass opacity (80.6%, N 36)
Reticulonodular opacity (13.9%, N 36)
Normal (5.6%, N 36)
Pleural effusions (16.7%, N 36)
Leukocytoclastic vasculitis (LCV) on
lung biopsy
69.2% (N 9/13); DAH alone 31.8% (N 4/13)
Immunoglobulin A positive on lung
biopsy
Surgical or autopsy (N 6/13); 50% (N 3/6)
Treatment before DAH None 38.9% (N 14/36)
Oral steroids 50% (N 18/36)
Pulse methylprednisolone 11% (N 4/36)
Treatment after onset of DAH None 8.3% (N 3/36)
Oral steroids alone 30.5% (N 11/36)
Methylprednisolone pulse alone 30.5% (N 11/36)
Steroids and immunosuppression 30.5% (N 11/36)
Mechanical ventilation 50% (N 36)
Duration of mechanical ventilation
(mean, SE)
26 4.8 d (N 10)
Duration of steroid therapy median,
range
9 mo (10 d to 36 mo, N 11)
Duration of immunosuppression
(median, range)
4.5 mo (2.5-36 mo, N 6)
Recurrences of vasculitis 27.7%
(N 10)
None 72.2% (N 36), all systemic 19.4% (N 7/36), PH alone
11.1% (N 4/36)
Complications of
immunosuppression 27% (N 11)
Zoster 9% (N 11), neutropenia 9% (N 11), anaphylaxis 9%
(N 11)
Mortality 27.8% (N 10/36)
Morbidity Persistent hematuria, albuminuria 12% (N 33), chronic renal failure
9% (N 33)
M, Male; F, Female; NA, not available; , present; absent; Cr, creatinine; DAH, diffuse alveolar hemorrhage; GN, glomerulonephritis;
MPGN, Mesangioproliferative glomerulonephritis; FSGN, Focal segmental glomerulonephritis; CGN, Cresentric glomerulonephritis; DPGN,
diffuse proliferative glomerulonephritis; PE, pleural effusions; R, right-sided; Bx, biopsy; LCV, leukocytoclastic vasculitis; IgA, Immunoglobulin
A; C3, complement 3; CYC, cyclophosphamide; AZA, azathioprine; MP, methylprednisolone; MV, mechanical ventilation; ESRD, end stage
renal disease; MPN, microscopic polyangiitis; PH, pulmonary hemorrhage.
S. Rajagopala et al. 397
and complications of therapy (27%) (11,13,17,19) were
frequently observed.
DISCUSSION
HSP is an acute, self-limited small-vessel vasculitis char-
acterized by deposition of immunoglobulin A (IgA) im-
mune complexes (55,56). It is predominantly a disease of
children, with the highest prevalence between 4 and 7
years (70 per 100,000) (55).
Age at onset and nephritis at presentation in HSP are
important determinants of severity (57,58). The older age
of this cohort and high prevalence of nephritis suggest
DAH occurs in patients at risk of recurrence of systemic
vasculitis.
DAH is exceedingly rare in HSP and large series have
often not reported any cases of DAH (52,53). Data from
the retrospective studies of Cream et al. (4) and Nadrous
et al. (22) have suggested a prevalence of 1.6% to 5% for
overt alveolar hemorrhage. Differences in reported prev-
alence are probably due to case selection bias.
The high frequency of clinically evident alveolar hem-
orrhage and the use of bronchoscopy in only 16.7% of
patients with DAH related to HSP suggest late clinical
recognition. In alveolar hemorrhage, clinically evident he-
moptysis is present in only 33% of patients at presenta-
tion because the total alveolar volume is large and can
accommodate bleeding without apparent symptoms;
early bronchoscopy is recommended for diagnosis (59).
Recurrent unrecognized DAH may be associated with
reticular inltrates mimicking an interstitial lung disease
and was observed in 13.9% of patients (11,22). Subclin-
ical reductions of transfer factor of the lung for carbon
monoxide (TLCO) with active vasculitis (60), the associ-
ation of reduced TLCO with systemic relapses (60,61),
and the nding of hemosiderin-laden macrophages in the
absence of pulmonary symptoms and normal radiographs
(21) suggest that incipient alveolar hemorrhage may be
more common than is clinically evident. The prognostic
implication of subclinical alveolar hemorrhage, however,
remains unclear.
Corticosteroids are not recommended in patients with
uncomplicated HSP (62,63); however, in patients with
nephritis, pulse methylprednisolone followed by oral cor-
ticosteroids plus azathioprine or cyclophosphamide is ef-
fective in reversing clinical nephritis and preventing dis-
ease progression (55,64). Patients with DAH secondary
to HSP capillaritis are treated with a similar regimen (59).
Given the severity of DAH, it is unlikely that signicant
delays existed after the diagnosis of DAH was made.
However, delays of 2 to 3 weeks after onset of DAH
(9,28) to the administration of steroids were seen, sug-
gesting that the recognition of DAH was delayed. Most
patients were on oral steroids for systemic vasculitis before
the onset of DAH, indicating that steroids alone are inef-
fective. At least 11% of patients had already received 3
doses of pulse methylprednisolone just before the onset of
DAH for systemic vasculitis. Also, patients had a recur-
rence of DAH with pulse methylprednisolone alone (25)
and responded to cyclophosphamide pulse after failing
pulse methylprednisolone for DAH (20). In patients on
steroids at the time of DAH, pulse methylprednisolone
followed by cyclophosphamide for a median duration of 6
months was the most commonly reported treatment reg-
imen and was associated with a mortality rate of 9% (n
1/11). A high frequency of vasculitis recurrence (27.7%)
and morbidity (27%) was seen.
DAHis a rare complication of HSP and often occurs in
adults. Clinical recognition is delayed, with high morbid-
ity and mortality. DAH is frequently severe, requiring
mechanical ventilation. Pulse methylprednisolone and
cyclophosphamide are associated with the least mortality,
particularly in patients on steroids at the time of DAH.
Further multicentric observational studies are required to
dene the optimal regimen and duration of treatment in
DAH related to HSP.
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