cm-iy PracticC sCbwK Goodpasture's Syndrome: A Nasty Dose of the 'Flu? J. A. McSHERRY, MD SUMMARY A case of tion of white sputum. There was an Goodpasture's syndrome encountered and diagnosed in epidemic of influenza in his com- family practice is described in this article. Goodpasture's original munity at the time. He had not sought article is reviewed with particular reference to the possible medical attention for this illness and etiological relationship of influenza virus infection and the made an apparent recovery. However, subsequent development of this strange, poorly understood disorder. in the ensuing weeks his health began to deteriorate. Dr. McSherry, a College certificant, practices family medicine Clinical examination in late Feb- in Sarnia, Ont. Address for reprints: Carruthers Clinic, ruary was essentially negative, but a 1150 Pontiac Drive, Sarnia, Ont. N7S 3A7. chest X-ray showed diffuse infiltrates in both lung fields. A seven-day course IN 1919, Ernest W. Goodpasturel and recurrent hemoptyses for about of oral tetracycline produced tempo- described two cases of rapidly fatal five or six weeks. During January 1976 rary alleviation of hemoptyses, but pulmonary disease following influenza. he had suffered an illness of one little change in chest X-ray appear- The lung pathology was a hemorrhagic week's duration, characterized by ance. alveolitis and in one case was com- coryza, sore throat, fever, and produc- His general condition continued to bined with subacute glomerulo- nephritis. Goodpasture's syndrome is now generally held to be an immunological disease where antibody is formed to the glomerular basement membrane, and where the alveolar damage is secondary to the production of this antibody. The trend is now increas- ingly to reserve the label 'Goodpas- ture's syndrome' for those cases where pulmonary hemorrhage and subacute glomerulonephritis co-exist, and where it is possible to demonstrate linear deposition of IgG along the basement membrane of the glomerulus using immunofluorescent technique. Case Report A 28 year old white male presented in late February 1976 with a history of increasing* malaise and dyspnea, Fig. 1. Renal biopsy, x 200 magnification. Glomerulus showing crescent accompanied by troublesome cough formation. Hematoxylin and eosin preparation. CAN. FAM. PHYSICIAN 23:195 FEB. 1977 83 merulonephritis with 25 percent out of hospital, in good health and crescent formation (Fig. 1) and im- spirits. munofluorescent studies demonstrated classical deposition of IgG along the Discussion glomerular basement membrane (Fig. Goodpasture's original paper em- 2). Serum for antiglomerular basement phasized the relationship of pulmo- membrane antibody was positive at nary lesions to influenza infection. He 1:64 and viral studies showed a high recorded the presence of proliferative titre of antibody to influenza and glomerulonephritis in one of his pa- Mycoplasma pneumoniae, suggesting tients, did not really make much of it, recent infection. but nonetheless was elected to donate The patient is currently under the eponym to the association of therapy; at the time of writing he is pulmonary and renal lesions by Fig. 2. Renal biopsy, x ZUU magnifica- tion. Glomerulus showing linear de- position of IgG. Immunofluorescent technique. TABLE 1 Laboratory Investigations Hemoglobin 6.9 G/100 ml deteriorate, and he complained of in- Reticulocyte count 11% creasing dyspnea and recurrence of hemoptyses. He was therefore ad- Sedimentation rate 68 mm mitted to Sarnia General Hospital on Urinalysis Albumen + April 7, 1976 for further assessment. Blood +++ Physical examination of the patient at that time revealed no abnormality Urine microscopy 8-10 RBC/HPF except for extreme pallor. He was a 5-8 WBC/HPF tall, obese young man of robust 2-4 granular casts/LPF physique. Laboratory investigations Urine culture negative are summarized in Table 1. Relevant AAFB negative past medical and family history was of cytology negative suspected tuberculous infection in in- Serum iron 21 ,ug% fancy and of proven TB nephritis in Total IBC 414 his father, who had received adequate Unsaturated IBC 395 therapy and follow-up. TB skin testing % Saturation 5.3% on the patient was negative on two occasions. Cholesterol normal Chest X-ray again showed diffuse Triglycerides early type IV rise bilateral pulmonary infiltrates, and ASO titre negative IVP was normal although upper GI series showed a duodenal ulcer, which Cold agglutinins negative was a surprise to the patient. Direct Coombs test negative The clinical picture was then in Liver profile Bilirubin 1.8 mg % essence one of a previously healthy otherwise normal young man with severe iron deficiency anemia, bilateral pulmonary infiltrates, BUN electrolytes normal urinalysis suggesting subacute glo- Prothrombin time normal merulonephritis without evidence of renal failure and asymptomatic Stools for occult blood negative duodenal ulcer. Serum folate normal A presumptive diagnosis of Good- Serum B12 165 Pg/ml pasture's syndrome was made and he (normal 200-900) was transferred to the care of Dr. R. M. Lindsay, Renal Unit, Victoria Serum proteins normal Hospital, London, Ont., to whom I am and electrophoresis normal indebted for details of renal biopsy Immunoglobins IgG 780 mg % (normal 800-1800) and photomicrographs, and for further IgA 80 mg % (normal 90-450) history. IgM 155 mg % (normal 60-250) The diagnosis of Goodpasture's LDH normal syndrome was confirmed by renal and fractionation normal biopsy. Microscopy of renal tissue showed diffuse proliferative glo- CAN. FAM. PHYSICIAN 23:197 FEB. 1977 85 authors who discounted his view of their etiology.2 However, with the march of time and the advance of medical knowl- EIAVIU 75~~~~~~~mgTablets edge, Goodpasture's syndrome has (amitriptyline hydrochlorde, MSD Std.) been shown to be a specific entity, basically immunological, but suspected Indications: In the drug management of depressive Adverse Reactions: Behavioural: Activation of of being precipitated by exposure to illness including that accompanied by anxiety. May latent schizophrenia; high doses may cause tempo- volatile or also be of value in persistent functional nocturnal rary confusion or disturbed concentration, or rarely, hydrocarbons by viral infec- enuresis when organic causes have been excluded. transient visual hallucinations; hypomanic reac- tion.3 Dosage Summary: Oral: Dosage should be tions; drowsiness which usually disappears with initiated at a low level and increased gradually, continuance of therapy; insomnia, giddiness, rest- A recent editorial4 on the role of noting carefully the clinical response and any lessness, agitation, fatigue, nightmares, disorienta- viruses in the etiology of multiple evidence of intolerance. tion, delusions, excitement, anxiety and jitteriness. Initial dose for adults: 25 mg three times a day. If Neurological: Epileptiform seizures; numbness, sclerosis suggests that the pathogenesis necessary, increase doses preferably in the late tingling, paresthesias of the limbs including peri- afternoon and/or bedtime to a total of 150 mg a day. pheral neuropathy; dizziness, fine tremor, head- may be virus infection altering cell Hospitalized patients may require 100 mg a day ache, ataxia, alteration in EEG patterns, extra- membrane antigenicity in such a way initially; increased gradually to 200 mg a day if pyramidal symptoms, tinnitus and incoordination; necessary. A small number need as much as severe tremor only observed with high doses. as to precipitate an autoimmune dis- 300 mg a day. Autonomic: Evidence of anticholinergic activity, order. If this Adolescent and Elderly Patients: In general, lower such as urinary retention, reversible dilatation of is so in MS, then why not dosages recommended: 10 mg three times a day the urinary tract, constipation, and more rarely in Goodpasture's syndrome with its with 20 mg at bedtime or less, may be satisfactory. paralytic ileus of particular concern in the elderly; Maintenance dose is usually 25 mg two to four dry mouth, blurred vision and disturbance of proven immunological basis? times a day. When satisfactory improvement has accommodation. Family physicians are only too been reached, reduce to lowest amount that will Cardiovascular: A quinidine like effect and other maintain relief of symptoms. reversible ECG changes such as flattening or inver- aware of the more common complica- Intramuscular Dosage: Initially, 20 to 30 mg four sion of T waves, and bundle branch block; ortho- times a day. ELAVIL' Tablets should replace the static hypotension, hypertension, palpitation, tions of influenza. Viral and bacterial Injection as soon as possible. arrhythmias, heart block, and, with toxic doses, infections at all levels of the respira- Usage in Children: Not recommended for treatment ventricular tachycardia and fibrillation; myocardial of depression in children under 12 years of age. infarction and stroke. A few instances of unex- tory tract are commonplace conse- In Enuresis: Children 5 to 11 years, 10 to 20 mg one pected death have been reported in patients with hour before bedtime. In older children 25 to 50 mg cardiovascular disorders. quences, and it is within my own may be required. Toxic and Allergic Effects: Bone marrow depression experience that fatal myocarditis may Contraindications: Known hypersensitivity. including agranulocytosis, leukopenia, eosino- Should not be given concomitantly with, nor with- philia, purpura and thrombocytopenia; jaundice also result from an attack of this in at least