John Strype.

Acta tuberculosea et pneumologica Scandinavica online

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already have passed an active stage when the radiogram is taken, partly
because differences in the exposure may mislead the estimate. Furthermore,
the certainty of the diagnosis is of great importance in this connection, for
if laboratory investigations are to tell us something about active phases we
must be sure that the case from which the laboratory test values are
estimated is actually case of sarcoidosis; much work must thus be done
to exclude the possibility of other diseases. Here the following diseases
should be considered in particular: Tuberculosis, silicosis, pulmonary
congestion, widespread bronchiectases, Hodgkin*s malignant lymphogra-
nulomatosis, toxoplasmosis and ornithosis, and leukaemic pulmonary in-
filtrates. The other collagen diseases like periarteritis nodosa and Hand-
Schiiller-Christian's disease may also resemble sarcoidosis in the lungs; and
recently we have had the opportunity in Hillered to see bronchiologenic
carcinosis present a course which bore a close resemblance to sarcoidosis.

A number of pictures are shown to demonstrate that radiograms of the
lungs in sarcoidosis may resemble those seen in several other diseases of



CONCLUSION

Figures have been avoided in this survey, and it will be seen that there
is no definite nosology in this disease, it may often be elusive in its
course and almost without sjmptoms, but may nevertheless be fairly
severe in several cases.



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DISCUSSION
Ingrid Oilg, Denmark:

In relation to the very numerous cases of sarcoid described in the
literature, very little has been published concerning their localisation
in muscles. A very valuable contribution to the study of this problem
has been given by the late neuropathologist, Dr. G. Vraa Jensen, with
whom I collaborated in the team work of which Dr. Gravesen will speak
later.

Within a period of two years Vraa Jensen demonstrated sarcoids in the
muscles of 12 patients. At this time there had been published in the entire
world literature a total of only 32 cases with this localisation — therefore
this was an astonishingly large number of cases.

The explanation for this is that while most of the previous investigators
have only looked for granulomas in a single specimen, Vraa Jensen
per patient. It was found that one could be fortunate if the first examined
examined entire blocks in 10 very large sections — i.e., 300 to 400 specimens
per patient. It was found that one could be fortunate if the first examined
specimen exhibited granulomas.

It is therefore necessary, if the report is: No muscular sarcoid, that
we are informed how many specimens were examined.

Using this technique it appeared that in our series all the patients with
diagnosed sarcoidosis also had sarcoids in the musculature.

However, also in this connection there is — as is always where sarcoid
examination is concerned — the problem of control material.

Is it not possible in all muscle biopsies to find tuberculoid granulomas,
regardless of the disease the patient may have? Here especially lies the
problem in the case of patients suffering from tuberculosis.

By that time Vraa Jensen had examined during ten years numerous
muscle biopsy specimens from patients with medical and neurological
affections (at least 5 specimens were examined in each individual case)
and in only a single case had he found a granuloma in a patient who did
not have sarcoidosis.

At the same time the examination was begun of muscle biopsy specimens
from tuberculous patients hospitalised at the Oresunds-hospitalet in
Copenhagen. Vraa Jensen was engaged in this work when he died. At least
25 patients had been examined and no granulomas could be demonstrated
in the musculature of any of these patients.

Muscular biopsy will probably never be significant as a diagnostic means,
but for research purposes I consider this examination extremely valuable.
Especially I feel that attention should be directed towards the combination
of severe muscular and heart sarcoidosis. We have seen two or three cases
at autopsy.



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DIAGNOSIS OF SARCOIDOSIS

By
Bo Carstensen

During the past ten years slightly over 300 cases of sarcoidosis have
been diagnosed at SoUidens Sanatorium. Three fundamental conditions
were set up for the diagnosis of this disease, as follows:

I. Typical clinical and/or x-ray picture

A typical case history, "multilocalisation", discrepancy between
outspread organic changes and relative absence of symptoms, hyper-
globulinaemia, etc.

II. "Exclusion methods"

^•S*» ^y thorough examinations other imaginable diseases must be
excluded: tuberculosis, pneumoconiosis, s^'stemic diseases (e.g.,
Hodgkin's etc.), neoplasm, mycosis, etc.

III. Biopsy — with histological support for the diagnosis of:

a) Spontaneously changed tissue (usually retroclavian bloc dis-
section a.m. Daniels)

b) Provocative changed tissue (Kveim's, Lemming's, Warfvinge's
skin test).

In the author's opinion the diagnosis may also be made on the basis
of only the first two conditions, but multilocalisation into not less than
three organs is then required. X-rays of the skeleton of the hands and
feet with one or more typical cysts may frequently be a good aid to
diagnosis.

The best method of biopsy in cases of suspected intrathoracic sarcoidosis
was found to be lymph node biopsy J)y the method of Daniels. Prom
January 1952 to May 1958 a total of 421 biopsies of this kind were per-
formed (Odelberg, later Rentzhog). Table 1 shows the results of the biopsy
examinations. (Editor's abstract.)



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TABLE 1.

Biopsy a,m. Daniels in 421 obscure lung cases. Definitive clinic diagnosis.



Preliminary



After Blopay. Confirmed m
or Altered to



StiU .
Uncertain



II



Biopsy of Mo

Aid to DlAgnosii

because



^1



r



Sarcoidosis or
Sarcoidosis f



330



197



121



Tuberculosis?
Sarcoidosis f



33



20



Neoplasm f
Tuberculosis?



46



35



Open



12



12



Total



421



202



13



188



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FROM THE DEPARTMENT OF PULMONARY DISEASES,

ST. GORAN'S HOSPITAL, STOCKHOLM, SWEDEN,

CHIEF: SVEN LOFGREN, M.D.



METABOLIC ASPECTS OF SARCOIDOSIS

By
SvEN LoFGREN and Ren^e Norberg

Regarding the metabolic aspects of sarcoidosis I shall restrict myself
mainly to reporting on our experience as to renal function and hjT)er-
calcaemia.

As is well known, Salvesen in 1935 drew attention to a peculiar kind of
renal involvement in a sarcoidosis patient: there was in his case a slight
proteinuria, a considerable decrease in function with urea retention and
h3rpo8thenuria, but no haematuria, h3rpertension or cardiac hypertrophy.
Subsequently similar observations were described by other workers.

In 1939 Harrell & Fisher gave an account of hypercalcaemia in some
sarcoidosis patients without impairment of renal function. Subsequently
hypercalcaemia associated with sarcoidosis has been reported by several
workers. In many instances, hypercalcaemia occurred coinjointly with
impaired renal function, but in several of the cases on record no signs of
renal disease were to be demonstrated.

Our knowledge of the patho-anatomical basis of the functional changes
just mentioned is by no means complete. For this reason we have carried
out comparative studies of the function and histopathology of the kidneys
in a number of cases of generalised sarcoidosis. For the histological
examination aspiration biopsies were carried out.*

So far the series includes sixteen cases, six with and ten without
hypercalcaemia.

All the six cases with hypercalcaemia presented moderate to serious im-
pairment of renal function, and in the renal biopsy specimens pronounced
lesions of the type previously described in occasional autopsy cases.

Hence, a more or less marked thickening and hyalinisation of Bowman's
capsule was invariably demonstrated in a portion of the glomeruli;
occasionally, a glomerulus was replaced by connective tissue. In five cases



* S. Lofgren, B. Snellman and A. G. H. Lindgren: Renal Complications
in Sarcoidosis. Acta med. Scandinav. 1957 : 159 : 295.



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41

scattered deposits of mineral salts (presumedly calcium phoshate) were
noted, partly in the interstitial connective tissue and partly in the tubular
lumen; in three instances interstitial epithelioid cell granulomas with
structures typical of sarcoidosis were observed.

The clinical picture associated with this renal damage — apart from
functional impairment — was rather deficient in symptoms: no hyper-
tension, slight albuminuria, if any, and spun deposit normal or mildly
pathological. The hypercalcaemia did not exhibit any relationship to
the amounts of proteins or inorganic phosphate in the serum. Of the
clearance tests, PAH clearance as a rule was more markedly depressed
than creatinine and inulin clearances, respectively. This difference would
seem to suggest damage to the tubules.

The findings were quite different in the ten cases of generalised sarcoid-
osis without h3rpercalcaemia. As to the renal function, this was but occasion-
ally mildly impaired. The histological lesions noted in biopsy specimens
within this group were, as a rule, so inconspicuous and indefinite that
they could not confidently be interpreted as having been caused by
sarcoidosis.

Hence the essential information supplied by this sarcoidosis material is
that definitely abnormal renal changes were demonstrable by studies of
function and histo-pathology only in those instances where hypercalcaemia
was present. This would seem to suggest that the renal damage in cases of
sarcoidosis were secondary to the hypercalcaemia.

As is well known, the origination of hypercalcaemia in sarcoidosis has not
been satisfactorily explained. In the main, three suggestions have been
advanced : that it is due to the disturbance of plasma proteins, that it is a
manifestation of parathyroid hyperfunction, or that it results from bone
destruction by sarcoid invasion.

The first theory could not be maintained for two reasons: the calcium
level in the serum does not vary in proportion to either the total serum
protein, albumin, or globulin contents, and further, under normal condi-
tions albumin and not globulin is the protein chiefly concerned in calcium
linkage; in sarcoidosis, as is well known, it is the globulin fraction that is
increased.

Parathyroid hyperplasia has been reported in some cases of sarcoidosis
with hypercalcaemia, but in other instances surgical exploration has shown
the glands to be normal. In none of the cases on record was there a decrease
in the amount of inorganic phosphate, which is definitely adverse to the
idea of parathyroid hyperactivity.

It has been postulated by some authors that the marked rise in blood
calcium might be connected with osseous involvement, particularly since
the alkaline phosphatase in the serum may also be moderately increased.



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42



On the other hand, some workers considered bone involvement and hyper-
calcaemia to be independent and suggested that the disorder of calcium
metabolism starts in the blood rather than in the bone.

In our attempts at solving the problem of the cause of the hypercalcaemia
we have not been more successful than other workers. However, I should
like to describe an observation on the significance of which we have much
speculated, viz., the h3rperuricaemia frequently found in instances of
sarcoidosis. For example, when cases of sarcoidosis are compared with cases
of tuberculosis, there is, as will be seen, a significant difference in the uric
acid content of the blood (Table 1).



TABLE 1
N,P.N. and uric acid in 47 cases of sarcoidosis and 120 cases of tuberculosis



N. P. N.



No. of

C«8M



Uric Acid ^ 3.5 mg%

No. of CMOS



Uric Acid ^ 5.0 mg%
No. of Cmm



SarcoidosLs
Tuberculosis

Sarcoidosis
Tuberculosis

Sarcoidosis
Tuberculosis



< 35



35—44



< 45



30
67

17
63

47
120



22 (73.3% ± 8.0)
21 (31.3% ± 6.7)
D :42.0% ± 9.9***



14 (82.4% ±

38 (71.5% ±

D:10.9%±


9.3)

6.2)

11.2)


36 (76.©% ±

59 (49.2% ±

D:27.4^c ±


6.2)
4.6)
7.7***



9 (30.0% ± 8.4)
3 ( 4.6% ± 2.6)
D:25.5%± 8.7**(*)



10 (71.4% ±
3 ( 5.7% ±
D:65.7%±


11.0)
3.2)
11.4»»*


19 (40.4% ±
6 ( 5.0% ±
D:35.4%±


7.2)
2.0)
7.4»**



In this table the cases are divided into two groups, one with an entirely
normal non-protein nitrogen rate, below 35 mg%, and another group
with a very slightly elevated rate, 35 to 44 mg%. In several of the sub-
groups and, above all, in the collected series a significant difference is
revealed between cases of tuberculosis and of sarcoidosis as regards the
uric acid content.

The estimations of the uric acid content reported just now were carried
out with Folin's method. This, as is well known, yields too low uric acid
values. The method of choice is the enzymatic method in which uricase
is used; this procedure yields higher, adequate values. To a limited extent
it was possible recently to use this method, and we were able to corroborate
the observation that patients with generalised sarcoidosis have a higher
uric acid blood level than controls. Whether this hyperuricaemia is due
to an increased production of uric acid or to a decreased destruction,
or to a decreased elimination, we cannot say at the present moment.

In a preliminary study we have followed the correlation between uric



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mgX
IS

12

II



Vitamin Df

90000 unif5/tfa«

H ?H



RL d". 27 voan



Strum calcium




WMks



Vitamin Pt

fOOOOiNritB/^



6.L ^. a







Strum cttchmi



Strum urk acid



VmIc*

Fi^. i. Simultaneous rise of serum calcium and serum uric acid (enzymatic
method) in two cases of sarcoidosis treated with calciferol.

acid and calcium in the blood during calciferol treiitment of sarcoidosis.
Invariably the uric acid content was found to rise to very markedly
pathologic values parallel with the development of hypercalcaemia (Fig. 1).
We are not prepared to give an explanation of this observation, but we
have asked ourselves whether the hyperuricaemia might possibly be a fac-
tor which contributes to the onset of hypercalcaemia in patients with
sarcoidosis.

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THERAPY OF SARCOIDOSIS

By

Bo Carstensen

A survey is first made of early therapeutic attempts, the author stating
that all reports on attempted therapies and results of therapy in cases of
sarcoidosis up to 1951 are conflicting and unconvincing.

When reports were received concerning adrenocorticotropin and corti-
sone and the biological effect of these hormones in arresting inflammatory
reactions, and not the least concerning their inhibitory effect on the devel-
opment of granulomas, it was quite natural that good results were to be
expected from these hormones in sarcoidosis. As is well known, this disease
is characterised especially by widely spread epithelioid cell granuloma-
tosis. In May 1951 this therapy was taken up for trial at SoUidens Sana-
torium and a total of 130 cases have been treated.

In patients with sarcoidosis of the lung the effect was good in recent cases
of miliary type. However, the high tendency of this type to spontaneous
remissions must be held in mind. In the type with large patches and in the
confluent infiltrative type the effect was considerably poorer and possibly
doubtful — even in recent cases. In fibrous sarcoidosis of the lung of older
standing there was only subjective amelioration, which nevertheless in some
cases was considerable and an important and welcome relief for the patient.

In cutaneous sarcoidosis the effect of steroid therapy was less beneficial
in the author's experience; this was especially true of lupus pernio. When
the disease was localised to the eyes, heart, kidneys or the central nervous
system, the author considered an attempt with steroid therapy absolutely
indicated, since this might prove life-saving or conserve vital or indispens-
able organic functions.

No serious untoward effects were seen. There had developed no cases of
tuberculosis or suspected tuberculous disease in the author's series of cases.
(Editor's abstract.)



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REHABILITATION OF LUNG PATIENTS DURING
SANATORIUM TREATMENT

By
Erkki Larmola ^

In taking care of a patient suffering from pulmonary tuberculosis —
or from a chronic pulmonary disease in general — it is not sufficient
to prescribe sanatorium treatment for a few months, to give antibiotics,
and, when necessary to perform an operation. If we restrict our help to
the medical treatment only and release the patient as soon as this is
finished, we jeopardize everything that has been gained with much work
and care. The goal is not reached before the patient is rehabilitated, i.e.,
before he has returned to the community as a worker, capable of taking
care of himself and his dependents.

From the viewpoint of rehabilitation the tuberculous patients are in a
particular position because of the infectiousness of their disease. Fear of
infection meets them everywhere and is perhaps a greater obstacle to their
rehabilitation than their lowered working capacity, which mostly depends
on the reduced function of the lungs and circulation more than on the
activity of the disease. They have been tuberculous and are lung invalids.

I am of the opinion that the risk of tuberculous infection is still too
much stressed — even in medical literature. As a bacteriologist and a
clinician I have been exposed for more than thirty years to infection by
tubercle bacilli both e vitro and e vivo, but I have never had cause to regard
this danger as significant, either for myself or for my assistants, and I
am sure that this conviction has saved us from needless worry in our work.
If we cannot convince the general public — and many of our colleagues as
well — of the fact that, even though tuberculosis is a contagious disease, its
contagiousness is mostly overrated and that infection can be prevented
by relatively simple measures, we shall meet very great difficulties in our
efforts to rehabilitate our patients.

In Finland we are now speaking of medical rehabilitation as conceptually
different from social rehabilitation. The former does not imply the medical



Kiljava Sanatorium, Kiljava, Finland.

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46

treatment as such, but something complementary to it. It may be defined
as including all those measures that are needed for rehabilitation beyond
the actual medical treatment and are performed at doctor's orders and
under his direct guidance.

Most doctors who work in the field of rehabilitation will perhaps stress
that all through the process the physician must be the central person, the
leader of the working team. My opinion is that this is neither necessary
nor desirable. A recovering patient will reach a point when sanatorium
treatment and medical rehabilitation are completed, and the essential need
is to find a new position for him and perhaps to give him necessary training.
The doctor is not qualified to solve these problems, because he is neither
an educator nor a sociologist. At this point he should retire to the position
of a counsellor and let others take over the lead in the social stage of reha-
bilitation work.

It is regarded as a fundamental principle of rehabilitation that the
work must begin as soon as the disease is diagnosed and the patient has
arrived at the sanatorium. Of course, this does not mean that the patient
must be submitted to active work, training and schooling from the very
beginning. The first task is to determine the patient's need for reha-
bilitation and his qualifications, thus: how does the disease change his
position as a working member of the community and what kind of help
does he need to adjust himself to this changed position, and, secondly,
what kind of faculties and abilities he has or will have to fulfil the
requirements of a new job or to receive training needed for a new
employment. It is the social worker who should clear up the first problem.
The second belongs in the sphere of the psychologist and the educator, but
in both cases the verdict of the doctor is decisive. The doctor alone can
estimate whether the decrease of the patient's working capacity is tempo-
rary and whether he may return to his previous work, or if it will be
necessary for him to find a new occupation and if a given type of work
wll then be suitable or not. The doctor must also judge to what degree the
patient's ovm wishes and plans may be taken into consideration in planning
his rehabilitation. However, if the doctor will make the decisions alone,
in many cases the result will be advice that cannot be taken, as it is based
on the medical viewpoints only and leaves the economical, social and voca-
tional aspects without consideration — because the doctor is not an expert
on them.

Thus, team-work will be necessary from the very beginning, and the
doctor can lead it only if he has some insight into the special areas covered
by the others. It is easier for him to make himself acquainted with these
than for the others to acquire the necessary knowledge in medicine.

As for the other team- members, the participation of the social worker



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begins at once, because the need of rehabilitation can be determined
earlier than the suitability of the patient for work and education. With
a view to the planning of the rehabilitation process, both the temporary
and the permanent invalidity as well as the intellectual qualifications of
the patient must be estimated as soon as possible during his stay in the
sanatorium.

The process of rehabilitation is often divided into three stages: "diver-
sional therapy", "occupational therapy", and "vocational therapy". The
first-mentioned we have practised for a long time. 'Personally, I have often
wondered if we have not succeeded perhaps too well in our efforts to
destroy the stamp of hospital from our sanatoria. In principle I am against
the regimen of prolonged "strict bed rest", because I think that as treatment
it is necessary only in selected cases and should be avoided as detrimental
to rehabilitation. But to take as much time as possible from the sana-
torium routine for arranging light diversional entertainments is not reha-
bilitation. The patient's intellectual activity and capacity should be used
more profitably and directed to practical instruction.

It is often emphasized that the purpose of occupational therapy is to
keep the patient's mind and hands busy, to lead his thoughts away from
brooding on his disease. The nature of the occupational work is regarded
as being of secondary importance. This may be sufficient in cases where
the patient will be able to return to his earlier work or where he is incurable
and needs mental support, but when further rehabilitation is required, also
occupational therapy should be planned in such a way that it will be useful
for the later stages, including as much vocational therapy as possible.

Shortly after the war there was established in Finland a special institute,
the Sanatorium Institute for planning and directing the diversional and
occupational therapy in our sanatoria. During the first years its main line
was general and social education. The patients entered the courses eagerly,
but their interest was of short duration and the practical benefit from the
courses was small. Now the programme is completely remodeled. Instead
of arranging courses that correspond to the education given in secondary
schools, the instruction is concentrated on courses preparing the patients
for vocational schools or training them in practical skills.

Of course, complete vocational training cannot be given during the
treatment in sanatorium. Among the participants there are many who never
Mrill be able to pursue a craft or trade. The patients enter the courses and
leave them at unpredictable points according to the progress of recovery.
When the convalescent has reached a stage at which he can do active work
for more than three hours a day, he will leave the sanatorium. If he can
then return to his earlier work — perhaps gradually and helped by ambu-
latory chemotherapy — he is fortunate.



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Many patients, however, are not in this fortunate position. We calculate
that about 10 per cent of the patients who are leaving our sanatoria both
need further rehabilitation and are capable of profiting by it. Of course,



Online LibraryJohn StrypeActa tuberculosea et pneumologica Scandinavica → online text (page 31 of 38)