Vol.3, No.8, 534-536 (2011) Health
Copyright © 2011 SciRes. Openly accessible at http://www.scirp.org/journal/HEALTH/
Necrotizing sarcoid granulomatosis: A case report and
review of progresses in this disease
Guido Epping1, Carlos A. Schwengle1, Riena P. Aliredjo2, Michiel Wagenaar 1
1Department of Pulmonary Medicine, Medisch Spectrum Twente, Enschede, The Netherlands; g.epping@mst.nl
2Department of Pathology, Medisch Spectrum Twente, Enschede, The Netherlands.
Received 9 May 2011; revised 27 June 2011; accepted 18 July 2011.
We present the case of a young male who vis-
ited the emergency room with progressive dys-
pnea and left sided chest pain. A chest X-ray
was performed which showed a pattern of dif-
fuse nodular and reticular opacities. The diag-
nosis of Necrotizing Sarcoid Granulomatosis
(NSG) was made by histopathological examina-
tion of a lung biopsy specimen and by exclud-
ing other causes of granulomatous disease. He
was treated with corticosteroids. Because of
persistent extrapulmonary (ocular) involvement
a maintenance dose of prednisone was needed.
Necrotizing Sarcoid Granulomatosis is a granu-
lomatous disease with necrosis and vasculitis
of unknown cause. The resemblance between
NSG and (nodular) sarcoidosis is strong, but
subtle differences exist. The prognosis is good
and therapy is usually not necessary. However
in some cases there is a need for therapy with
corticosteroids, depending on the involved or-
gans and/or the degree of impairment.
Keywords: Necrotizing Sarcoid Granulomatosi s;
Sarcoidosis; Extrapulmonary Involvement;
Computed Tomography; Pathology
A 21 year old non-smoking male was presented at the
emergency department of our hospital. He suffered from
pain in his right shoulder and on the left side of his chest,
with progressive shortness of breath for two days. He
had a productive cough with clear blank sputum. There
was no haemoptysis, nor were there co nstitution al symp-
toms like fever or night sweats. There were no com-
plaints that could be related to a vasculitis-like disease,
e.g. vision problems, joint aches, skin problems or ab-
dominal discomfort. He had a blank history, and didn’t
use any medication.
Our patient was tachypnoeic with an oxygen satura-
tion of 98% with 3 litres of supplemental oxygen per
minute. His blood pressure was 110/70 mmHg with a
regular pulse of 115/minute. He had a low grade fever,
with a temperature of 38,2oC. There were no palpable
lymph nodes. Chest examination revealed wheezing on
both sides and crackles at the bases. There were no other
abnormalities at the physical examination.
The posteroanterior view of the chest X-ray showed
diffuse reticular and nodular opacities of both lungs
(Figure 1). A computed tomography of the chest and
upper abdomen (Figure 2) showed mediastinal and right
hilar lymphadenopathy. Diffusely dispersed alveolar
opacities were present in both lungs, which were more
pronounced in the lower lobes.
Laboratory findings showed an elevated erythrocyte
sedimentation rate. The C-reactive protein and white cell
counts were both minimally elevated. The serum angio-
tensin converting enzyme (ACE) level was low. Anti-
nucleair antibodies (ANA) and anti-neutrophil cytopla-
smatic antibodies (ANCA) were both negative. Analysis
of his urine showed no abnormalities. Pulmonary func-
tion tests showed a restrictive pattern (TLC = 53% of
reference) with a diminished diffusion capacity (DLCO =
48%). Arterial blood gas analysis (ABGA) breathing
room air showed a mild hypoxemia (pO2 9.6 kPa), with
normocapnia ( pC O2 5.1 kPa).
We performed extensive microbiological testing. All
tests on tuberculosis were negative (Mantoux; Quanti-
feron TB Gold test; Acid-fast staining; PCR tests and
cultures). Serological tests on HIV, viral respiratory infe-
ctions (adenovirus; influenza A/B; parainfluenzae 1/2/3/;
RS virus) and so-called atypical pathogens (chlamydia
pneumoniae and psittaci; coxiella burnetii; legionella
pneumophila; mycoplasma pneumonia) were all nega-
Bronchoscopic examination was performed. No en-
dobronchial abnormalities were noted. Central biopsy
and broncho-alveolar lavage (BAL) showed no signs of
malignancy or (specific) inflammation. Cultures were
G. Epping et al. / Health 3 (2011) 534-536
Copyright © 2011 SciRes. Openly accessible at http://www.scirp.org/journal/HEALTH/
Figure 1. Chest X-ray (PA view) with diffusely dispersed re-
ticular and nodular opacities in both lungs.
Figure 2. Computed tomography of the chest with enlarged
lymph nodes both mediastinal and hilar, and diffusely dis-
persed nodular opacities.
negative. The BAL contained a lymphocytosis and neu-
trophilia, with in the differential: 1% eosinophils (nor-
mal < 1% - 2%), 9% neutrophils (normal < 3%), 58%
macrophages (normal > 80%) and 32% lymphocytes
(normal < 15%). The CD4/CD8 ratio was slightly ele-
vated (3,0) and the CD103/CD4 ratio was decreased
(0,12). The BAL results with lymphocytosis, slightly ele-
vated CD4/CD8 ratio and a diminished CD103/CD4
ratio were compatible with sarcoidosis.
Vasculitis and malignancy were less probable but
could not be excluded. A video-assisted thoracoscopic
(VATS) biopsy was performed of the right lung. Histo-
logical examination showed nodular abnormalities con-
sisting of granulomas with epitheloid cells, Langerhans
type giant cells and necrosis. The granulomas were dis-
persed through the parenchyma and along the bronchi a
reflection of vasculitis of the adj acent blood vessels was
seen (Figure 3). The pathological and clinical diagnosis
therefore was: Necrotizing Sarcoid Granulomatosis.
Because of the extensive pulmonary involvement and
the seriousness of his complain ts we decided to treat our
patient with corticosteroids. We started with a dose of
60 mg of prednisone a day and gradually tapered the
dose till zero, over a period of six months. With this re-
gime complete recovery was achieved, with normaliza-
tion of his chest X-ray. His chest pain was completely
disappeared. However, after seven months he developed
a sicca like syndrome of the eye. He was examined by an
ophthalmologist and an octreotide scan was performed,
the diagnosis of dacryoadenitis was made. Prednisone
was reintroduced in a dose of 30 mg a day. Again the
dose was gradually reduced. This time we were unable
to reduce it below a dose of 10 mg a day, because of a
flare of his pulmonary complaints. With a maintenance
dose of ten milligrams he is free of symptoms for a year
NSG is a rare entity which was first described by
Liebow in 1973. [1-3] The incidence of NSG is not
known. NSG is more common in females than males and
most are non-smokers. [4] Few cases are published with
a total of seven publications since the case-series by
Churg and Gupta in 1979. [4] The age of presentation
varies, with distribution of cases between the second and
the sixth decade of life. [2,4].
Figure 3. VATS biopsy specimen: in the center vasculitis sur-
rounded by tissue with multiple granulomas (Hematoxylin-
Eosin stain, magnification X 50).
G. Epping et al. / Health 3 (2011) 534-536
Copyright © 2011 SciRes. Openly accessible at http://www.scirp.org/journal/HEALTH/
NSG was originally characterized by the following
three characteristics [2]: 1) on pathological examination
there was a background of sarcoid-like granulomas, a
prominent and usually granulomatous vasculitis, and
varying degrees of necrosis, often superimposed on a
mass of confluent granulomas; 2) on chest X-ray, there
were pulmonary nodules but no enlarged hilar lymph
nodes; and 3) a clinically benign course with minimal or
no therapy.
We present a patient diagnosed with Necrotizing Sar-
coid Granulomatosis, with extrapulmonary involvement.
We reviewed the available literature and addressed dif-
ferent aspects of this disease. NSG is viewed by some as
a vasculitis with sarcomatoid reaction, while others look
at it as a variant of sarcoidosis. Either way it is a disease
of unknown etiology, with a good prognosis that some-
times requires treatment with corticosteroids.
There are histological resemblances between NSG and
sarcoidosis but also with vascu litis. The question wheth-
er NSG is a vasculitis with sarcoid reaction (angiocentric
granulomatosis) or a variant of sarcoidosis, still remains
unanswered. [5] Some believe it is a variant of nodular
sarcoidosis e.g. nodular sarcoidosis with necrosis. [3,6]
Others suggested that some cases of NSG are actually
inactive granulomatous infections. [7] The etiology and
pathogenesis of NSG is still unknown . [4]
[1] Liebow, A.A. (1973) The J. burns amberson lecture.
Pulmonary angiitis and granulomatosis. American Re-
view of Respiratory Disease, 108, 1-18.
[2] Churg, A., Carrington, C.B. and Gupta, R. (1979) Ne-
crotizing sarcoid granulomatosis. Chest, 76, 406-413.
NSG usually involves the lungs, but as in our case ex-
tra-pulmonary involvement has been described. There
are case reports of pleural, eye, skin, liver, spleen, kid-
ney and neurological involvement. [4,8-11]
[3] Churg, A. (1983) Pulmonary angiitis and granulomatosis
revisited. Human Pathology, 14, 868-883.
[4] Quaden, C., Tillie-Leblond, I., Delobbe, A., et al. (2005)
Necrotising sarcoid granulomatosis: Clinical, functional,
endoscopical and radiographical evaluations. European
Respiratory Journal, 26, 778-785.
The diagnosis of NSG can be suspected based upon
clinical findings, laboratory tests, lung function and ra-
diographic findings (chest x-ray and CT scan). Patients
can present with systemic symptoms, such as fatigue,
fever, night sweats and weight loss. Extra-pulmonary
disease can be symptomatic. Besides, patients can have
respiratory symptoms such as cough, dyspnoea and chest
pain. Physical examination is usually non-specific or
completely normal. Although crackles at lung examina-
tion, joint swelling and splenomegaly can sometimes be
found. All these findings are nonspecific.
[5] Rosen, Y. (2007) Pathology of sarcoidosis. Seminars in
Respiratory and Critical Care Medicine, 28, 36-51.
[6] Popper, H.H., Klemen, H., Colby, T.V. and Churg, A.
(2003) Necrotizing sarcoid granulomatosis—Is it differ-
ent from nodular sarcoidosis? Pneumologie, 57, 268-271.
[7] Koss, M.N., Hochholzer, L., Feigin, D.S., Garancis, J.C.
and Ward, P.A. (1980) Necrotizing sarcoid-like granulo-
matosis: clinical, pathologic, and immunopathologic
findings. Human Pathology, 11, 510-519.
Pulmonary function tests are usually normal. [4] La-
boratory findings are non-specific and non-diagnostic.
The erythrocyte sedimentation rate is usually elevated.
ACE is typically low and ANCA is usually negative. [4]
Both sarcoidosis an d vasculitis however, cannot be ruled
out by these laboratory investigations. A chest X-ray or
Computed Tomography of the chest can show alveolar
opacities or infiltrates, a solitary nodule or diffuse nod-
ules, hilar and/or mediastinal lymphadenopathy, pleural
thickening and cavitations. There are no specific radio-
graphic findings that can distinguish NSG from other
granulomatous diseases with certainty. [4]
[8] Alberti, S., Falleni, M., Tarsia, P., et al. (2006) A 13-year-
-old female with shortness of breath and pleuritic chest
pain. European Respiratory Journal, 28, 876-882.
[9] Chittock, D.R., Joseph, M.G., Paterson, N.A.M. and
McFadden, R.G. (1994) Necrotizing sarcoid granuloma-
tosis with pleural involvement: Clinical and radiographic
features. Chest, 106, 672-676.
[10] Dykhuizen, R.S., Smith, C.C., Kennedy, M.M., et al.
(1997) Necrotizing sarcoid granulomatosis with ex-
trapulmonary involvement. European Respiratory Jour-
nal, 10, 245-247. doi:10.1183/09031936.97.10010245
To confirm the diagnosis a histological examination of
lung tissue or other involved organs is necessary. A rep-
resentative lung biopsy can be obtained through a VATS
or open lung procedure.
[11] Strickland-Marmol, L.B., Fessler, R.G. and Rojiani, A.M.
(2000) Necrotizing sarcoid granulomatosis mimicking an
intracranial neoplasm: Clinicopathologic features and re-
view of the literature. Modern Pathology, 13, 909-913.
Prognosis is generally good with more than 80%
complete recovery, either spontaneous or with therapy.
[2,12] There are no guidelines for the treatment of NSG.
Depending upon disease progression and the degree of
impairme nt, cort i cost eroids should be consid ered .
[12] Lynch III, J.P., Kazerooni, E.A. and Gay, S.E. (1997)
Pulmonary sarcoidosis. Clinics In Chest Medicine, 18,
755-785. doi:10.1016/S0272-5231(05)70417-2