G. Epping et al. / Health 3 (2011) 534-536
Copyright © 2011 SciRes. Openly accessible at http://www.scirp.org/journal/HEALTH/
536
3. CONCLUSIONS
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]
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[1] Liebow, A.A. (1973) The J. burns amberson lecture.
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[2] Churg, A., Carrington, C.B. and Gupta, R. (1979) Ne-
crotizing sarcoid granulomatosis. Chest, 76, 406-413.
doi:10.1378/chest.76.4.406
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
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doi:10.1183/09031936.05.00024205
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
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[6] Popper, H.H., Klemen, H., Colby, T.V. and Churg, A.
(2003) Necrotizing sarcoid granulomatosis—Is it differ-
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doi:10.1055/s-2003-39365
[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.
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[9] Chittock, D.R., Joseph, M.G., Paterson, N.A.M. and
McFadden, R.G. (1994) Necrotizing sarcoid granuloma-
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[10] Dykhuizen, R.S., Smith, C.C., Kennedy, M.M., et al.
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trapulmonary involvement. European Respiratory Jour-
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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
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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