BACKGROUND: Necrotizing sarcoid granulomatosis (NSG) was initially defined as a granulomatosis with features in between sarcoidosis and Wegener's granulomatosis (WG), but without extrapulmonary involvement. Subsequent reports have shown that extrapulmonary involvement does exist, and some have suggested NSG as a variant of sarcoidosis. MATERIAL AND METHODS: We studied 10 cases from 3 institutions, and compared clinical and histologic features with those of nodular sarcoidosis and WG. We have analyzed the 10 cases for mycobacterial chaperonin and for the insertion sequence 6110 by PCR. RESULTS AND CONCLUSIONS: Nodular aggregates of granulomas in NSG were similar to those seen in nodular sarcoidosis. Granulocytic vasculitis, a hallmark of WG was not seen in any of the NSG cases. Granulomatous vasculitis was a common feature in cases of NSG, and did not differ from that seen in sarcoidosis. The only unique feature of NSG is infarct-like necrosis, induced by the vasculitis, which might also be interpreted as a function of the duration of the vasculitis, leading ultimately to vascular obstruction. NSG based on our morphologic findings is best classified as a variant of nodular sarcoidosis. In contrast to our findings in sarcoidosis mycobacterial DNA was not found in any of the 10 cases.

Necrotizing sarcoid granulomatosis: a rarity in childhood.Pediatr Pulmonol. 2003 May;35(5):407-11.

Necrotizing sarcoid granulomatosis (NSG) is characterized by pulmonary nodular infiltrates, a typical histology, and a benign clinical course. The etiology and pathogenesis of the disease are still unknown. In childhood, it is extremely rare, with only three reported cases so far. Here we report on an 8-year-old girl, who to our knowledge is the youngest reported patient with NSG. The girl presented with shortness of breath and a sore throat. Chest X-ray and computed tomography (CT) scan revealed multiple nodular opacities of the lung. The symptoms and radiological findings disappeared within 6 months without any treatment. The diagnosis was based on the typical signs and symptoms of NSG and on the exclusion of other diseases. As abnormal immunological findings such as the lack of specific diphtheria antibodies in spite of vaccination against diphtheria were present, we suggest that immunologic mechanisms could play an etiologic role in the pathogenesis of NSG. In addition, the ratio of CD4+/CD8+ T-cells in the peripheral blood was significantly reduced, whereas the CD4+/CD8+ T-cell ratio in the immunohistochemical staining of the lung tissue was elevated. Since this compartmentalization is a typical finding in sarcoidosis, it supports the theory that NSG may represent a variant of sarcoidosis. However, because some characteristics of NSG are uncommon in typical sarcoidosis, NSG may also be an entity in its own right.

Sarcoid-like necrotizing granulomatosis. A case report.Rev Pneumol Clin. 2002 Dec;58(6 Pt 1):351-4.

Necrotizing sarcoid granulomatosis is a rare granulomatous pulmonary angeitis that was first described by Liebow in 1973. We report the case of a 36-year-old woman who was admitted for exploration of chest pain, cough and fever at 39 degrees which had progressed for more than 2 months. Chest X-ray showed several inconstant pulmonary opacities. The physical examination, the radiographic and endoscopic aspects were non-specific. The diagnosis was made only by open lung biopsy which showed histological findings of necrotizing sarcoid granulomatosis. The opacities disappeared with no recurrence spontaneously at one year.

Necrotizing sarcoid granulomatosis in a 14-yr-old female.Eur Respir J. 1999 Mar;13(3):703-5.

A case of a 14-yr-old female with necrotizing sarcoid granulomatosis (NSG) is presented. She was referred because of chest pain and malaise, and radiography revealed multiple pulmonary nodules. Her history showed seasonal sensitization to aeroallergens and hay fever. Infectious agents or malignancies did not characterize these nodules. However, she was treated with macrolide antibiotics because of suspected infection with Chlamydia pneumoniae. Open lung biopsy showed histological findings of NSG, with epithelioid granulomatous inflammation, including giant cells, and vasculitis. No further treatment was performed, and symptoms disappeared within a few weeks. The chest radiograph showed gradual improvement. The aetiology of NSG is poorly understood, and is postulated to represent either sarcoidosis or rare forms of pulmonary vasculitis such as Wegener's granulomatosis or the Churg-Strauss syndrome. In the case presented, a coincidence of infection with Chlamydia pneumoniae suggests an involvement of infectious agents in the pattern of formation of immune complexes in the aetiology of NSG.

Necrotizing sarcoid granulomatosis: a case report and review of the literature.An Med Interna. 1998 Apr;15(4):202-4.

A patient with asymptomatic necrotizing sarcoid granulomatosis is reported. There were bilateral nodules in the chest radiograph but no mediastinal adenopathies were seen on a computed tomography scan. The transbronchial biopsy specimens under fluoroscopy guidance achieved the diagnosis. Steroid treatment was started. The nodules disappeared in three months and reappeared two months later when the patient decided to stop treatment. After two years of follow up the nodules disappeared spontaneously. This is a rare and seldom reported entity, considered by some authors as being and atypical form of sarcoid.

Pulmonary angiitis and granulomatosis: radiologic-pathologic correlation. Radiographics. 1998 May-Jun;18(3):687-710.

Five distinct clinical syndromes of pulmonary angiitis and granulomatosis are currently recognized: Wegener granulomatosis, lymphomatoid granulomatosis, necrotizing sarcoid granulomatosis, bronchocentric granulomatosis, and allergic angiitis and granulomatosis (Churg-Strauss syndrome). Patients typically present in middle age with fever, cough, hemoptysis, dyspnea, or chest discomfort. Upper airway involvement such as sinusitis suggests Wegener granulomatosis. Medical renal disease is associated with Wegener granulomatosis and Churg-Strauss syndrome. Asthma may be present in bronchocentric granulomatosis and Churg-Strauss syndrome. Pathologic examination of these entities demonstrates vasculitis, granulomatous inflammation, and parenchymal necrosis. The radiologic manifestations of pulmonary disease are varied, but the most typical appearance is that of multiple nodules or masses that may demonstrate cavitation. Diffuse multifocal air-space opacities with or without cavitation may also be seen. Pulmonary hemorrhage is a well-known presenting manifestation of Wegener granulomatosis and, less commonly, of Churg-Strauss syndrome. Because of the multifocal lung involvement in these diseases, pulmonary metastases and infectious causes are often considered in the differential diagnosis. Affected patients are treated with cytotoxic agents and corticosteroids. The prognosis is variable, depending on the specific syndrome, but may be favorable in the absence of significant complications.

Necrotizing sarcoid granulomatosis diagnosed by video thoracoscopic lung biopsy.Nihon Kyobu Shikkan Gakkai Zasshi. 1997 Aug;35(8):905-9.

A 28-year-old woman was admitted to our hospital because of chest pain. A chest roentgenogram and a chest computed tomogram revealed many nodular shadows on both sides. Examinations of specimens obtained by and by transbronchial lung biopsy during fiberoptic bronchoscopy were not diagnostic, and therefore video thoracoscopic lung biopsy was done. The lung lesion was characterized by aggregates of epithelioid cell granulomas, along with granulomatous and necrotizing angitis. We therefore diagnosed necrotizing sarcoid granulomatosis, and began to administer prednisolone. The nodular shadows disappeared within four weeks. In this case video thoracoscopic lung biopsy was useful in the diagnosis of necrotizing sarcoid granulomatosis in the lung.

Necrotizing sarcoid granulomatosis with extrapulmonary involvement.Eur Respir J. 1997 Jan;10(1):245-7.

Pulmonary lesions, with sarcoid-like granulomas exhibiting noncaseous necrosis, with associated granulomatous arteritis fulfil the diagnostic criteria of necrotizing sarcoid granulomatosis (NSG). We report the case of a woman who presented with recurrent headaches, transient right hemipareses and left-sided ophthalmoplegia. An excised left retro-orbital lesion demonstrated sarcoid like changes, and the illness responded to steroid therapy. Twelve years later, the patient developed a tumour in the right lung. The resected specimen showed the histological hallmarks of NSG, and careful review of the retro-orbital lesion, removed 12 years previously, revealed similar histology. Extrapulmonary involvement in NSG is rare and has been histologically proven on only one previous occasion. The presentation of necrotizing sarcoid granulomatosis in two different systems 12 years apart is unusual and was considered worth reporting.

Necrotizing sarcoid granulomatosis with and without extrapulmonary involvement.Pathol Res Pract. 1996 Mar;192(3):306-13; discussion 314.

Three cases of necrotizing sarcoid granulomatosis (NSG) are reported. All 3 patients had pulmonary lesions; one of them had additional extrapulmonary lesions which were ophthalmologic (dacryoadenitis) and digestive (ulcerative colitis). This patient was followed for 5 years and developed several respiratory, ophthalmologic and digestive recurrences. In 2 cases the diagnosis of NSG had been initially overlooked and the authors emphasize the difficulties of this histologic diagnosis in terms of the differential diagnosis with other necrotic and granulomatous pulmonary diseases such as tuberculosis and Wegener's granulomatosis. They stress the possibility of extrapulmonary lesions in NSG and discuss the relationship between NSG and sarcoidosis.

Pulmonary angiitis and granulomatosis: a review.Can Assoc Radiol J. 1989 Jun;40(3):127-34.

Vasculitis is a clinical-pathological process characterized by inflammation and necrosis of blood vessels. It has been effectively classified by Fauci. Granulomatosis in the lung may be angiocentric or bronchocentric in distribution. The angiocentric forms of granulomatosis and vasculitis include Wegener's granulomatosis, allergic angiitis and granulomatosis of Churg and Strauss, lymphomatoid granulomatosis, and necrotizing sarcoid granulomatosis. Wegener's granulomatosis is a well-defined syndrome characterized by necrotizing granulomatous vasculitis of the upper and lower respiratory tracts, segmental necrotizing glomerulonephritis, and systemic small vessel vasculitis. Allergic angiitis and granulomatosis is a less common multisystem vasculitis with many features similar to polyarteritis nodosa. Lymphomatoid granulomatosis is an angiocentric and angiodestructive lymphoreticular proliferative and granulomatous disease involving predominantly the lungs and resembling lymphoma. Necrotizing sarcoid granulomatosis is probably a variant of sarcoidosis in which an angiitis is a prominent feature. Because the radiology of these diseases can be similar, their important differences are highlighted. The appearance of multiple nodules, often with cavities, and pleural-based consolidations resembling pulmonary infarcts should suggest pulmonary angiitis and granulomatosis, especially if improvement occurs in one area while disease is progressing elsewhere. Bronchocentric granulomatosis is not a primary vasculitis but is discussed because of its similarity to the other diseases.

Necrotizing sarcoid granulomatosis with suppurative features.Am J Clin Pathol. 1984 Nov;82(5):602-7.

Necrotizing sarcoid granulomatosis has become a well-defined entity within the spectrum of disorders classified as pulmonary granulomatosis with angiitis. It is characterized clinically by disease generally restricted to the chest, steroid sensitivity, and a good prognosis. Pathologically, confluent granulomas, vasculitis, and bland necrosis are seen. The authors report a patient initially presenting with the typical clinical and pathologic features of necrotizing sarcoid granulomatosis who, on subsequent recurrence, demonstrated a suppurative character to the necrosis. This feature has not been reported previously, and its recognition will allow more cases to be diagnosed correctly. Serum angiotensin converting enzyme (ACE) was not elevated, and ACE could not be demonstrated in tissue from the lung biopsy obtained during recurrence of disease. This further suggests significant differences between this entity and sarcoid.

Pulmonary angiitis and granulomatosis revisited.Hum Pathol. 1983 Oct;14(10):868-83.

Re-examination of the pathologic and clinical features of the entities traditionally classified under the heading "pulmonary angiitis and granulomatosis" indicates that there is little advantage in retaining this artificial category and that these entities should be considered variants of diseases to which they are actually related. Wegener's granulomatosis and allergic angiitis and granulomatosis appear to be examples of true systemic vasculitides in which the lung is a predominant but not the only or even the most important site of involvement. Wegener's granulomatosis may manifest with involvement limited to lung, a form that has been called limited Wegener's; however, many or most such cases progress to classic disease involving kidney and often upper respiratory tract. Similarly, Wegener's granulomatosis may present with disease limited initially to the upper respiratory tract (a form of midline granuloma); this process may also spread to involve lung and kidney. It seems unlikely that limited Wegener's is truly a separate disease category. Evaluation of the pathologic and clinical features of necrotizing sarcoid granulomatosis indicate that it very much resembles ordinary sarcoid in most histologic features, in the nature of extrapulmonary involvement, and in its clinical course and that it probably corresponds to the clinical--radiographic entity of nodular sarcoid. Lymphomatoid granulomatosis appears to have little relationship to the other members of the angiitis and granulomatosis group; its behavior and histologic features are those of a lymphoproliferative disorder that in most cases is or becomes histiocytic lymphoma. Some cases of so-called benign lymphocytic angiitis also fall into this category; the remainder appear to represent a variety of completely unrelated pathologic processes. Last, bronchocentric granulomatosis is most commonly one of the histologic manifestations of allergic bronchopulmonary aspergillosis, although it is likely that other agents or processes produce the same histologic pattern. Although the presence of a common set of pathologic features makes the concept of angiitis and granulomatosis attractive from a morphologic point of view, there is minimal clinical similarity among them, and these diseases appear to be totally separate entities.

Necrotizing sarcoid granulomatosis.Chest. 1979 Oct;76(4):406-13.

Twelve cases of necrotizing sarcoid granulomatosis are presented as a retrospective study. The population of patients consisted of ten women and two men, with an average age of 50 years. Nine patients initially had a variety of pulmonary and nonpulmonary complaints, while three were asymptomatic. Chest roentgenograms demonstrated bilateral nodules in seven patients, solitary nodules in four, and a miliary pattern progressing to nodules in one. Enlarged hilar nodes were found in six. Histologically, all biopsies showed a combination of individual granulomas, confluent masses of granulomas which formed the nodular masses seen on the roentgenograms, a variable amount of necrosis of tissue and hyalinization, and a granulomatous vasculitis. Follow-up periods ranged from four months to 11 years. Eleven patients are alive and asymptomatic. The sole death occurred in a patient treated with an immunosuppressive agent (cyclophosphamide). We conclude that the clinical behavior of necrotizing sarcoid granulomatosis is not similar to that of the other angiocentric granulomatoses and that most patients with this disease can be left untreated or be treated with steroids alone. We suggest the possibility that necrotizing sarcoid granulomatosis may be the histologic counterpart of so-called nodular sarcoid.

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