Granulomatous inflammation sarcoidosis

Sihcosis, due to the inhalation of crystalline tetrahedral (quartz, tridymite, cristobalite) - but not octahedral (stishovite) - silicon dioxide, is characterised by granulomatous inflammation of the lung. As in various granulomatous diseases, such as other pneumoconioses, sarcoidosis, and tuberculosis, sihcosis is characterised by an early mononuclear cell infiltration of the lung parenchyma followed by resident cell activation and proliferation (Becklake 1994). 

A 56-year-old black woman developed with bilateral orbital swelling in the region of the lacrimal glands after taking interferon-alfa + ribavirin for 4 months for occupationally acquired hepatitis C infection [43 ]. Bilateral lacrimal gland biopsies showed granulomatous inflammation. All other tests were negative for sarcoidosis. 

COPE with Cytokines. 2006. Available at http //www.copewithcytokines.de/. Kunitake R, Kuwano K, Miyazaki H, et al. Apoptosis in the course of granulomatous inflammation in pulmonary sarcoidosis. Eur Respir J 1999 13(6) 1329-1337. Agostini C, Zambello R, Sancetta R, et al. Expression of tumor necrosis factor-receptor superfamily members by lung T lymphocytes in interstitial lung diseases. Am J Respir Crit Care Med 1996 153 1359-1367. 

Sarcoidosis is a rare complication of type 1 interferons (IFNs) (IFN-a or IFN-p) used to treat viral hepatitis and diverse autoimmune and malignant disorders (28,149-154). Type 1 IFNs evoke a Thl lymphocyte bias and amplify granulomatous inflammation (151,152). Most cases resolve following withdrawal of rIFN-a or dose reduction (149,155), but CSs are required in some patients (152,156). 

Cutaneous sarcoidosis lesions are divided into two categories specific and nonspecific. Specific lesions reveal granulomatous inflammation on biopsy. Nonspecific skin findings are reactive inflammatory lesions that do not exhibit sarcoidal granulomas. 

The diagnosis of specific sarcoidosis skin lesions usually requires a confirmatory biopsy. On occasion, a clinical diagnosis of skin sarcoidosis may be made if the lesions are typical (e.g., lupus pernio or lesions present on scar tissue). Sarcoidosis is not the only cause of granulomatous inflammation of the skin, and other potential causes must be carefully excluded. Usually the diagnosis of skin sarcoidosis is not secure without evidence of extracutaneous granulomatous disease. 

The diagnosis of skin sarcoidosis tends to be made rapidly relative to other organ involvement with sarcoidosis because the skin lesions are evident and can be easily biopsied. In a cohort of ACCESS patients, the patients with skin sarcoidosis were diagnosed significantly faster than those with pulmonary sarcoidosis (30). Patents with nonspecific skin lesions such as erythema nodosum do not demonstrate granulomatous inflammation on biopsy. Therefore, skin biopsies should be avoided in these patients as the procedure has no value in their diagnosis. 

Although 25% of patients show evidence of granulomatous inflammation affecting the heart on autopsy (108), only 5% of patients with sarcoidosis have signs or symptoms of cardiac involvement pre-mortem (109). Sarcoidosis also seems to be much more common in the Japanese. In the United States, 13% to 50% of sarcoidosis deaths have been attributed to cardiac involvement (110, 111) compared to 85% in Japan (112,113). ACCESS (an American study with few individuals of Japanese descent) did not demonstrate a predilection for the presence or cardiac sarcoidosis at diagnosis or its development over time on the basis of race, age, or gender (28,114). 

Sarcoidosis may cause peripheral lymphadenopathy (224,225). Isolated granulomatous inflammation in a peripheral lymph node is not diagnostic of sarcoidosis, as in approximately eight percent of cases this may represent a sarcoid-hke reaction from malignancy or inflammatory disease (224). A sarcoid-like reaction may also occur in intrathoracic and intra-abdominal lymph nodes from visceral malignancies (226,227). 

Peritoneal sarcoidosis is rare and can present with ascites (240). The CA-125 serum level may be elevated and therefore, this entity may be confused with ovarian carcinoma (241). Very rarely sarcoidosis may involve the gastrointestinal tract (242). Any portion of the gastrointestinal tract may be involved (242), and care must be taken to distinguish it from the granulomatous inflammation from Crohn s disease (242,243). Rarely sarcoidosis can affect the thyroid gland, presenting as thyroiditis, a nodule, or mass (244,245). 

Patient files (752) from 19 skin cancer cenfres were screened and analysed for rare ipilimumab-associated adverse event. Previously unreported adverse events including drug reaction (rash) with eosinophilia and systemic symptoms, granulomatous inflammation of the CNS and aseptic meningitis were associated with ipilimumab for tiie first time. Other rare, often life-threatening or even fatal, adverse events documented were Guillain-Barri syndrome, neuropathy, myopathy, leucopenia, uveitis and sarcoidosis [159 ]. 

Ocular sarcoidosis has been reported in three patients, in two of whom conventional interferon alfa was used and in one peginterferon alfa-2b all had granulomatous panuveitis with choroidal granulomata of various sizes [42 ]. All had also taken ribavirin. The intraocular inflammation was managed by reducing the dose of interferon and all patients received topical glucocorticoids. 

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