| Table 2: Case reports and case series of vasculitis in CF |
|
|
Author
|
No
of Cases
|
Age
(Years)*
|
Site
|
No
of Deaths**
|
Years
to Death
|
Laboratory
Results
|
Sputum
Microbiology
|
|
Nielsen
et al (10)
|
4
|
17
(14-20)
|
Cutaneous Arthralgia |
3
|
0.8
(0.4-1.5)
|
Raised IgA, IgG
|
P
(4), S (4), H (4)
|
|
Soter
et al (23)
|
2
|
18
(16-20)
|
Cutaneous
|
2
|
1.3
(0.6-2)
|
Raised IgA, IgM, IgG Necrotizing Venulitis |
P (2), S (2), H (1)
|
|
John
et al (36)
|
2
|
16
|
Cutaneous
|
-
|
1.5
|
Raised
IgA, IgM, IgG & immunocomplexes
|
|
|
Summers
et al (35)
|
1
|
19
|
Cutaneous
|
None
|
Raised immunocomplexes
|
S
|
|
|
Fradin
et al (42)
|
1
|
18
|
Cutaneous Arthralgia |
Leukocytoclastic
Vasculitis
|
P
|
||
|
Bourke
et al (7)
|
1
|
21
|
Cutaneous Arthralgia |
None
|
0.1
(0.1-0.2)
|
Raised
immunocomplexes
|
P
|
|
Finnegan
et al (6)
|
12
|
22
(12-32)
|
Cutaneous (12), CNS (1), Renal (1) |
5
|
Raised IgA, IgG. ANCA positive |
S
(5), H (4), P (7)
|
|
|
Garty
et al (27)
|
1
|
13
|
Cutaneous
|
none
|
Raised immunocomplexes & Cryoglobulins |
P
|
|
|
Schidlow
(9)
|
16
|
20
(9-33)
|
Cutaneous
|
13
|
1.3
(0.2-4.9)
|
Raised IgA, IgG,IgM |
|
|
Parameswaran (37)
|
1
|
20
|
Gastrointestinal
|
none
|
Reduced Hb Raised IgA, IgG |
S, H
|
|
|
Total
|
41
|
Mean
Age 20 years
|
23
|
Mean
1 year
|
Abbreviations: - * = Data
expressed as mean (range), ** = Number of patients who had died at the time
of reporting. In the majority of patients the death was not directly related
to vasculitis. P = Pseudomonas aeruginosa, H = Haemophilus influenzae, S = Staphylococcus
aureus.
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