isolated or established as a factor beyond doubt. Gruber in 1925 first suggested that the pathol- ogical lesion is one of hyperergic reaction in the arterial wall.
POLYARTERITIS NODOSA REPORT OF A
CASE
Bj- B. P. TRIBEDI, m.u. (Cal.), D3. (Lond.), f.n.i. Professor of Pathology and Bacteriology, Medical College, Calcutta and N. K. CHANDA,
Curator,
Pathological
ma
Museum,
(Cal.) Medical
College,
Calcutta
The disease first described by Kussmaul and Maier in 1866 was named periarteritis nodosa,
Plate XLIV POLYARTERITIS NODOSA
:
of right 1.?The posterior branch of right Fig. Fig. 1.?The descending branch posterior descending of both both branches of and short short descending coronary artery and descending branches coronary artery left coronary vessels show show characteristic characteristic and left right coronary vessels right and nodosa,. nodules nodules of of polyarteritis polyarteritis nodosa.
REPORT OF A CASE
:
B. P. TRIBEDI & N. K. CHANDA.
(0. A.)
PAGE 450
2.?A noduie the epicardium fibrinoid necrosis of the noduie under under the necrosis of the showing fibrinoid Fig. 2.?A showing Fig. epicardium of the and infiltration infiltration with muscle muscle coat coat of the vessels vessels and with neutrophils, neutrophils, lymphocytes, lymphocytes, filled up with aa blood of the the vessel vessel is is filled blood clot Lumen of clot which which isis up with eosinophils. eosinophils. Lumen infiltrated with with neutrophils. neutrophils. again again infiltrated shown above heart muscle The perivascular The tissue shown above between between heart muscle and and blood blood perivascular tissue and infiltrated infiltrated with with cells cells mostly vessels is is cedematous cedematous and vessels mostly neutrophils, neutrophils, few eosinophils. and few eosinophils. lymphocytes and lymphocytes
Plate XLV
1 POLYARTERITIS NODOSA
:
REPORT OF A CASE
Fig. 3.?Kidney showing fibrinoid necrosis of media and intima in a vessel c narrowing of lumen and infiltration of all coats and perivascular tissue with lymphocytes, monocytes and few eosinophils. The tubules at the top have taken an acidophilic staining.
:
B. P. TRIBEDI & N. K. CHANDA.
(O. A.) PAGE 450
Fig. 4.?Liver showing fibrinoid necrosis of vessel wall, perivascular infiltration of round cells and intravascular clot with cellular infiltration. infiltration.
POLYARTERITIS NODOSA
Oct., 1952]
better known at present as polyarteritis nodosa, because many vessels are affected. Along with
Periarterites there is panarterites. The disease is an affection of small arteries arterioles, characterized by a segmental fibrinoid necrosis beginning in the media and extending to other coats along with infiltration of mainly neutrophils and eosinophils. Grossly, multiple occurrence of nodular lesions 2 to 3 mm. in diameter along the course of small
is characteristic. The nodosity is by necrosis and granulomatous proliferation or by aneurysm formation. Secondary changes occur in the vessel wall in the form of thrombosis, organization, recanalization and healing with fibrosis in the wall of the arteries. Harris et. al. (1939) have analysed the frequency of involvement of visceral organs in 87 cases of polyarteritis nodosa and observed the following frequency : heart in 84 per ecnt, kidney in 87 per cent, liver in 71 per cent, spleen in 31 per cent and lung in 25 per cent ?f cases.
arteries
caused
Affection of vessels in muscle, nerves and central nervous system reported (Arkin, 1930). Rarely a vessel may also be affected.
peripheral
has been
peripheral
the form of intravascular thrombosis resulting in infarction specially in organs supplied by end-arteries such as kidneys, intestines, liver, spleen and myo-
Secondary changes
.
occur
in
cardium. Aneurvsmal
dilatation resulting in rupture another complication. The rupture is due to excessive thinning of the media or adventitia. In healed stage of periarteritis only fibrous scars are seen in the arterial wall and may result in contracted kidney with renal insufficiency. cardiac fibrosis and heart failure, adrenal atrophy, hepar lobatum, muscle atrophy and Js
Peripheral
nerve
?f the disease reported below
a
degeneration. Owing to rarity case diagnosed by autopsy is
:
Case report T. M.,
male, agecj 20, was admitted to Medical College Hospitals on 2nd January, 1952, with the complaint of burning pain in upper abdomen for last 3 years increased after taking meals and relieved! by taking alkalies with occasional nausea and vomiting. He used to suffer from constipation off and on. a
Hindu
The patient
for gastric
was
operated
upon 5 months back
perforation. No history of taking sulphonamide drugs, iodine containing drugs or arsenical compounds. Examination.?He
was
of moderate build and
nutrition, very asthenic and showed a moderate pallor of the face. Tongue, coated and moist. Temperature on admission 98.2?F., flulse and respiration 86 and 20 respectively. Heart and lung showed no abnormality, V poor
:
TRIBEDI &
$ HAND A
451
Abdominal examination showed no rigidity or No lump could be felt anywhere.
tenderness.
Murphy's sign negative. Blood pressure was 125 systolic and 85 diastolic. Laboratory findings.?Haemoglobin was 50 per cent. White cell count, 13,600/c.mm.; neutrophils, 83 per cent; lymphocytes, 12 per cent; monocytes, 1 per cent; eosinophils, 4 per cent. Total plasma protein content was 6.6 per cent, albumin 4.5 per cent and globulin 2.1 per cent.
30 mg. per 100 cc. Gastric analysis showed a low acid curve. Barium meal examination showed a diverticulum in pyloro-duodenal junction.
Blood
urea was
Progi'ess of the case.?On 23rd February, 1952, the patient complained of pain in right testis. On 28th February, his temperature rose with chill and rigor, no M.P. found. Paludrine given orally 2 tablets thrice a day. Temperature continued as irregular and intermittent. He developed marked tenderness in right hypochondriac and epigastric region and parenteral penicillin was started on 1st March. The temperature touched normal on 6th March. He expired on 8th March, 1952, from gradual asthenia and respiratory failure. Cli?iical diagnosis.?Duodenal with asthenia.
diverticulum
Autopsy findings.?Post-mortem examination held 48 hours after death, the body beingpreserved in the cold chamber. The body was fairly developed but the skin and mucosa were very pale. On opening the thoracic cavity the pericardial was
sac was found to contain 2 oz. of strawcoloured fluid. Heart was normal in size. There were firm whitish nodules along the course of coronary vessels and its branches specially along the course of anterior descending branch of left coronary artery and marginal branch of right coronary artery. They were firm in consistency arid whitish in colour. Similar nodules found on the interior surface along the course of interventricular branch of right coronary artery and the short descending branches of both the right and left coronary artery (plate XL IV, figure 1). The bigger nodules were round or oval, 2 or 3 mm. in diameter, and on cutting showed the lumen of the vessel within. Some of the nodules showed aneurysmal dilatation of the lumen of the vessel with thrombosed blood clot inside. No infarction, hemorrhage or fibrosis of myocardium was seen.
Kidneys were easily. Surface
size, capsule stripped slightly irregular. On it there were red and pale areas. The areas were irregular at the margins varying' from 2 cm. to 4 cm. in diameter. The consistency specially of the pale areas was firm. The left kidney showed a big pale area 5 cm. X 3.5 cm. Lungs were normal in ske. No changes seen in the parenchyma. normal in was
TflE
452
INDIAN MEDICAL GAZETTE
was
upper
Microscopic
findings
(1)
:
_
(9) Arteries.?Femoral artery, brachial artery and basilar artery are all normal in appearance. Discussion The rarity of the disease can well be gauged the fact that only 350 cases were reported in American and Continental literature until 1940, and another 200 between 1940 and 1943 (Wilson and Alexander, 1945). The disease has been mostly diagnosed by autopsy and only in a few cases by biopsy. In India the first authentic case was reported by autopsy (Menon and Veliath, 1945). Chakraboi'ty (1952) later reported a case with affection of brachial artery diagnosed by biopsy. The aetiology of the disease is unknown. Filtrable viruses and allergy have been suggested as setiological factors. Arkin (1930) first thought of a filtrable virus but no virus has been isolated or established as a factor beyond doubt. Gruber in 1925 first suggested that the pathological lesion is one of hyperergic reaction in the arterial wall. The clinical observation of Rich (1942) that polyarteritis nodosa developed as a result of hypersensitive reaction following foreign serum and sulphonamide therapy prompted experimental works and Rich and Gregory (1943) produced typical lesions of periarteritis'' nodosa in rabbits by injections of foreign serum.
by
Heart.?The
nodules under the epicardium on section show the coronary arteries, the media and intima showing fibrinoid degeneration and subintimal proliferation. There are infiltrations of the intima, media and adventitia with neutrophils, lymphocytes, eosinophils and monocytes and a few fibroblasts. The perivascular tissue is (edematous, shows a round cell infiltration of neutrophils, eosinophils, lymphocytes and mononuclear cells. The lumen of the vessel at places shows an intravascular clot with canalization. The clot is infiltrated with neutrophils (plate XLIV, figure 2). The sections made from the muscle of the heart show a similar picture in the blood vessels as above. At some places the necrosed media has given way, at others, the weakened coats of the vessels have led to aneurysmal dilatation of the vessel wall. No infarction or fibrosis of myocardium seen. (2) Kidneys.?They show marked fibrinoid necrosis of media and intima in vessels and infiltration of all coats and- perivascular tissue with lymphocytes, eosinophils and monocytes (plate XLIV, figure 3). There are areas of haemorrhage and small infarction in the kidney substance. At places, there is a great subintimal proliferation of the arterioles, the walls' being infiltrated with lymphocytes and mononuclear cells. Areas of kidnev tissue also show" acidophilic staining of the tubules. (3) Spleen.?It shows similar fibrinoid change in vessel wall and perivascular cellular infiltration. The hemorrhagic area seen on the surface i microscopically is really an infarct, (4) Lungs.?They show affection of a few vessels with fibrinoid necrosis of media and intima, with infiltration by lymphocytes and eosinophils in the wall and perivascular tissue. (5) Liver.?It shows an infarct in the area which was described as a circumscribed homogeneous mass, at other places the vessels show fibrinoid necrosis and cellular infiltration (plate XLIV, figure 4V. (6) Arterioles.?They are in musculature of stomach, intestine, prostate and pancreas also show fibrinoid necrosis and infiltration, of all the coats of the vessels and perivascular tissue with lymphocytes, eosinophils, etc. (7) Suprarenal glands.?They show haemorrhage in medulla.
1952
(8) Testes.?They show fibrinoid necrosis of the muscle coat of the arteriole, haemorrhage and infarction of parenchyma.
normal in size, 1,190 gm. in weight. surface there was a spot cf which on cutting showed haemorrhage irregular a rounded homogeneous area 2 cm. in diameter looking like an infarct. Spleen was 140 gm. in weight. There was a spot of haemorrhage 2 cm. X 2 cm. under the capsule, possibly an infarct. Brain showed no change in the arterial wall in the circle of Wills. On cutting the brain substances no changes in any of the vessel wall Other organs were normal. were seen.
Liver On the
[Oct.,
Periarteritis nodosa has also been produced to iodine (Rich, 1945), arsenicals (Miller and Nelson, 1945; Turner and Paterson, 1946), and to sulphonamide therapy (Rosenak and Maschmeyer, 1945"). It is suggested that allergic diathesis behind the disease will evoke eosinophilic response. Eosinophilia is suggestive, not diagnostic. The case reported above had only 4 per cent ! eosinophils in blood. The rise of eosinophils in ! polyarteritis nodosa is often, due to associated asthmatic condition (Wilson and Alexander, 1945), and not due to the disease process itself. The patient had an operation for gastric ulcer with perforation four months back, post mortem revealed no ulcerations or any scar in stomach or duodenum. Actually ulcers in gastro-intestinal An acute tract can be found in this disease. abdominal pain may also lead to laparotomy
by hypersensitivity
'
(Harris et al, 1939). The patient complained of pain in the testis possibly due to infarction and haemorrhage in parenchyma of the testis. He died of gradual asthenia possibly due to Addisonian syndrome due to ha?morrhage in suprarenal. Polyarteritis, periarteritis, panarteritis and necrosing denote the same morbid condition.?Editor, IM.G, *
arteritis
Oct., 1952]
CAMOQUIN
IN MALARIA
:
Our thanks are due to Dr. D. C. Chakraborty, f.r.c.s. Principal and Superintendent, Medical College, Calcutta, for permission to report the case and to Dr. S. Ghosh, f.r.c.s. (Eng.), Additional Surgeon, Medical College, under whom the patient was admitted.
(Eng.),
REFERENCES
Arkin, A. (1930) Chakrabortt, A. (1952). Orttber, G. B. (1925) ..
..
Sarris,
A. W., Lynch, G. W., and O'Hare,, J. P. (1939).
Amer. J. Path., 6, 401. Indian Heart J., 3, 239. Virchow's Arch. Path. Anat., 258, 441. Arch. Intern. Med., 63, 1163.
Kussmaul. and A., Maier, R. (1866). Menon, T. B., and Veliath, G. D. (1945).
Deut. Arch. Klin. Med., 1> 484. Indian Med. Gaz., 80, 452.
Miller,
Lancet, ii.
Nelson,
Rich,
H. G., and M. G. (1945).
A. R. (1942)
Idem
..
(1945) Rich, A. R., and Gregory, J. E. (1943). Rosenak, B. D., and ..
Maschmeyer,
R.
H.
200.
Bull. Johns Hopkins Hosp.,
71, 123. Ibid., 77, 43. Ibid., 72, 65. Lancet, i,
305.
Ibid., ii,
143.
(1945).
Turner,
J. W. A., and H. (1946).
Paterson, J.
Wilson,
K.
Alexander,
8.. H.
and L.
J. Lab. and Clin. Med., 30, 195.
(1945).
BIBLIOGRAPHY
Anderson, (1948).
W.
A.
D.
Pathology. London.
Henry Kimpton,
CHAUDHURI & CHAKRAVARTY
453
