Granulomatous liver disease

Hepatic granulomata are not infrequently encountered in liver biopsy and often are associated with systemic disease. The clinical presentation varies with the particular systemic process. From a biochemical standpoint, the most common abnormalities are elevated serum alkaline phosphatase and γ-glutamyltransferase. The observation of granulomata in a liver biopsy specimen warrants workup to identify a possible cause. Clues may be obtained in the medical history, on physical examination, or with specialized blood testing or radiologic studies. Treatment involves therapy of the underlying cause of the disease associated with the development of the granulomatous hepatitis.     

Granulomatous liver disease

Hepatic granulomas are often encountered on liver biopsy and may represent a primary hepatic process, a manifestation of a systemic illness, or an innocent finding of no clinical relevance. Hepatic granulomas are a unique inflammatory response that may be idiopathic or may be a response to a bacterial, fungal, viral, or parasitic infection; a manifestation of drug-induced liver injury; or a manifestation of underlying malignancy. When granulomas are found on liver biopsy, clinicians must correlate the histologic findings with historical and clinical data to help provide an accurate diagnosis and guide management. Therapy may be warranted, either directly for the granulomatous inflammation of the liver or for a systemic process. For some patients, observation may be the most appropriate form of management.     

Granulomatous liver disease and cholestasis

Development of hepatic granulomas, especially those located within portal tracts, is associated with injury to septal and interlobular bile ducts. Dysfunction of the bile ducts in turn leads to cholestasis,and ongoing damage by the granulomatous inflammation eventually results in ductopenia. A multitude of causes of hepatic granulomas have been described over the last few decades. Diligent and prioritized search for cause of hepatic granulomas should proceed within the environmental and epidemiologic context of a given patient. The therapeutic approaches, including amelioration of the cholestatic syndrome, are often determined by successful identification of specific etiologic factors causing the granulomas in the liver.     

Granulomatous synovitis and Crohn's disease

A case of Crohn's disease of the colon, diagnosed after the discovery of a granulomatous synovitis of the wrist in a 40-year-old woman is described. The clinical and histological features of the articular and extraarticular inflammatory lesions of Crohn's disease are reviewed.     

Granulomatous peritonitis in hydatid disease

We report a rare case of persisting granulomatous inflammation within the peritoneal cavity due to previous leakage from a liver hydatid cyst, with the granulomata bearing hooklets originating from protosolices of Echinococcus granulosus. The patient was treated with a full three-month course of albendazole. We believe this represents a rare case of 'pseudotuberculous peritonitis'.     

Granulomatous arthritis in Crohn's disease

A case is described of non-necrotizing, epithelioid cell granulomatous synovitis of the ankle of a 22-year-old man with Crohn's disease. It appears to be the first published example of granulomatous arthritis in association with Crohn's disease.     

Granulomatous disease in common variable immunodeficiency

Common variable immunodeficiency (CVID) is a primary immunodeficiency of unknown etiology characterized by low levels of immunoglobulin (Ig)G, failure to make specific antibodies in response to infection or immunization, and variable T-cell abnormalities. Multisystemic granulomatous disease is a well-documented complication of CVID, and its presence is associated with significant morbidity and early mortality. Although the lung is the most common organ system affected, granulomas are also found frequently in other organs, including skin, liver, spleen, and the gastrointestinal tract. Autoimmune disorders are common in these patients, and there appears to be an increased propensity to develop lymphoproliferative disorders. Common physical, radiographic, and laboratory abnormalities in patients with CVID and granulomatous disease include splenomegaly, hilar and mediastinal lymphadenopathy with ground glass or nodular opacities in the lung parenchyma, and reduced T-cell numbers and function. The etiology of granulomatous disease in patients with CVID is unknown, and optimal treatment of granulomatous disease in CVID remains to be established. Further studies are needed to elucidate the underlying etiology of granulomatous lymphoproliferative interstitial lung disease and to delineate appropriate treatments for this disease.     

Granulomatous disease in selective IgA deficiency

Although common variable immunodeficiency (CVID) is sometimes associated with sarcoidosis/granulomatous disease, there have only been isolated reports of selective immunoglobulin A (IgA) deficiency and granulomatous disease. We present a patient with IgA deficiency who developed Heerfordt syndrome, a variant of neurosarcoidosis. This specific entity has not been previously reported to occur in IgA deficiency. Our case expands the reported associations of IgA deficiency and provides another example to the paucity of reported cases of sarcoidosis occurring in patients with IgA deficiency. As CVID and IgA deficiency have common underlying genetic factors, such an association is biologically plausible.     

Granulomatous disease in common variable immunodeficiency

PURPOSE: Common variable immunodeficiency (CVID), defined by defective production of immunoglobulins, is the most common primary immunodeficiency in adulthood requiring a medical follow-up. Repeated bacterial infections and/or autoimmune manifestations and/or benign lymphoproliferation (including follicular hyperplasia and/or granulomatous disease) are the hallmark of the disease. This review aims at describing recent advances in the understanding and treatment of granulomatous disease in CVID. CURRENT KNOWLEDGE AND KEY POINTS: Clinical features of granulomatous disease in CVID can mimic sarcoidosis, remarkable by the low levels of circulating immunoglobulins. Granulomas may be found in several organs in a single patient, and the main features are pulmonary, lymphoid, cutaneous, hepatic or splenic. The features of CVID is remarkable by the high frequency of autoimmune diseases complicating the immunodeficiency. Some immunological abnormalities have been described in such patients, including lymphopenia, decreased T-cells proliferations to mitogens and antigens. Rare polymorphisms in the gene encoding TNFalpha (Tumor Necrosis Factor) have been identified in CVID patients with granulomatous disease. FUTURE PROSPECTS AND PROJECTS: The evolution of the disease is severe, particularly when the lung is involved. Treatment consists in immunoglobulins substitution, immunosuppressive agents (corticosteroids, cyclophosphamide) and anti-TNFalpha antibodies. These treatments are difficult to manage in such immunocompromised patients.     

Granulomatous osteonecrosis in Crohn's disease.

A 25-year-old white woman was diagnosed with Crohn's disease involving the small and large intestines. She had a complex clinical course that required treatment with multiple pharmacological agents, including intravenous, oral and rectal corticosteroids. She also received parenteral nutrition with lipid emulsions. Finally, repeated intestinal resections and drainage of perianal abscesses were required. Her disease was complicated by gallstones, urolithiasis and hip pain. After osteonecrosis was diagnosed, joint replacements were performed. Review of the pathological sections from the resected hip, however, resulted in detection of granulomatous inflammation with multinucleated giant cells - the histological 'footprint' of Crohn's disease in the gastrointestinal tract. Because prior specialized perfusion fixation pathological studies of the intestine in Crohn's disease have shown that granulomas are located in the walls of blood vessels, a possible mechanism for the pathogenesis of osteonecrosis in Crohn's disease is chronic microvascular ischemia of bone.     

Granulomatous cheilitis and Crohn's disease.

Granulomatous cheilitis is characterized by recurrent swelling of the labial tissues and granulomatous histology. Granulomatous cheilitis has been recognized as an early manifestation of Crohn's disease. It may follow, coincide with or precede the onset of Crohn's disease. The first case presented involved an extraintestinal manifestation of Crohn's disease, and the second case presented is of development of granulomatous cheilitis a year before the onset of symptomatic Crohn's disease. Although chronic granulomatous cheilitis is a very rare disorder, once it is diagnosed, the patient should be followed up carefully. These patients should be investigated for asymptomatic Crohn's disease either when the diagnosis of granulomatous cheilitis is confirmed or when gastrointestinal symptoms develop.     

Granulomatous hypophysitis associated with Takayasu's disease

We report a case of Takayasu's disease, presenting with symptoms of fever, anaemia, elevated erythrocyte sedimentation rate, anterior pituitary failure and mild diabetes insipidus. A pituitary mass with suprasellar extension mimicking a pituitary adenoma was found, and histological examination revealed granulomatous hypophysitis. The diagnosis of Takayasu's disease was established after the development of a multiple arterial occlusive disease. We suggest that Takayasu's disease should be considered in the differential diagnosis of granulomatous hypophysitis of unknown origin.     

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肺部多发结节性病变是指在肺部影像学和(或)病理组织学上以多个结节样病灶为主要表现的疾病群,可由许多病种引起。肉芽肿是病理组织学上慢性炎症的主要类型,是致炎因子长期持续性刺激所致的迟发型变态反应,在炎症局部形成以巨噬细胞增生为主、境界清楚的结节状病灶[1-2]。1肉芽肿性肺疾病的常见病因和分类根据病因可将肉芽肿性肺疾病分为感染性和非感染性两大类:(1)感染性肉芽肿:包括各种病原体的感染,主要有:①细菌感染:结核、麻风、梅毒等。②真菌感染:隐球菌、曲霉菌、组织胞浆菌、球孢子菌等。③寄生虫感染:肺吸虫、血吸虫、弓形虫等。(…     

Granulomatous musculoskeletal disease: sarcoidosis versus tuberculosis

A patient with systemic sarcoidosis insidiously developed tuberculous arthritis. The concurrence of these diagnoses in any individual patient is rare. Musculoskeletal features of sarcoidosis and tuberculosis are reviewed, and the importance of obtaining appropriate cultures to differentiate these granulomatous disorders is illustrated.     

Granulomatous lymphocytic interstitial lung disease in infancy

The authors report a case involving a child with chronic respiratory symptoms, who did not respond to conventional treatment. Low serum immunoglobin levels and pathological findings on lung biopsy revealed an unusual diagnosis for his age group. A specific treatment led to clinical improvement.     

Granulomatous bowel disease presenting as a bladder tumor

Summary A case of Crohn's disease of the terminal ileum and rectosigmoid, with extension to the bladder by contiguity from the former, which was thought to be a vesical neoplasm, is presented in detail. In spite of the frequency of ileal involvement by granulomatous disease, bladder extension is infrequent. Evaluation of the vesical lesion alone may present serious difficulties in diagnosis. The presence of known granulomatous disease of the bowel suggests the possibility of secondary involvement of the bladder, but this cannot be considered to be conclusive without further evaluation.     

Granulomatous chest disease following intravesical bacillus Calmette-Guerin immunotherapy

Bacillus Calmette-Guerin (BCG) is well accepted as an intravesicular method of treatment of bladder cancer. Although well tolerated in the majority of patients, adverse local and systemic adverse effects of this therapy have been described. In particular, lung disease complicating intravesicular use of BCG has been reported, producing 5 recognized forms of chest disease: 1) allergic interstitial lung disease, 2) miliary infection, 3) empyema, 4) diffuse alveolar damage, and 5) localized consolidative or cavitary lung disease. We report an asymptomatic form of pulmonary disease complicating intravesical BCG therapy, that of pulmonary granulomas with mediastinal and hilar lymph node enlargement.