Characteristics and outcomes of patients with Goodpasture's syndrome.

ABSTRACT

Background. Goodpasture's syndrome is a rare disorder characterized by pulmonary hemorrhage, glomerulonephritis, and antiglomerular basement membrane antibodies.

Methods. Case reports of Goodpasture's syndrome between 1993 and 2000 were identified using MEDLINE. Each case was reviewed for clinical manifestations, laboratory features, pathophysiology, treatment options, and outcomes.

Results. Eighty-five cases of Goodpasture's syndrome were reported in the English language literature. The mean age of patients in this review was 44 years. Most patients had multiple risk factors. Smoking was strongly associated with pulmonary hemorrhage. Most patients presented with hemoptysis and crackles. More than 90% had antiglomerular basement membrane antibodies, and 80% had crescenteric glomerulonephritis on renal biopsy.

Conclusion. Most patients were treated with immunosuppression and plasma exchange and were alive at follow-up. Patients with either pulmonary or renal signs and symptoms should be evaluated for Goodpasture's syndrome, since early diagnosis allows prompt treatment and improved outcome.

**********

THE FIRST reported case of Goodpasture's syndrome was in an 18-year-old male patient with hemoptysis and renal failure during the influenza pandemic of 1919. (1) However, at autopsy, the patient was found to have vasculitis. In 1958, Stanton and Tange (2) applied the term "Goodpasture's syndrome" to patients with pulmonary hemorrhage and glomerulonephritis. It was not until 1967 that the autoimmune nature of Goodpasture's syndrome was postulated. This occurred with the discovery of antiglomerular basement membrane antibodies. (3)

Numerous disorders affect both the lungs and kidneys and are collectively referred to as pulmonary-renal syndromes. However, each syndrome is clinically distinct. Goodpasture's syndrome is a classic example of a pulmonaryrenal syndrome. Goodpasture's syndrome refers to the triad of pulmonary hemorrhage, glomerulonephritis, and antiglomerular basement membrane antibodies. Goodpasture's disease is used to refer to glomerulonephritis and antiglomerular basement membrane antibodies in the absence of pulmonary hemorrhage. The term antiglomerular basement membrane antibody disease describes patients with serum antibodies against the basement membrane and encompasses both Goodpasture's syndrome and Goodpasture's disease. (4)

Although Goodpasture's syndrome is an unusual disorder, it has been associated with significant morbidity and mortality. Identification of its autoimmune nature and the use of immunosuppresive agents and plasma exchange have improved outcomes. In addition to reviewing the outcomes of all the cases reported over an 8-year period, we review the clinical features, diagnostic features, pathophysiology, treatment options, and prognostic factors described for Goodpasture's syndrome.

MATERIALS AND METHODS

We searched MEDLINE using the keywords Goodpasture's syndrome, Goodpasture's, case report, and antiglomerular basement membrane disease in various combinations. Articles were reviewed to identify validity of Goodpasture's syndrome. To ensure all the reported cases were found, the references in the articles acquired that were not found in the MEDLINE search were further investigated. After the review process, a total of 85 cases were identified in which Goodpasture's syndrome was present. The information regarding these cases is summarized in Table 1. (5,61)

RESULTS AND DISCUSSION

Epidemiology

Goodpasture's syndrome is a rare disease. The annual incidence of disease is unknown, but it has been proposed that it represents 1% to 2% of all cases of glomerulonephritis. (62) Studies have reported a male predominance ranging from 2:1 to 9:1. (63,64) Our review showed a male-female ratio of 3:2 (male 60%, female 40%) (Table 1). There is a bimodal distribution with respect to age, with peaks at 20 to 30 and 60 to 70 years of age. (65,66) Similarly, 53% of patients in this review were more than 40 years old. The mean age in this review was 44 years, the youngest patient being 9 years old and the oldest being 79. This is similar to mean ages reported in previous Studies. (67,68) Goodpasture's syndrome has been reported in all racial groups, but it remains primarily a disease of white populations. (16) Our results confirm this statistic; 83% of cases in which race was identified occured in whites.

Pat ho genesis

Goodpasture's syndrome is an autoimmune disorder in which there are autoantibodies against the glomerular and alveolar basement membranes. The main component of these basement membranes is type IV collagen, which is composed of three [alpha] chains that form a triple helix. (69) The Goodpasture antigen has been localized to the [alpha]3 chain of type IV collagen.70 The antigen is concealed within the tertiary structure of the protein so antibodies have a difficult time binding to the site. It is thought that damage to either alveolar or glomerular tissue results from a triggering event or agent that facilitates binding of the autoantibody to the Goodpasture antigen. Studies have implicated smoking, upper respiratory tract infections, genetic predisposition, and hydrocarbon exposure as potential triggers. (62,64,71,74)

Etiology

What causes production of autoantibodies in patients with Goodpasture's syndrome remains unknown, but there are many hypotheses. One theory is injury to the kidney or lung by hydrocarbon exposure. Bombassei and Kaplan (75) state that hydrocarbon exposure is a prominent risk factor for triggering the initial event. They reviewed 31 cases with documented exposure history and Goodpasture's syndrome. The types of hydrocarbons these patients were exposed to included cleaning solvents, insecticides, industrial solvents, diesel fumes, hard metals, and chlorine gas. Our study indicated either documented exposure or possible hydrocarbon exposure in approximately 32% of cases; however, hydrocarbon exposure alone was found in only 8% of cases. Other studies report that hydrocarbon exposure is found in less than 5% of cases. (16) Hydrocarbon exposure may not be a major factor in the development of Goodpasture's syndrome but may play a role in certain predisposed individuals.

Another proposed triggering event is an upper respiratory tract illness or a flu-like illness. (63,64,71) Goodpasture's syndrome was initially described during the pandemic of influenza. (1) Our study showed that 32% of the patients had an upper respiratory tract illness before their disease, whereas in 5% of cases it was the only risk factor. Previous studies have shown up to 60% of patients had an upper respiratory tract illness before presentation. (63,64,71)

Another association with Goodpasture's syndrome is tobacco use. Smoking may also increase the likelihood of pulmonary hemorrhage in patients with Goodpasture's syndrome. (72) Donaghy and Rees (72) reported pulmonary hemorrhage in 41 of 51 patients, 37 of whom were known smokers. All smokers had pulmonary hemorrhage, while only 2 of the 10 nonsmokers had pulmonary hemorrhage. (72) Other studies have validated these results. (62) Our study showed 72% of patients were smokers or had a significant smoking history. In 42% of the cases, it was the only risk factor. Furthermore, 75% of the patients who were smokers had pulmonary hemorrhage, confirming the findings of Donaghy and Rees. (72)

Finally, studies in identical twins, siblings, and cousins have identified a genetic link to Goodpasture's syndrome. (73,74) A link to the histo-compatibility complex HLA-DR2 has been described by Rees et al, (76) who found two cases with this complex; however testing was not done in a majority of the cases reported in the literature. It is postulated that a triggering event causes exposure of the antigen and that antibody production occurs only in genetically predisposed individuals. Once the trigger is removed or tolerance develops, antibody production ceases. (16)

Clinical Manifestations

Hemoptysis is the most common presenting symptom. Hemoptysis occurs in more than 80 of patients, and almost all patients with Goodpasture's syndrome will have some degree of hemoptysis over the course of the disease. (63,64,77) In more than 50% of patients, it precedes glomerulonephritis. (77) Other common symptoms are cough, dyspnea on exertion, fatigue, and weakness. Chills, fever, sweats, weight loss, gross hematuria, chest pain, and history of upper respiratory tract infection may also be present. (16) In our study, 65% of patients had hemoptysis, 19% had chest pain, 28% had shortness of breath, 26% had dyspnea on exertion, 28% had cough, 17% had lower extremity edema, and 28% had fever (Table 2). More than 75% of patients had pulmonary hemorrhage (Table 1).

The most common finding on physical examination is pallor, which often correlates with the degree of anemia. (63,64) Crackles and rhonchi are often heard on chest examination. Other physical signs are a heart murmur, hepatomegaly, edema, hypertension, visual changes, and a skin rash. (16) Crackles were most frequent (46%), followed by lower extremity edema (32%). Tachycardia and tachypnea (26%) were also present. Ten patients (29%) had normal results of physical examination (Table 2).

Antiglomerular Basement Membrane Antibody

The diagnosis of Goodpasture's syndrome is made by the finding of antiglomerular basement membrane (anti-GBM) antibodies, either circulating in the blood or bound to tissue. A variety of methods are used to identify these antibodies. Previously, an indirect immunofluorescence technique was used, but this has been replaced by the more sensitive radio-immunoassay and enzyme-linked immunosorbent assay. (62) Our study indicated that 92% of patients had circulating anti-GBM in serum, usually at high titers (Table 1). In two cases, the circulating anti-GBM was IgA and not IgG. (53,55)

If the diagnosis is uncertain, a histologic sample can be probed for autoantibodies. The kidney is the most common biopsy site. The sample is examined for tissue bound antiGBM antibodies. Lung biopsy is not as accurate as kidney biopsy for making a definitive diagnosis, though seven patients did have positive lung biopsies. Under light microscopy, renal biopsy samples usually show crescentic glomerulonephritis. (16) Immunofluorescence reveals linear staining with IgG in the glomerulus. In our study, 80% of patients had crescentic glomerulonephritis by light microscopy. Forty patients had linear deposition of antibodies (generally IgG), 4 patients had IgA, and 16 had C3 deposition (Table 1).

Laboratory Data and Radiologic Manifestations

The most common laboratory finding is anemia, (16) which is considered to be a result of blood loss from pulmonary hemorrhage with secondary iron deficiency. A hemoglobin value <12 g/dL was reported in 90% to 100% of cases. (16) Benoit et al (63) reported a mean hemoglobin value of 7.5 g/dL. In our study, the mean value was 9.0 g/dL. Other common laboratory findings are increased blood urea nitrogen (BUN) and creatinine from renal dysfunction. The mean BUN in our patients was 74.3 mg/dL, and the mean creatinine was 6.62 mg/dL (Table 3). Urinalysis in patients with Goodpasture's syndrome indicates proteinuria, microscopic or gross hematuria, and red blood cell casts. (16) Urinalysis in our patients showed that 15% of them had proteinuria, hematuria, and granular casts, 59% had proteinuria and hematuria, 8% had hematuria alone, and 10% had normal findings. Table 3 shows common laboratory abnormalities found in patients with Goodpasture's syndrome. Despite the frequency of abnormal function, patients may present with normal renal function or pulmonary function, or both. (8, 78)

Chest radiographs generally reveal diffuse alveolar filling and diffuse opacities. (4) The presence of fluffy hilar and basilar infiltrates is thought to be indicative of pulmonary hemorrhage. (16) However, radiographs are nonspecific and patients may have other superimposed pulmonary processes including pnemonia, atelectasis, and pulmonary edema. More than 75% of the patients in our review had radiographic changes, the most common being interstitial or alveolar infiltrates, whereas about 25% had normal findings. The use and value of computed tomography and magnetic resonance imaging with Goodpasture's syndrome remain unknown. (16)

Treatment and Prognosis

There are three goals in the treatment of Goodpasture's syndrome. The first is to remove circulating autoantibodies, which is accomplished by plasmapheresis. The second is to stop the production of the anti-GBM antibodies using immunosuppressive medications. The final goal is to remove the triggering event. (4) In this review, a majority of patients were treated with steroids, cyclophosphamide, and plasmapheresis (39%), while 12% of patients were treated with hemodialysis. Seven patients were treated with only steroids and cyclophosphamide (Table 1).

Historically, the prognosis of Goodpasture's syndrome was poor. Patients died of uncontrollable pulmonary hemorrhage or renal failure. (63, 64) Recognition of the autoimmune aspect and the use of immunosupressive agents and plasma exchange have improved the prognosis. One study on Goodpasture's syndrome indicated that after a short follow-up 78% of patients were alive, and 57% were receiving maintenance dialysis. (16) Levy et al (79) showed a 5-year survival of 63% to 94%, depending on the initial renal function. Our study showed that 20% of patients had normal recovery, 39% were receiving maintenance hemodialysis, and 12% received transplants and were doing well. In addition, 5% were awaiting transplants, and 24% of patients died. Numerous studies have shown poor outcome in patients with initial creatinine levels greater than 5 mg/dL, crescent formation in more than 50% of glomeruli, oligoanuria, and circulating anti-GBM. (65, 68) Early treatment affords better results. (80) Although recurrence of disease ha s been reported, it occurs in a minority of patients (33, 81)

CONCLUSION

Goodpasture's syndrome is a rare autoimmune disease in which patients have autoantibodies directed against their lung and kidney basement membranes and exhibit pulmonary hemorrhage and glomerulonephritis. The triggering event for development of these autoantibodies remains unknown, but smoking seems to play a role in the development of the pulmonary hemorrhage in this disorder. There may be a combination of triggering events (such as upper respiratory tract illness, hydrocarbon exposure, and smoking) that lead to the development of autoantibodies. Furthermore, the autoantibodies may occur only in patients genetically predisposed to the disease. Regardless, early recognition of Goodpasture's syndrome allows prompt treatment and improves survival. Most patients are treated with immunosuppressive agents and plasmapheresis. Most patients live, but they have renal dysfunction and require maintenance on hemodialysis. It is important to consider the diagnosis of Goodpasture's syndrome in patients who present with pu lmonary or renal disease, since it is often misdiagnosed and early treatment affords the best chance for recovery. To target treatment and further improve outcome, additional research is needed to elucidate the exact mechanism of disease.

Table 1

Case Reports of Goodpasture's Syndrome Between 1993 and 2000

Reference,  Race       Pulmonary                           Anti-GBM
  Year      Sex   Age  Hemorrhage     Type GN               (Serum)

5, 1993     x, M  47   x           x/linear, IgG, IgA         Yes
6, 1993     x, M  59   x           Cr/linear, IgG             Yes
6, 1993     x, F  17   No          Cr/linear, IgG             Yes
7, 1993     W, M  25   x           x                           x
8, 1993     W, F  23   No          Cr/linear, IgG             No
9, 1993     x, M  40   Yes         x                          Yes
9, 1993     x, M  58   Yes         Cr/x                       Yes
10, 1993    W, M  24   Probably    Normal/linear, IgG, C3     No
11, 1993    x, M  26   Yes         RP/linear, IgG, C3         Yes
12, 1993    x, M  75   x           x/linear, IgG              Yes
13, 1993    x, F  13   Yes         Cr/linear, IgG, C3         Yes
13, 1993    x, F  17   Yes         Cr/linear, IgG, C3         Yes
14, 1993    x, F  19   Yes         x                          Yes
15, 1994    W, M  31   Yes         Cr/linear, IgG             Yes
16, 1994    B, M  60   No          Cr/IgG, C3                 No
16, 1994    B, M  56   Yes         x                          Yes
16, 1994    B, M  29   Yes         x                          Yes
16, 1994    W, M  45   Yes         Cr/IgG, IgA, IgM, C3       Yes
16, 1994    W, M  60   Yes         PM/linear                  Yes
16, 1994    W, M  21   Yes         x                          Yes
16, 1994    B, F  26   Yes         Me/linear, IgG, C3         No
16, 1994    W, F  59   Yes         Cr/linear, IgG, IgM        Yes
16, 1994    W, F  65   Yes         Cr/linear, IgG             Yes
17, 1994    x, M   x   Yes         x                          Yes
18, 1994    x, F  40   Yes         x                          Yes
19, 1994    W, x  30   x           x                           x
20, 1994    x, M  21   No          RP/linear, IgG             Yes
21, 1994    x, F  72   x           Cr/x                       Yes
22, 1995    x, F  67   No          Cr/linear, IgG             Yes
22, 1995    x, M  75   Yes         x                          Yes
22, 1995    x, M  79   Yes         x                          Yes
22, 1995    x, F  70   No          x                          Yes
22, 1995    x, F   9   Yes         x                          Yes
22, 1995    x, M  34   No          x                          Yes
22, 1995    x, M  23   No          x                          Yes
22, 1995    x, F  52   Yes         x                          Yes
22, 1995    x, F  64   Yes         x                          Yes
22, 1995    x, F  77   Yes         x                          Yes
22, 1995    x, F  27   No          x                          Yes
22, 1995    x, M  31   No          x                          Yes
22, 1995    x, M  77   No          x                          Yes
22, 1995    x, M  25   Yes         x                          Yes
22, 1995    x, F  15   No          x                          Yes
23, 1995    W, M  24   Yes         x/IgG, C3                  Yes
25, 1995    x, F  70   No          Cr/linear, IgG, C3         Yes
26, 1995    x, F  72   x           Cr/linear, IgG             Yes
27, 1995    x, M  24   Yes         C/x                        Yes
28, 1995    B, M  57   Yes         Cr/IgG, C3                 Yes
29, 1996    W, M  72   Yes         Cr/x                       Yes
30, 1996    x, M  65   No          x/linear                   Yes
31, 1996    W, M  59   Yes         FS, Cr/linear IgG          Yes
32, 1996    x, M  11   Yes         x                           x
33, 1996    W, M  20   Yes         Cr/IgG                     Yes
34, 1996    x, M  22   Yes         x                          Yes
35, 1996    W, M  68   No          Cr/linear, IgG             Yes
36, 1996    W, F  49   Yes         x                          Yes
37, 1996    x, F  22   Yes         x                           x
38, 1997    W, F  21   Yes         Cr/linear, IgG             Yes
39, 1997    W, F  53   No          Cr, FS/Iinear, IgG         Yes
40, 1997    x, M  32   Yes         x/linear, IgG              Yes
41, 1997    x, F  68   x           Cr/linear, IgG             Yes
42, 1997    x, M  70   Yes         Cr/linear, IgG             No
43, 1997    x, F  19   Yes         Normal/linear, IgG         Yes
44, 1998    W, M  57   No          Cr/IgG                     Yes
45, 1998    W, M  49   Yes         x/IgG                      Yes
46, 1998    x, F  44   Yes         Cr/linear                  Yes
47, 1998    x, F  73   Yes         x/linear IgG               Yes
48, 1998    x, M  18   Yes         Cr/linear                  Yes
49, 1998    x, F  78   Yes         Cr/linear, IgG, C3         Yes
49, 1998    x, M  65   x           Cr/IgG, C3                 Yes
50, 1998    W, M  18   Yes         Cr/linear, IgG, C3         Yes
51, 1998    x, F  71   Yes         Cr/linear, IgG, C3         Yes
52, 1998    W, F  20   Yes         Cr/linear, IgG             Yes
52, 1998    W, F  63   x           Cr/linear, IgG             Yes
53, 1999    x, M  54   Yes         x/IgA                      Yes (IgA)
54, 1999    W, M  21   Yes         Normal/linear, IgG, C3     No
55, 1999    x, M  67   x           Cr/linear, IgA             Yes (IgA)
56, 1999    x, M  35   Yes         Cr/linear, IgG, C3         Yes
57, 1999    x, M  39   Yes         Cr/linear, IgG, C3         Yes
58, 1999    W, M  23   Yes         FS/linear                  Yes
59, 2000    x, M  19   Yes         Cr/linear, IgG             Yes
60, 2000    x, M  20   Yes         GS,Cr/linear, IgG          Yes
61, 2000    x, F  18   Yes         Cr/linear, IgG             No
61, 2000    x, F  26   Yes         Cr/linear, IgG             Yes

Reference,          Risk
  Year             Factors                Treatment

5, 1993     Tobacco                       C,P
6, 1993     x                             S,C,P,HD
6, 1993     x                             S,C
7, 1993     x                             S,P
8, 1993     URI, tobacco                  S,P,Az
9, 1993     x                             S,C,P
9, 1993     x                             S,C,P
10, 1993    HC                            S,C,P
11, 1993    URI, HC                       S,C,P
12, 1993    x                             S,P
13, 1993    x                             S
13, 1993    URI, tobacco                  S,C
14, 1993    Tobacco, HC                   x
15, 1994    Tobacco, possible HC          S,C,Az
16, 1994    None                          S,C,P
16, 1994    Tobacco                       S,C,P
16, 1994    URI                           S,C,P
16, 1994    Tobacco, possible HC          S,C,P
16, 1994    Possible HC                   x
16, 1994    Tobacco, possible HC          S,C,P
16, 1994    URI, tobacco                  S,C,P
16, 1994    URI, tobacco, possible HC     S,C,P
16, 1994    None                          S,C,P
17, 1994    x                             S,C,P
18, 1994    Tobacco                       x
19, 1994    x                             x
20, 1994    x                             Az, HD
21, 1994    x                             HD
22, 1995    x                             HD
22, 1995    Tobacco                       HD
22, 1995    None                          HD, S
22, 1995    None                          HD
22, 1995    None                          HD, S
22, 1995    None                          HD
22, 1995    Tobacco                       HD, S
22, 1995    None                          HD, S
22, 1995    None                          HD, S
22, 1995    None                          HD, S
22, 1995    None                          HD
22, 1995    Tobacco                       HD
22, 1995    Tobacco                       HD
22, 1995    Tobacco                       S
22, 1995    None                          HD, S
23, 1995    x                             S,C,P
25, 1995    None                          S,P,HD
26, 1995    x                             S,C
27, 1995    x                             S,C,P
28, 1995    x                             S,C,P
29, 1996    Tobacco history               S,C,P
30, 1996    None                          S
31, 1996    Tobacco history, possible HC  S,C
32, 1996    x                             x
33, 1996    Tobacco                       S,C,P
34, 1996    URI, tobacco, HC              S,C,P
35, 1996    x                             S,C,P
36, 1996    Tobacco history               S,P,Az
37, 1996    x                             S, HD
38, 1997    URI, possible tobacco         HD
39, 1997    x                             S,C,P,Az,HD
40, 1997    Tobacco                       x
41, 1997    Tobacco                       x
42, 1997    Tobacco                       S,C,P,HD
43, 1997    Tobacco                       S,C
44, 1998    None                          S,C,P
45, 1998    URI                           S,C,P
46, 1998    x                             S,P,HD
47, 1998    x                             S
48, 1998    x                             S,C,P
49, 1998    x                             S,C,P
49, 1998    x                             S,C,P
50, 1998    URI, tobacco                  S,P,HD
51, 1998    x                             S,C,P
52, 1998    x                             S,C,P
52, 1998    x                             S,C,P
53, 1999    Possible HC                   S,C,P
54, 1999    URI, tobacco                  S,C
55, 1999    Tobacco                       S,C
56, 1999    Tobacco                       S,C
57, 1999    URI, possible HC              S, P, Az
58, 1999    Tobacco                       S,P
59, 2000    None                          S,C,P,HD,MY
60, 2000    x                             S,C,P,HD
61, 2000    HLA-DR2                       S,C,HD,Im
61, 2000    HLA-DR2                       S, Im

Reference,
  Year        Outcome

5, 1993     x
6, 1993     HD
6, 1993     x
7, 1993     Transplantation
8, 1993     Recovered
9, 1993     HD
9, 1993     HD
10, 1993    Recovered
11, 1993    Recovered
12, 1993    Died
13, 1993    Recovered
13, 1993    Died
14, 1993    HD
15, 1994    Awaiting transplant
16, 1994    Died
16, 1994    HD, awaiting transplant
16, 1994    Died
16, 1994    HD
16, 1994    Normal renal function
16, 1994    Died (PH)
16, 1994    Recovered
16, 1994    Recovered
16, 1994    Died
17, 1994    Transplantation
18, 1994    Transplantation
19, 1994    HD
20, 1994    x
21, 1994    HD
22, 1995    HD
22, 1995    HD
22, 1995    Died
22, 1995    HD
22, 1995    HD
22, 1995    HD
22, 1995    HD
22, 1995    HD
22, 1995    HD
22, 1995    Died
22, 1995    HD
22, 1995    HD
22, 1995    HD
22, 1995    HD
22, 1995    HD
23, 1995    x
25, 1995    x
26, 1995    HD
27, 1995    x
28, 1995    HD
29, 1996    Died
30, 1996    x
31, 1996    x
32, 1996    HD
33, 1996    Transplantation
34, 1996    Recovered
35, 1996    Died (CVA)
36, 1996    HD
37, 1996    Transplantation
38, 1997    x
39, 1997    Recovered
40, 1997    HD
41, 1997    Died (CVA)
42, 1997    Died
43, 1997    Recovered
44, 1998    Recovered
45, 1998    HD
46, 1998    Died (RF)
47, 1998    Died (RF)
48, 1998    Transplantation
49, 1998    PD
49, 1998    HD
50, 1998    Recovered
51, 1998    Died
52, 1998    Recovered
52, 1998    Transplantation
53, 1999    Recovered
54, 1999    Recovered
55, 1999    Died
56, 1999    HD, awaiting transplant
57, 1999    Transplantation
58, 1999    x
59, 2000    Transplantation
60, 2000    HD
61, 2000    Recovered
61, 2000    Recovered

x = Unknown, C = cyclophosphamide, P = plasmapheresis, Cr = crescentic,
S = steroids, HD = hemodialysis, URI = upper respiratory illness, Az =
azathioprine, HC = hydrocarbon exposure, RP = rapidly progressive, PM =
proliferative mesangial, PH = pulmonary hemorrhage, Me = mesangial, FS =
focal segmental, CVA = cerebrovascular accident, RF = respiratory
failure, PD = peritoneal dialysis, My = mycophenolate, GS = global
sclerosis, Im = immunoadsorption.

TABLE 2

Signs and Symptoms in Patients With Goodpasture's Syndrome

                                       No. of
                                      Patients

Symptoms
    Hemoptysis                       35/54 (65%)
    Cough                            15/54 (28%)
    Shortness of breath              15/54 (28%)
    Fever                            15/54 (28%)
    Dyspnea on exertion              14/54 (26%)
    Nausea/vomiting                  11/54 (20%)
    Chest pain                       10/54 (19%)
    Lower extermity edema             9/54 (17%)
    Oliguria/decreased urine output   9/54 (17%)
    Fatigue                           8/54 (15%)
    Myalgia                           7/54 (13%)
    Malaise                           7/54 (13%)
    Weight loss                       5/54 (9%)
    Hematuria                         7/54 (7%)
    Weakness                          4/54 (7%)
    Chills                            3/54 (6%)
    Orthopnea                         2/54 (4%)
    Anemia                            2/54 (4%)
Signs
    Crackles                         16/35 (46%)
    Lower extermity edema            12/35 (34%)
    Normal                           10/35 (29%)
    Tachycardia                       9/35 (26%)
    Tachypnea                         9/35 (26%)
    Rhonchi                           4/35 (11%)
    Fever                             2/35 (6%)
    Murmur                            2/35 (6%)
    Jugular venous distention         1/35 (3%)
    Pallor                            1/35 (3%)
    Visual changes                    1/35 (3%)
    Hemoptysis                        1/35 (3%)

* Of 54 patients reporting symptoms and 35 patients reporting signs.

TABLE 3

Common Laboratory Abnormalities in Patients with Goodpasture's Syndrome

                       No. of
Laboratory            Patients

Hemoglobin
  <12                33/37 (89%)
  Normal              4/37 (11%)
Blood urea nitrogen
  Increased          27/36 (75%)
  Normal              9/36 (25%)
Creatinine
  Increased          35/49 (71%)
  Normal             14/49 (29%)
Urinalysis
  Proteinuria        41/60 (68%)
  Hematuria          43/60 (72%)
  Granular casts      7/60 (12%)
  Normal              4/60 (7%)

References

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(5.) De Marchi S. Cecchin E, Pirisi M, et al: Atypical antiglomerular basement membrane disease associated with defective leukocyte phagocytosis and hyper-IgE syndrome. Nephrol Dial Transplant 1993; 8:88-92

(6.) Esnault VL, Testa A, Jayne DR, et al: Influence of immunoadsorption on the removal of immunoglobulin G autoantibodies in crescentic glomerulonephritis. Nephron 1993; 65:180-184

(7.) Hoscheit AM, Austin JK, Jones WL: Nonrhegmatogenous retinal detachment in Goodpasture's syndrome: a case report and discussion of the clinicopathologic entity. J Am Optom Assoc 1993; 64:563-567

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KEY POINTS

* Goodpasture's syndrome is a rare disorder characterized by pulmonary hemorrhage, glomerulonephritis, and antiglomerular basement membrane antibodies.

* Most patients present with hemoptysis and crackles, while more than 90% had antiglomerular basement membrane antibodies.

* Most patients have multiple risk factors, including hydrocarbon exposure, upper respiratory tract illness, and tobacco use.

* Treatment for Goodpasture's syndrome included immunosuppression, plasma exchange, and hemodialysis.

* Physicians should consider the diagnosis of Goodpasture's syndrome in patients who present with renal or pulmonary disease, since early diagnosis affords better outcomes.

From the Department of Medicine, Tulane University School of Medicine, New Orleans, La, and the Department of Medicine, Massachusetts General Hospital, Boston.

Reprint requests to Mrugeshkumar K. Shah, MD, 10 Summer St, Suite 303S, Maiden, MA 02148.