Proc. roy. Soc. Med. Volume 69 August 1976
Polyarteritis Nodosa with Peripheral Gangrene and Myocardial Infarction L K Holland MB (for M F Grayson MB MRCP) (North Middlesex Hospital, London N18) J J, woman aged 46 History: 1971: Presented with arthralgia, a photosensitive butterfly rash over the malar region, Raynaud's phenomenon and purpura on the.toes. She had a mild normochromic anmmia, a high ESR (85 mm in 1 hour, Westergren) and a strongly positive sheep cell agglutination titre (SCAT). Although no antinuclear antibodies were detected, a clinical diagnosis of systemic lupus erythematosis (SLE) was made before she was lost to follow up. 1975: Admitted to hospital, after being well for two years, with flitting arthritis, associated with crops of a pink, papular rash on all limbs (Fig 1) which became purpuric within a few hours. Investigations were similar to those of 1971, no antinuclear antibodies were detectable, but SCAT was negative. In addition, C3 complement levels were low and IgM levels were raised on two occasions. There was no evidence of renal disease (urine microscopy, blood urea and creatinine clearance were normal) and biopsy of a skin papule revealed inflammatory changes around capillaries consistent with allergic vasculitis. At this point precise diagnosis was difficult. However within a space of four weeks shedeveloped a transient right trochlear nerve palsy, incipient, and later frank gangrene of three left toes (Fig 2), and a silent anterior myocardial infarction followed by severe left ventricular failure. A clinical diagnosis of polyarteritis nodosa was made and treatment started with 80 mg prednisone daily. Azothiaprine was given but was withdrawn because of severe neutropenia. During recent months she has been taking 17.5 mg prednisone
Fig 1 Left thigh showing allergic vasculitic rash
34 and has a normal ESR (12 mm) and normal renal function. She did, however, have an extension to her myocardial infarct shown on electrocardiography and her heart failure is controlled with digoxin and diuretic therapy. Discussion The case underlined very well the difficulties in coming to a firm diagnosis. within the spectrum of connective tissue disease. The initial presentation was almost a classical clinical picture of SLE but this diagnosis was untenable in the absence of antinuclear or antiDNA antibodies. When she presented the second time with a cutaneous vasculitis and arthritis and no specific serological changes, precise diagnosis was impossible. The appearance of gangrene, mononeuritis and silent myocardial infarction, however, was felt to be good evidence for polyarteritis nodosa although no specific biopsy evidence was available. The skin biopsy report of allergic vasculitis was not decisive as this is the commonest vasculitis to affect the skin and can be seen in a number of conditions such as Henoch-Schdnlein purpura and SLE as well as polyarteritis. When Borrie (1972) reviewed 17 cases of polyarteritis nodosa with skin lesions he found 4 with allergic vasculitis. These cases had the same downhill course as the majority of patients with polyarteritis, but the others with frank polyarteritis of the skin had a benign course with involvement of only skin, muscle and nerve. Various etiological possibilities have been suggested for polyarteritis and include hypersensitivity reactions to drugs such as thiouracils and sulphonamides, and to organisms. Pay (1975) described a case of allergic vasculitis and peripheral gangrene where culture of throat swab grew a hxemolytic streptococcus. Gocke et al. (1-970) described an association between polyarteritis nodosa and Australia
Fig 2 Frank gangrene of three left toes
antigen and postulated immune complex disease involving the antigen, IgM and complement. Although no precipitating cause could be found in this patient, it is interesting to note the raised IgM and lowered C3 complement, despite the fact that she was Australia antigen negative. Because we have no positive biopsy evidence we cannot place this patient's disease into a classification of necrotizing angiitis such as that suggested by Zeek (1952). Rose & Spencer (1957), however, divided their cases of polyarteritis nodosa into pulmonary and classic types. The absence of eosinophilia and lung disease in this woman suggests that we are dealing with the 'classic' rather than the 'pulmonary' disease. In this same series it was found that kidney involvement is the cause of death in 65 % of 'classical' cases. This figure is higher when hypertension secondary to kidney involvement is considered. Therefore, although the long-term prognosis is poor in this patient because of myocardial problems the outlook is brightened by the unusual and fortunate absence of overt renal disease.
One month later in England she was depressed and the gross ECG changes are illustrated in Fig 1. These changes disappeared in a period of nearly sixteen months. In January 1975 a sudden episode of mental confusion, disorientation and restlessness led to admission to St Stephen's Hospital, Fulham, where confusion and incontinence of feces and urine persisted for over a month. Investigations: ECG: normal voltage, sinus bradycardia with flat T waves in leads I, II, III, aVR, aVL, aVF. Cardiac enzymes normal. Serum sodium 114, chlorides 76 mmol/l. Other electrolytes and urea normal. Electroencephalogram (Dr N de M Rudolf) (Fig 2) one month after admission, suggested a metabolic disorder but no localized lesion. The trace was normal in October 1975 after treatment. Thyroid function: PBI 280 nmol/l, T3 resin uptake 0.91 (Thyopac-3), serum thyroxine 39 nmol/l, TSH 48 ,uu/l, 1131 uptake in the neck 10.6 %
Bornie P (1972) British Journal of Dermatology 87, 87 Gocke D J, Morgan M, Lockshin M, Hsu K, Bombardieri S & Christian C L (1970) Lancet ii, 1149 Pay B W (1975) Proceedings of the Royal Society of Medicine 68, 328 Rose G A & Spencer H (I957) Quarterly Journal of Medicine 26, 43 Zeek P M (1952) American Journal of Clinical Pathology 24, 889
Hypothyroidism with Episodic Psychiatric and Cardiac Manifestations P S Jayaratna MB (for B Gottlieb MD FRCP) (St Stephen's Hospital, London S WJO 9TH) M F, female aged 61 History: She had rheumatic fever at the age of 28 and a tendency to depression for fifteen years. In 1963 substernal pain required admission to hospital and the ECG showed T wave inversion in leads I, aVL, V5 and V6 with normal voltage. These changes reverted to normal in two months. The cardiac enzymes and ESR remained normal. The patient continued to have pain on effort till June 1975. In 1971 she was treated with lithium for ten weeks in a nursing home in Spain, for mental confusion and disorientation. The diagnosis was
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hypomania. In 1973 she was in a coma for three days in a hospital in Paris, and the ECG showed T wave inversion in all leads. The serum sodium was 115 mEq/l but the cardiac enzymes were normal.
Fig 1 Tinversion in all leads except a VL and VI normal voltage