Bullous sarcoidosis: a report of three cases

Three cases of pulmonary sarcoidosis presented as bullous emphysema with severe airflow obstruction, and the diagnosis of sarcoidosis was unsuspected for at least 2 years. Potential mechanisms of bullous emphysema from sarcoidosis are discussed. The physician should suspect sarcoidosis as the cause of bullous emphysema when young patients who have smoked relatively few pack-years present with emphysema or severe airflow obstruction. Additional clues are the presence of mediastinal adenopathy on a chest radiograph or a CT scan and a history consistent with extrapulmonary sarcoidosis.     

The ex vivo effect of the herbal medicine sho-saiko-to on histamine release from rat mast cells

OBJECTIVE: This report describes subcutaneous sarcoidosis, focusing on the radiological and magnetic resonance (MR) features of the disease. DESIGN AND PATIENTS: The cases of four patients (one male and three female, age range 36-75 years) who had subcutaneous sarcoidosis with no other organs affected were reviewed. Lesions were nodular in two cases, and in the other two were diffuse. RESULTS: Computed tomography (CT) demonstrated a well-defined, homogeneous, and enhanced lesion in the nodular cases. However, in the diffuse cases, CT showed a heterogeneous, honeycomb-like appearance and little enhancement. Angiography showed a fine stain in the arterial phase. MR imaging of the nodular lesions was homogeneous with a signal intensity similar to muscle on T1-weighted images but heterogeneous with a higher signal than muscle on T2-weighted images. Diffuse lesions showed a striped or mesh pattern with intermediate signal intensity on both T1- and T2-weighted images. Contrast-enhanced MR images showed slight enhancement. CONCLUSIONS: Subcutaneous sarcoidosis should be considered in the differential diagnosis when a patient presents with the radiological and MR features described.     

Nodular sarcoidosis of the liver and spleen: analysis of 32 cases

PURPOSE: To describe the computed tomographic (CT) appearance of nodular hepatosplenic sarcoidosis and its association with stage with chest radiography and clinical status. MATERIALS AND METHODS: Thirty-two patients (21 women, 11 men; aged 25-68 years) with nodular hepatosplenic sarcoidosis were evaluated. CT findings were described along with chest radiographic stage, clinical status, and level of angiotensin-converting enzyme (ACE). RESULTS: Nodules were small, multiple, and of low attenuation. Organomegaly was common. Abdominal adenopathy was present in 76% of the patients. Chest radiographs were normal in 25%; 61% had stage 1 or 2 radiographs. Abdominal or systemic symptoms were present in 66%. ACE level was elevated in 10 (91%) of 11 patients tested. No change in chest radiographic stage was noted in 74% of patients with follow-up radiographs. CONCLUSION: Nodular hepatosplenic sarcoidosis is associated with organomegaly, adenopathy, and symptoms. Nodules were not associated with advanced lung disease and did not herald a change in chest radiographic stage. An elevated ACE level may be helpful in diagnosis.     

Digital color imaging colposcopy: a matter of choice

PURPOSE: The objective of this study was to correlate the findings of sarcoidosis on high resolution CT (HRCT) with indexes of disease activity as measured with 67Ga scan, bronchoalveolar lavage (BAL), and serum angiotensin-converting enzyme (SACE) assay. METHOD: Twenty-nine patients with proven sarcoidosis underwent HRCT scan, 67Ga scan, BAL, and SACE assay within a 1 month period. The extent of parenchymal involvement by nodules, consolidation, ground-glass attenuation, and linear opacities was quantified to the nearest 10% of surface area affected on the CT examination. Whole-lung gallium uptake was quantified and the percentage of BAL-recovered lymphocytes (BAL-%LC) and SACE levels obtained by chart review. CT scores of disease extent were correlated with measured indexes of activity using the Spearman rank correlation coefficient. RESULTS: The mean extent of nodules, consolidation, ground-glass attenuation, and linear opacities on HRCT images was 15.1 +/- 16.6, 1.6 +/- 4.0, 17.5 +/- 25.4, and 7.6 +/- 9.6%, respectively. The extent of nodules and consolidation correlated with the intensity of lung gallium uptake (r = 0.46, p < 0.02), BAL-%LC (r = 0.50, p < 0.01), and SACE levels (r = 0.38, p < 0.05). No significant correlation was found between extent of ground-glass attenuation or linear opacities with any indexes of disease activity. CONCLUSION: On HRCT scan, nodules and consolidation in sarcoidosis reflect disease activity as measured by 67Ga scan, BAL, and SACE assay.     

Extraocular muscle involvement in sarcoidosis

BACKGROUND: Sarcoidosis is a granulomatous inflammatory disease that may have a variety of ocular and orbital manifestations. The most common ocular manifestation is uveitis, and the most common orbital manifestation is dacryoadenitis. Extraocular muscle involvement in sarcoidosis has rarely been reported. The authors report a case of sarcoidosis involving the extraocular muscles of a 15-year-old boy with bilateral, painful, external ophthalmoplegia and enlargement of all extraocular muscles on computed tomography (CT) scan. RESULTS: Lateral rectus muscle biopsy and transbronchial lung biopsy showed noncaseating granulomas characteristic of sarcoidosis. Cultures and serologic studies excluded fungal and mycobacterial diseases. Treatment with oral corticosteroids improved symptoms and signs. CONCLUSIONS: The authors report the first case of sarcoidosis in a patient with symptomatic extraocular muscle involvement, and only the third case in which extraocular muscle involvement has been shown histologically.     

Pulmonary sarcoidosis: morphologic associations of airflow obstruction at thin-section CT

PURPOSE: To identify relationships between the obstructive defects of pulmonary sarcoidosis and the computed tomographic (CT) patterns of disease. MATERIAL AND METHODS: CT scans obtained in 45 patients were scored semiquantitatively for extent of five CT patterns, and the functional importance of each pattern was evaluated. RESULTS: The most prevalent CT patterns were decreased attenuation (n = 40), a reticular pattern (n = 37), and a nodular pattern (n = 36). At univariate and multivariate analyses, a reticular pattern was the main determinant of functional impairment, particularly airflow obstruction. The extent of a reticular pattern was independently associated with airflow obstruction, as shown by the inverse relationships with the forced expiratory volume in 1 second (FEV1) (P < .001), FEV1-forced vital capacity ratio (P < .01), maximum expiratory flow at 25% above residual volume (P < .001), and maximum expiratory flow at 50% above residual volume (P < .001) and the positive relationship with the residual volume-total lung capacity ratio (P < .001). CONCLUSION: In sarcoidosis, CT features compatible with small airways disease are common but contribute little to airflow obstruction, particularly in more advanced disease, which is characterized by an extensive reticular pattern. A reticular pattern at CT is the major morphologic association of airflow obstruction.     

CT evaluation of the anterior mediastinum: spectrum of disease

Computed tomography (CT) is the study of choice for evaluating disease in the anterior mediastinum. Mediastinal CT is usually performed with intravenously administered contrast material, and spiral CT is the preferred technique for evaluating a mediastinal mass. CT demonstrates thymic hyperplasia and thymic cysts and can help differentiate thymoma and thymic Hodgkin lymphoma. It is also useful in staging Hodgkin lymphoma and non-Hodgkin lymphoma. In thyroid malignancy, CT can depict mediastinal extension and lymphadenopathy; it also allows detection of goiter and ectopic parathyroid glands. Germ cell tumors such as teratoma and seminoma have characteristic appearances at CT. CT can also demonstrate miscellaneous mediastinal masses, such as lymphangioma, hematoma, those due to fibrosing mediastinitis, and pericardial cysts. Adenopathy due to tuberculosis or sarcoidosis is evident at CT, as is osteomyelitis due to a postsurgical abscess. Finally, CT features can suggest the pathologic origin of metastasis in the anterior mediastinum.     

Nonpainful swelling of the palate and loosening of the maxillary incisors

Bone lesions in the maxilla have rarely been described in relation to sarcoidosis. This patient exhibited many of the classic signs of sarcoidosis, including lack of symptoms at the time of presentation, which resulted in the diagnosis of sarcoidosis being made serendipitously. The presence of musculoskeletal lesions is often regarded as a poor prognostic sign and, in general, sarcoidosis follows a more virulent course in black patients. Sadly, the course of the disease in this patient continues to march onward despite a 1-year therapeutic trial of corticosteroids.     

Association of sarcoidosis, low-grade B-lymphoma and epidermoid carcinoma

We report on a case of the so-called sarcoidosis-lymphoma syndrome in a 65-years-old man diagnosed as having sarcoidosis and, four years later, neurosarcoidosis. The diagnoses of epidermoid carcinoma of the skin and of stage IV monocytoid, small cell lymphocytic lymphoma were made five and seven years, respectively, after the initial diagnosis of sarcoidosis. It has been suggested that the increased mitotic activity of lymphocytes observed in sarcoidosis, favours their malignant transformation. Hypothetically, sarcoidosis might also influence the development of epidermoid carcinomas by depletion of circulating T4 lymphocytes and decreased resistance to oncogenic viruses that could lead to decreased tumour rejection in the epithelia exposed to carcinogenic stimuli.     

Detection and quantification of protein-losing enteropathy with indium-111 transferrin

BACKGROUND: Acid fast cell wall deficient forms (CWDF) of bacteria have been grown from blood, bronchial washings, and ocular anterior chamber fluid from patients with sarcoidosis. A monoclonal antibody raised against Mycobacterium tuberculosis whole cell antigen (H37RV) was used to characterise further CWDF grown from the blood of patients with sarcoidosis. METHODS: Blood from 20 patients with active sarcoidosis and from 20 controls was cultured using methods favourable for the growth of CWDF. Isolates were further characterised by indirect fluorescent antibody analysis using a monoclonal antibody highly reactive with M tuberculosis. RESULTS: CWDF were grown from the blood of 19 of 20 subjects with sarcoidosis. All isolates stained positively with the monoclonal antibody and with a modified Kinyoun stain. No organisms were grown from the blood of controls. CONCLUSIONS: These data demonstrate that CWDF can be grown from the blood of nearly all patients with active sarcoidosis. The results confirm that the organisms are mycobacterial in origin and are similar, if not identical, to M tuberculosis. Their role in the pathogenesis of sarcoidosis is unknown.     

Retinoic acid and its receptors

We report on a patient presenting with cutaneous leucocytoclastic vasculitis in association with sarcoidosis. Detailed investigations did not show evidence of infectious or neoplastic diseases. Vasculitis was therefore considered to be secondary to sarcoidosis. A review of 4 other previously reported cases reveals a common presentation with the association of vasculitic skin lesions with deep cutaneous nodules, mediastinal lymph nodes and arthritis. The role of antibody response in leucocytoclastic vasculitis is discussed.     

Bilateral 3rd cranial nerve palsy disclosing oligodendroglioma

The patient was a 30 year-old man. He had no previous history. For several months he experienced a slowly progressive horizontal diplopia which was the expression of a bilateral third cranial nerve palsy with an intact intrinsic component. Muscular or neuro-muscular pathology as myasthenia was initially suspected but not confirmed. CT scan and MRI revealed an atypical left temporo-insular lesion which led us to discuss a chronic inflammatory pathology as sarcoidosis or a tumoral process. Finally cerebral biopsy showed a high grade oligodendroglioma. Symptomatology was attributed to infiltration of the peduncles from this tumor. Such a case has never been seen before. Early neuroradiological explorations would be useful in case of clinical suspicion of cranial nerve palsy.     

Brain tumors in patients with Fanconi''s anemia

OBJECTIVES: To study whether an association between polyglandular autoimmune (PGA) syndrome type III [including autoimmune thyroid disease (ATD) and insulin-dependent diabetes mellitus (IDDM)], coeliac disease and sarcoidosis, exists. DESIGN: In patients with documented sarcoidosis, the presence of the disease constellation of ATD, IDDM and coeliac disease was examined. SETTING: The patients were recruited at the Department of Pulmonary Medicine, and the study was conducted at the Department of Endocrinology, Lund University Clinics, General Hospital, Malm?, Sweden. SUBJECTS: Of all patients (n = 89) with documented sarcoidosis attending the Department of Pulmonary Medicine between January 1980 and December 1991, 78 patients (44 males, 34 females: median age at the time of the study 48 years, range 22-81 years: median observation time since the diagnosis of sarcoidosis 120 months, range 1-468 months) were examined in the present study. RESULTS: Amongst the 78 patients with documented sarcoidosis, one female patient was found with PGA syndrome type III, coeliac disease and sarcoidosis. CONCLUSIONS: This present patient further indicates the existence of an association between polyglandular autoimmune (PGA) syndrome type III, coeliac disease and sarcoidosis. To determine whether this disease constellation might constitute a new syndrome, further studies on larger groups of patients with sarcoidosis are demanded.     

Sarcoidosis with nasal obstruction and septal perforation

Although sarcoidosis is predominantly a pulmonary disorder, it often produces disease in the head and neck. For this reason, otolaryngologists should be comfortable in evaluating and treating the disease. The diagnosis of sarcoidosis often can be made with a biopsy of conjunctival or oral mucosa, even when disease manifestations are not apparent in those parts of the body. In a case of sarcoidosis involving the nose, a 54-year-old black man had a proliferation of small lumps on the tip of his nose. For many years, he had had daily epistaxis, anosmia and severe difficulty breathing through this nose. The bridge of his nose was deformed, and he had a large anterior septal perforation. Physical examination revealed palpable bilateral cervical lymphadenopathy. Laboratory test results were, for the most part, normal; however, fine-needle aspiration of one of the cervical lymph nodes showed multiple epithelioid granulomas with interspersed lymphocyte, and an incisional biopsy of the nasal tissue was highly suggestive of sarcoidosis. Unenhanced computed tomography also revealed widespread nasal inflammation and an abnormal soft tissue mass anterior to the maxilla consistent with chronic osteomyelitis. The patient's condition dramatically improved following treatment with oral prednisone; however, he considered undergoing reconstructive surgery for the noticeable nasal septal deformity.     

Dermatomyositis complicated by sarcoidosis

We encountered a patient with dermatomyositis complicated by sarcoidosis. A 57-year-old woman was admitted to our hospital because of fever dry cough, and myalgias. There were reticular shadows on her chest X-ray film. Although the typical skin rash and myositis suggested the diagnosis of dermatomyositis biopsy specimens from a salivary gland, muscle, and lung revealed noncaseating granulomas as well. Uveitis was also noted. These findings suggested the coexistence of sarcoidosis with dermatomyositis. Examination of the lung-biopsy specimens showed interstitial pneumonia compatible with dermatomyositis, except for the granuloma. The typical rash of dermatomyositis and pathological findings of the lung specimen were inconsistent with sarcoidosis. Therefore we concluded that this patient had both dermatomyositis and sarcoidosis. This case sheds new light on the importance of pathological examinations.     

Use of somatostatin analogue scintigraphy in the localization of recurrent medullary thyroid carcinoma

Detection of recurrence of medullary thyroid carcinoma (MTC) remains a diagnostic problem. Increased serum tumour marker levels frequently indicate recurrence while conventional imaging techniques (CIT) are non-diagnostic. In this study, we performed indium-111 octreotide scintigraphy and CIT in a series of 20 patients with MTC presenting with elevated serum tumour markers after surgery. 111In-octreotide whole-body studies detected 15 pathological uptake foci in 11 of the 20 patients studied and CIT detected 17 lesions in 11 of the 20 patients. Ten patients underwent reoperation, five of them with positive 111In-octreotide scintigraphy and CIT and two with positive isotopic exploration and negative CIT. Surgical findings demonstrated that the results of isotopic study and CIT had been false-positive for MTC in one case (sarcoidosis). The six patients with true-positive 111In-octreotide studies had significantly higher basal calcitonin (CT) and carcinoembryonic antigen (CEA) levels than the patients with negative isotopic studies. The expression of somatostatin receptor (SSTR) subtypes by PC-PCR could be investigated in four cases with a positive isotopic study. Among the three cases with a true-positive study, SSTR2, the SSTR subtype that preferentially binds to the somatostatin analogue octreotide, was detected in two, SSTR5 was demonstrated in the three, and SSTR3 was detected in one. No subtype of SSTR was detected in the case with a final diagnosis of sarcoidosis. We conclude that 111In-octreotide has limited sensitivity in detecting recurrence in patients with MTC, although its sensitivity may improve with high serum CT levels. This radionuclide imaging technique should be employed when conventional imaging techniques are negative or inconclusive or when the presence of somatostatin receptors may provide the basis for treatment with somatostatin analogues.     

Primary lung cancer associated with diffuse granulomatous lesions in the pulmonary parenchyma

A 60-year-old man was admitted to our hospital for productive cough. Chest roentgenography and CT scan disclosed a left hilar tumor invading the mediastinum, with mediastinal lymphadenopathy and diffuse micronodular shadows in both lung fields. A biopsied sample of the tumor revealed squamous cell carcinoma, while noncaseating epithelioid cell granulomas were observed in the samples obtained by transbronchial lung biopsy. The granulomas in the pulmonary parenchyma were determined to be sarcoid reactions secondary to lung cancer, since there was no evidence of sarcoidosis. Combination chemotherapy was effective for the tumor, and the granulomas disappeared after completion of the chemotherapy. These findings suggest the presence of a relationship between sarcoid reactions and lung cancer in this case.     

Sarcoidosis in a patient with autoimmune hemolytic anemia

A 65-year-old woman was admitted to our hospital because of severe anemia. A skin biopsy was done in January 1994 and sarcoidosis was diagnosed. Diffuse reticular shadows were seen in both lung fields on a chest X-ray film and mediastinal lymph node swelling was seen on a chest CT scan. She was followed as an outpatient and was not treated. She suddenly experienced vertigo and general fatigue in March 1995. Laboratory findings on admission were as follows: Hb 6.2 g/dl, MCV 115.9 fl, Ret 198%, LDH 732 IU/L, I-Bil 1.9 mg/dl, and Coombs' test was positive. Autoimmune hemolytic anemia was diagnosed, and she was treated with prednisolone (1 mg/kg). As of the time of this writing, she has no relapse of hemolytic anemia though prednisolone was discontinued 6 months ago.     

Detection of antibodies to Borrelia species among patients with confirmed sarcoidosis in a region where Lyme disease is nonendemic

BACKGROUND: Lyme disease is a multisystemic disorder caused by the spirochete Borrelia burgdorferi, while sarcoidosis is a multisystemic granulomatous disease of unknown etiology. The purpose of this study was to evaluate the relationship between Lyme disease and sarcoidosis. METHODS: We examined the seroprevalence of antibody to Borellia species in patients with sarcoidosis. We performed the enzyme-linked immunosorbent assay, using three Japanese Borrelia species in addition to B. burgdorferi, and dotblot analysis using purified Borrelia-specific proteins in 38 patients with histopathologically confirmed sarcoidosis and 80 healthy controls. RESULTS: Two patients (5.3%) were positive for antibodies to Borrelia species according to one or both assays, and one (1.2%) healthy control was positive. In both patients it was suspected that Borrelia infection had developed prior to the development of sarcoidosis. CONCLUSION: Borrelia species were thought not to be responsible for the development of sarcoidosis in a nonendemic region in Japan. Since clinical manifestations of Lyme disease share certain similarities with those seen in sarcoidosis, ophthalmologists should be aware of the need to differentiate between the two diseases and the need for prompt treatment in each case.     

Coincidence of sarcoidosis and amyloidosis

Amyloidosis was diagnosed in a patient with sarcoidosis. There was no evidence of any other disease known to be associated with amyloidosis. The joint occurrence of amyloidosis and sarcoidosis appears to be extremely rare, and to our knowledge, this is the second such patient to be reported. Whether the association of the two diseases in this patient represents a causal relationship remains an open question.