Gastric sarcoidosis with noncaseating granuloma is rare. Although corticosteroid produces a dramatic clinical response, it is unknown whether azathioprine show efficacy in prednisolone-dependent cases. Here, we report a case of gastric sarcoidosis in a 25-year-old man with severe epigastlargia. Gastroendoscopy revealed multiple map-like ulcerations. Histological examination showed multiple noncaseating granulomatous lesions in gastric mucosa, which were incompatible with diagnoses of Crohn's disease or tuberculosis. He was started on prednisolone at 30 mg/d, and his symptoms improved within 7-d. The prednisolone was gradually tapered by 5 mg every 2-wk, but oral azathioprine at 50 mg was added after symptoms recurred at tapered dose of 10 mg. Endoscopy 4-wk later showed healing ulcers, and, lymphocytic infiltration was absent. The efficacy of additional azathioprine in gastric sarcoidosis is not well defined. Here, we report a case of prednisolone-dependent gastric sarcoidosis that improved after additional azathioprine, and also review the literature concerning the treatment, especially for prednisolone-dependent cases.

Sarcoidosis is a multisystemic disorder that is characterized by the formation of noncaseating granulomas. Although sarcoidosis can affect any organ, gastrointestinal tract involvement in sarcoidosis is very rare, and gastric cancer associated with gastric sarcoidosis has hardly been reported. A 64-year-old female with a 10-year history of the medical treatment of gastric sarcoidosis received a routine follow-up gastrointestinal endoscopy and an irregular-shaped, elevated lesion was detected in the gastric corpus. The gastric mucosal surface was nodular and ulcerated throughout the stomach. The gastric lumen was narrow, and the gastric wall was stiff and nondistensible, resembling linitis plastica. The biopsies of the elevated lesion in the gastric corpus revealed well-differentiated adenocarcinoma. An endoscopic ultrasonography was then performed, but it failed to assess precisely the depth of cancer invasion because of sarcoidosis-related gastritis and fibrosis of the gastric wall. The patient underwent a laparoscopic total gastrectomy under the diagnosis of gastric cancer associated with gastric sarcoidosis. Histologic examination of the surgical specimen demonstrated well-differentiated adenocarcinoma in the gastric corpus, and the histologic mapping of cancer cells revealed that the tumor spread within the mucosal layer of the stomach. No lymph node metastasis was found. The patient's postoperative course was uneventful. We experienced a rare case of early gastric cancer associated with gastric sarcoidosis, which identified the troublesome issue that the assessment of depth of cancer invasion is difficult, because patients with longstanding gastric sarcoidosis may involve various degrees of fibrosis of the gastric wall.

This chapter deals with non-vascular intracranial disorders resulting in headache. Headache attributed to high or low cerebrospinal fluid pressure is separated into headache attributed to idiopathic intracranial hypertension (IIH), headache attributed to intracranial hypertension secondary to metabolic, toxic, or hormonal causes, headache attributed to intracranial hypertension secondary to hydrocephalus, post-dural puncture headache, cerebrospinal fluid (CSF) fistula headache, headache attributed to spontaneous (or idiopathic) low CSF pressure. Headache attributed to non-infectious inflammatory disease can be caused by neurosarcoidosis, aseptic (non-infectious) meningitis or lymphocytic hypophysitis. Headache attributed to intracranial neoplasm can be caused by increased intracranial pressure or hydrocephalus caused by neoplasm or attributed directly to neoplasm or carcinomatous meningitis. Other causes of headache include hypothalamic or pituitary hyper- or hyposecretion and intrathecal injection. Headache attributed to epileptic seizure is separated into hemicrania epileptica and post-seizure headache. Finally headache attributed to Chiari malformation type I (CM1) and the syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL) are described.

A 40-year-old man, who had been diagnosed with stage 2 pulmonary sarcoidosis, was referred to our hospital for further evaluation of dyspnea and cardiac function. The echocardiogram displayed thinning of the basal interventricular septum (IVS) and a reduced ejection fraction of 21%. Contrast-enhanced cardiac high resolution MRI (3 tesla) showed patchy subepicardial late gadolinium enhancement in the IVS, and anterior and lateral walls. There was no abnormality in the coronary angiography and the cardiac biopsy showed several small and well-defined noncaseating epithelioid granulomas. The granulomas contained multinucleated giant cells and asteroid bodies (a typical finding of sarcoidosis). Late gadolinium enhancement in high resolution MRI provided information on the pathophysiological condition of cardiac sarcoidosis very clearly, because 3 of 5 samples of endomyocardial biopsy from the septal wall of the right ventricle where late gadolinium enhancement was detected had positive findings for cardiac sarcoidosis (very high rate). These findings indicate that high resolution late gadolinium enhanced MRI might be very useful as a guide for endomyocardial biopsy in patients with cardiac sarcoidosis.

Sarcoidosis and prostate cancer are 2 separate conditions that are more prevalent among the African American population. However, sarcoidosis of the prostate is a very rare clinical entity. Its association with prostate cancer is described in clinical case series. The use of PSA (prostate specific antigen) test for screening prostate cancer may be associated with false-positive results in this patient population. We report a patient who had an elevated PSA but had a biopsy proven to be sarcoidosis of the prostate gland.

Sarcoidosis is a multisystemic disease that usually involves the lungs and lymph nodes, but almost any organ can be involved. Genitourinary involvement with sarcoidosis is extremely rare. We report the case of a 30-year-old African-American male who presented with a right-sided intrascrotal mass and diffuse lymphadenopathy. On further workup, he was found to have sarcoidosis. Two months of corticosteroid treatment resulted in the disappearance of his intrascrotal mass.

Jonathan Hutchinson is a convenient starting point for a glimpse through the history and milestones of sarcoidosis. The influence of various countries is recognized by its pioneers of sarcoidosis. This historical account is brought up-to-date by the word "NOW," implying how are we addressing the enigma that continues to elude us--namely, the cause of sarcoidosis. This review of the past 150 years or so outlines the countries and personalities that have carried the Olympic torch.

Sarcoidosis is a multisystemic disorder characterised by the presence of multiple noncaseating granulomas. Clinically recognisable nervous system involvement occurs in 5-16% of patients with sarcoidosis. However, the incidence of subclinical neurosarcoidosis may be higher. The following article presents a review of the disease, including its pathophysiology, clinical and radiological characteristics and treatment. Neurosarcoidosis should be included in the differential diagnosis of infectious and noninfectious neurological syndromes.

An early diagnosis of cardiac sarcoidosis is important, particularly when considering the need for administering corticosteroid therapy. However, no reports are available on the success rate of diagnosis on the basis of biopsy findings in patients with cardiac sarcoidosis. This study investigated the diagnostic success rate of histologic evaluation of endomyocardial biopsy specimens in patients with this disease.

Right ventricular endomyocardial biopsy was performed in 26 patients in whom cardiac sarcoidosis was strongly suspected according to the Diagnostic Criteria of Sarcoidosis, plus abnormalities on the electrocardiogram, cardiac radionuclide images, or in left ventricular wall motion. A mean of 4.0 sites were sampled per patient. In each case we determined whether a definitive diagnosis of cardiac sarcoidosis could be made histologically. Noncaseating granulomas were found in only 5 (19.2%) of the 26 cases, thus permitting a histologic diagnosis of cardiac sarcoidosis. A histologic diagnosis was made in 4 (36.4%) of 11 patients who exhibited a dilated cardiomyopathy-like clinical picture, in contrast to only 1 (6.7%) of 15 patients in whom conduction disturbances were the major clinical feature and whose left ventricular ejection fraction was within normal limits.

The diagnostic rate achieved with biopsy in cardiac sarcoidosis is low; the patients with sarcoidosis and evidence of significant cardiac involvement should be treated for cardiac sarcoidosis despite negative myocardial biopsies for this disease.

Testicular involvement by sarcoidosis is rare. We report a case of a patient with known sarcoid who had a unilateral testicular nodule with apparent capsular invasion on sonography. The epididymis was normal on both sides. Despite these atypical features, pathology showed the nodule to be a sarcoid granuloma. In patients with sarcoidosis, the differential diagnosis of an intratesticular mass should include testicular involvement by sarcoid.

Sarcoidosis is set within the framework of a large family of granulomatous disorders, so it has many mimics. Granuloma formation is defined with its immunologic basis. The criteria of activity of sarcoidosis are included in the broadly based definition. Future knowledge will derive from basic immunology and molecular biology. Historic aspects are based on pioneers, who have died, and their seminal contributions. Evolution of knowledge derives from information gained at international conferences and from our biennial journal, "Sarcoidosis, Vasculitis and Diffuse Lung Diseases."

In Japan the majority of sarcoidosis-related deaths are due to cardiac sarcoidosis. One of the most common electrocardiographic abnormalities in patients with this disease is atrioventricular block. This study surveyed the incidence of cardiac sarcoidosis in Japanese patients (40 men and 49 women; mean age, 69.1 years) with high-degree atrioventricular block who were admitted to the hospital to receive a permanent pacemaker. We excluded cases in which sarcoidosis had been diagnosed from the involvement of other organs. Patients with the characteristic signs of sarcoidosis underwent echocardiography, radionuclide imaging, and biopsy. Ten cases (11.2%) of cardiac sarcoidosis were diagnosed, most frequently in women aged 40 to 69 years (8 of 25, 32%). Thus the possibility of cardiac sarcoidosis should be carefully considered in middle-aged or elderly Japanese women who show high-degree atrioventricular block.

Sarcoidosis has been recognized for 125 years, but in every generation it makes a chameleon-like change, so updating is needed every so often. In Jonathan Hutchinson's day it was a dermatological curiosity, which gradually evolved into a multisystem disorder associated with bone cysts, and, with the introduction of X-rays, respiratory abnormalities. During the 1960s, epidemiology showed that this disorder, involving all systems of the body, was widely disseminated throughout the world. Each fresh approach, by investigators in different disciplines, has provided a new dimension, particularly in biochemistry and immunology. Each advance in knowledge brings us nearer the enigma which continues to elude us, namely the aetiology of sarcoidosis (Tables 1 and 2). This paper draws attention to the pioneers and personalities of the movement, and records the succession of congresses which have helped to define it worldwide.

Sarcoidosis is seen by the urologist only rarely but it may present a diagnostic and therapeutic dilemma. We describe a rare case of prostatic sarcoidosis. The literature relating to sarcoidosis throughout the genitourinary system is reviewed.

Sarcoidosis is a multisystem disorder that rarely involves the genitourinary tract. To date only 28 cases of histologically proved sarcoidosis involving the epididymis have been described in the literature. Although uncommon, sarcoidosis should be considered in any differential diagnosis of testicular lesions. We present 2 cases of epididymal sarcoidosis. A 27-year-old asymptomatic black man had multiple nontender scrotal nodules on routine physical examination. A 34-year-old black man was initially diagnosed with sarcoidosis by transbronchial biopsy. He received 10 months of prednisone therapy before noticing a mass in the right testicle. Surgical exploration of both patients demonstrated noncaseating granulomatous inflammation consistent with sarcoidosis.

More than a century ago, Jonathan Hutchingson, a surgeon-dermatologist, identified the first case of sarcoidosis at King's College, London. The disease is now known as a commonplace multisystem disorder characterized by the formation of noncaseating granulomata. The diagnosis of sarcoidosis is established by recognizing clinicoradiologic findings and providing histologic evidence of non-caseating granuloma. Serum angiotensin converting enzyme levels are high in about two thirds of the patients and hypercalcemia is a feature in one of every ten victims of sarcoidosis. Immunologic abnormalities include depression of cutaneous delayed-type hypersensitivity, accumulation of T-cells at the site of activity, hyperactive B-cells, and the presence of circulating immune complexes. The course and prognosis of the disease usually correlate with the mode of onset. An acute onset with erythema nodosum indicates a good prognosis and spontaneous resolution; whereas, an insidious onset may be followed by relentless, progressive fibrosis. Mortality and morbidity are caused by pulmonary fibrosis, cardiac arrhythmias, renal failure, neurologic involvement, and blindness. Corticosteroids and chloroquine relieve symptoms and suppress inflammation and granuloma formation.

Two cases of gastric sarcoidosis are reported. Both patients presented with a history suggestive of peptic ulceration and the diagnosis of sarcoidosis was made after operation.

Sarcoidosis is considered rarely in the differential diagnosis of scrotal masses. We report a case of epididymal sarcoid in an otherwise asymptomatic young man. The findings on the chest x-ray initially suggested a metastatic testicular neoplasm. Familiarity with this lesion may avert unnecessary orchiectomy. A rational treatment approach is presented and the literature is reviewed briefly.

In a series of 34 patients with sarcoidosis affecting the upper respiratory tract and nose, 26 had lupus pernio (LP) and 17 had sarcoidosis of the upper respiratory tract (SURT). In nine patients these features coexisted. A patient presenting with SURT carried a 50% risk of developing LP although one feature could be present without the other. Both were disorders of women of the child-bearing years of life. SURT, like LP, was an indicator of chronic fibrotic sarcoidosis, developing insidiously and progressing indolently over the years. It was complicated by ulceration, septal perforation, and LP. Three patients had nasal septal perforations, in two instances following submucous resection. This operation is contraindicated in patients with active sarcoidosis, particularly when granulomas are found on nasal biopsy. The Kveim-Siltzbach skin test was positive in all patients with SURT, making it invaluable in the differential diagnosis of granuloma of the nasal cavity.

Ophthalmologic examination of a 31-year-old black man presenting with an intractable gastric ulcer revealed small conjunctival nodules in the lower cul-desac that were sarcoid granulomas by microscopy. Previous endoscopic examination had revealed polyps in the gastric antrum, and a biopsy and microscopic examination had revealed granulomatous lesions, but a definite diagnosis could not be made until the ophthalmoscopic examination.