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At least 19 recordsLinked to original sources
PubMed2000

Corticosteroids for pulmonary sarcoidosis.

BACKGROUND: Pulmonary sarcoidosis is a common condition with an unpredictable course. Oral or inhaled steroids are widely used in its treatment, but there is no consensus about when and in whom therapy should be initiated, what dose should be given and for how long. Corticosteroids given for several months have deleterious side-effects so it is important to know whether they have any maintained benefit in pulmonary sarcoidosis. OBJECTIVES: To determine the randomised controlled trial (RCT) evidence for the benefit of corticosteroids (oral or inhaled) in the treatment of pulmonary sarcoidosis. SEARCH STRATEGY: The Cochrane Airways Group interstitial lung disease RCT register was searched using the terms: sarcoidosis and (steroid* OR corticosteroid* OR prednisolone OR prednisone OR beclomethasone OR budesonide OR fluticasone). Bibliographies of retrieved RCTs and reviews were searched for additional RCTs. Pharmaceutical companies and authors of identified RCTs were contacted for other published and unpublished studies. SELECTION CRITERIA: Two reviewers independently assessed full text articles for inclusion based upon the following criteria: the study had to be a RCT or controlled clinical trial in adults with histological evidence of pulmonary sarcoidosis. DATA COLLECTION AND ANALYSIS: Study quality was assessed and data extracted independently by two reviewers. Outcomes were analysed as continuous and dichotomous outcomes, using standard statistical techniques. MAIN RESULTS: Eight RCTs were identified, two had insufficient data for any analysis. There were 338 patients in the four usable trials of oral steroids, and 66 patients in two trials of inhaled steroids. The oral steroid dose was equivalent to prednisolone 15-40 mg/day. The inhaled steroid was budesonide 0.8 - 1.2 mg/day. Outcomes were symptoms, chest X-ray (CXR) changes, lung function and global scores (a combination of all three outcomes). Oral steroids improved the CXR over 6-24 months. One study showed no improvement in lung function, in another there was an improvement in diffusing capacity in the treated group. Global scores improved in patients with stage 2 and 3 disease but not with stage 1 disease. There were no data on side-effects. Inhaled steroids had no effect on CXR. In one study diffusing capacity improved. In another, symptoms improved at the end of six months of treatment. REVIEWER'S CONCLUSIONS: Oral steroids improved the chest X-ray and a global score of CXR, symptoms and spirometry over 6-24 months. There is little evidence of an improvement in lung function. There are no data beyond 2 years to indicate whether oral steroids have any modifying effect on long-term disease progression. Oral steroids are indicated for patients with Stage 2 and 3 disease with moderate - severe or progressive symptoms or CXR changes. The available data provide no guidance for the management of this disease after 2 years. Short term (less than six months) of inhaled steroids may improved symptoms, perhaps in patients who mainly have cough.

Administration, Inhalation↗
PubMed2003

Papular sarcoidosis of the knees: a clue for the diagnosis of erythema nodosum-associated sarcoidosis.

BACKGROUND: In recent years we have systematically explored the skin whenever sarcoidosis was suggested and we have observed with increasing frequency the presence of granulomatous cutaneous lesions of sarcoidosis involving the knees. OBJECTIVE: We sought to evaluate the specific cutaneous lesions of sarcoidosis involving the knees. METHODS: A total of 18 patients with biopsy-proven specific cutaneous sarcoidosis predominantly involving the knees were included in the study. Biopsy specimens were evaluated under polarized light. RESULTS: Of these cases, 4 corresponded to scar-sarcoidosis, 1 to plaque-type sarcoidosis, and 13 were an admixture of papules and minute scars frequently associated with erythema nodosum (papular sarcoidosis of the knees). Foreign particles were observed in 10 of 13 patients with papular sarcoidosis. CONCLUSION: Papular sarcoidosis of the knees can be considered a frequent form of cutaneous sarcoidosis, mainly observed in acute forms of the disease, and frequently associated with erythema nodosum.

Erythema Nodosum↗
PubMed1999

Defining organ involvement in sarcoidosis: the ACCESS proposed instrument. ACCESS Research Group. A Case Control Etiologic Study of Sarcoidosis.

BACKGROUND: Sarcoidosis is a multiorgan granulomatous disease of unknown cause. Lack of an objective system for assessment of sarcoidosis to evaluate disease course and effectiveness of therapy is a major problem. METHODS: The sarcoidosis assessment instrument was developed by the Steering Committee of A Case Control Etiologic Study of Sarcoidosis (ACCESS) which included investigators at the ten ACCESS Clinical Centers, the Clinical Coordinating Center, and representatives of the National Heart, Blood, and Lung Institute. This system was developed to assess sarcoidosis organ involvement in ACCESS patients who would be followed over a two-year period. The system represents a consensus of opinions of members of the Steering Committee based on review of their experience and the medical literature. RESULTS: Criteria for involvement in patients with biopsy-confirmed sarcoidosis are presented for organs and systems that are commonly involved (lung, skin, eyes, liver, calcium metabolism), unusual but clinically important (nervous system, kidney, heart) and other sites (non-thoracic lymph nodes, bone marrow, spleen, bone/joint, ear/nose/throat, parotid/salivary glands, muscles). CONCLUSION: The proposed instrument is partially subjective in that it depends upon the clinician's diligence in pursuing evidence for sarcoidosis involvement of various organs. It is hoped that this instrument will lead to increased standardization in the definition of sarcoidosis organ involvement to help clinicians and researchers better characterize patients with sarcoidosis.

Bone Diseases↗
PubMed1998

Sarcoidosis and cancer revisited: a long-term follow-up study of 555 Danish sarcoidosis patients.

For more than 20 yrs it has been debated whether the systemic disease sarcoidosis predisposes to malignant neoplasms. The aim of this study was to examine the occurrence of cancer in Danish sarcoidosis patients observed for 9-30 yrs. The clinical data of 555 consecutive sarcoidosis patients were linked with the nationwide Danish Cancer Registry in a database, comparing the results with the expected incidence in the Danish population, adjusted for sex, age and calendar time. The sarcoidosis patients were diagnosed in two areas during the periods of 1960-1971 and 1970-1981, respectively, and followed until December 31, 1991. No excess of cancer was observed in sarcoidosis patients. A total number of 48 patients were observed with cancer (20 males and 28 females). Cancer occurred 1-29 yrs after sarcoidosis diagnosis (median 14 yrs) The observed versus expected (O/E) ratio was 1.16 (95% confidence interval (CI) 0.75-1.79) in males and 1.28 (95% CI 0.88-1.86) in females. No increased occurrence of lung cancer or malignant lymphoma (O/E ratios 0.23, 95% CI 0.00-1.25 and 1.25, 95% CI 0.02-6.95, respectively) was found. Neither age at diagnosis of sarcoidosis nor clinical sarcoidosis features were indicators of later occurrence of malignancy. The study could not confirm previous reports of an increased occurrence of malignant neoplasms in Danish sarcoidosis patients.

Adolescent↗
PubMed1992

[Non-Hodgkin's lymphoma in a patient with sarcoidosis (the sarcoidosis-lymphoma syndrome)].

A-31-year-old man with right cervical and supraclavicular lymphadenopathy was admitted in March, 1991. He was diagnosed as having muscular sarcoidosis at the age 8 year, and was treated with corticosteroids. Since age 18, his skin was erythematous and ulcerous, and later his skin became gradually atrophic. Lymph node biopsy revealed diffused large cell non-Hodgkin's lymphoma. Lymphoma cells showed TCR-beta gene rearrangement by Southern blot hybridization. His lymphoma was refractory to CHOP and CHOP-Bleo regimens. Complete remission was achieved with cisplatin and etoposide. However, early relapse occurred, and he died of pulmonary hemorrhage 4 months after the diagnosis of non-Hodgkin's T-cell lymphoma. The so called "sarcoidosis-lymphoma syndrome" is uncommon in Japan. In 9 of 10 cases previously reported, malignant lymphoma occurred during the course of sarcoidosis. Most of the sarcoidosis cases were chronic active type, and required systemic administration of corticosteroids. Hodgkin's disease coexistent with sarcoidosis as reported in other countries, was not found in Japan. These findings suggest that the low incidence of sarcoidosis-lymphoma syndrome in our country is due to the relative rareness of Hodgkin's disease. The sarcoidosis-lymphoma syndrome possibly appears as a consequence of immunological abnormalities observed in sarcoidosis.

Adult↗
PubMed2001

Familial aggregation of sarcoidosis. A case-control etiologic study of sarcoidosis (ACCESS).

Despite reports of familial clustering of sarcoidosis, little empirical evidence exists that disease risk in family members of sarcoidosis cases is greater than that in the general population. To address this question, we estimated sarcoidosis familial relative risk using data on disease occurrence in 10,862 first- and 17,047 second-degree relatives of 706 age, sex, race, and geographically matched cases and controls who participated in the multicenter ACCESS (A Case-Control Etiology Study of Sarcoidosis) study from 1996 to 1999. Familial relative risk estimates were calculated using a logistic regression technique that accounted for the dependence between relatives. Sibs had the highest relative risk (odds ratio [OR] = 5.8; 95% confidence interval [CI] = 2.1-15.9), followed by avuncular relationships (OR = 5.7; 95% CI = 1.6-20.7), grandparents (OR = 5.2; 95% CI = 1.5-18.0), and then parents (OR = 3.8; 95% CI = 1.2-11.3). In a multivariate model fit to the parents and sibs data, the familial relative risk adjusted for age, sex, relative class, and shared environment was 4.7 (95% CI = 2.3-9.7). White cases had a markedly higher familial relative risk compared with African-American cases (18.0 versus 2.8; p = 0.098). In summary, a significant elevated risk of sarcoidosis was observed among first- and second-degree relatives of sarcoidosis cases compared with relatives of matched control subjects.

Adolescent↗
PubMed1983

Cutaneous sarcoidosis associated with sarcoidosis of the upper airway.

The diagnosis of laryngeal sarcoidosis is frequently delayed because it is rare and often develops insidiously. A 24-year-old woman with the annular scarring form of cutaneous sarcoidosis later contracted laryngeal sarcoidosis. Despite a two-year history of upper respiratory tract symptoms, the patient's progressive respiratory distress was attributed to sarcoidal pulmonary involvement. Dermatologists should be aware of the risk of airway obstruction from laryngeal granulomas in patients with cutaneous sarcoidosis. Although most commonly associated with the lupus pernio form of sarcoidosis, upper airway granulomas also develop in patients with other cutaneous manifestations of sarcoidosis.

Adult↗
PubMed2000

[Heart failure as a cardiac symptom of sarcoidosis. Successful treatment of heart failure with steroids, digitalis and an angiotensin-1-receptor antagonist in sarcoidosis].

BACKGROUND: Sarcoidosis is a multisystemic disorder that may involve every organ. A symptomatic manifestation of the myocardium is possible, in these cases arrhythmias are the most common symptoms. CASE REPORT: This case report presents a 26-year-old female with the recurrence of Boeck's sarcoid. Fever, chill and a severe reduction in stress tolerance were the first symptoms. At the time of admission she complained of Grade III dyspnea according to the NYHA classification. The echocardiogram showed a severe impairment of the global and left ventricular function. The left ventricular ejection fraction was reduced to 30% and the Tei index was elevated to 1.0. A specimen taken from a mediastinal tumor confirmed the hypothesis of the recurrence of the sarcoidosis. Myocardial perfusion scintigraphy showed typical lesions for myocardial sarcoidosis. There were signs of an old anteroseptal infarction in the resting ECG without evidence of myocardial ischemia during a stress test. Repeated Holter-ECGs were without signs of severe arrhythmias whereas ventricular late potentials were positive. After the combined therapy with steroids, digitalis and an angiotensin-1 receptor antagonist, mediastinal mass and Tei index were reduced and the ejection fraction moved to 56%. Dyspnoea was classified with Grade II according to the NYHA classification. CONCLUSION: Treatment of asymptomatic sarcoidosis is still controversial, whereas the treatment of life-threatening sarcoidosis, eye involvement or severe hypercalcemia is accepted. This case report presents the successful treatment of severe heart failure with prednisone, glycosides and an angiotensin-1 receptor antagonist. With this combined therapy an improvement of subjective and objective parameters was possible.

Adult↗
PubMed2000

Sarcoidosis and common variable immunodeficiency. A case of a malignant course of sarcoidosis in conjunction with severe impairment of the cellular and humoral immune system.

The occurrence of sarcoidosis in combination with common variable immunodeficiency (CVID) has been described in a small number of patients. In these patients, sarcoidosis consisted of lymphadenopathy, mild to moderate pulmonary involvement and hepatosplenomegaly. However, severe and rapidly progressive pulmonary fibrosis in combination with a severe defect of the cellular and humoral immune system has not been described yet. In our patient, defects of the T and B cell system resulted in severe immunodeficiency. The defect of the humoral immune system was characterized by the impairment of specific antibody production in vivo. In addition, hypogammaglobulinemia with missing IgA and IgE along with a marked defect in IgM and IgG production was noted. There was a progressively reduced lymphocyte proliferation in response to T cell mitogens, while proliferation after specific IL-2 stimulation was normal. A Th1 lymphocyte-subset-like profile might thus play a role in the pathogenesis and might form the connecting link between sarcoidosis and CVID. This is the report of a so far new and unique combination of severe immunodeficiency and sarcoidosis also associated with a congenital dysmorphia consisting of a palatal cleft. The findings of the 40 patients with CVID and sarcoidosis reported so far are discussed in order to point out the typical features of patients with this uncommon syndrome.

Adult↗
PubMed1989

Studies of bronchoalveolar lavage cells and fluids in pulmonary sarcoidosis. I. Enhanced capacity of bronchoalveolar lavage cells from patients with pulmonary sarcoidosis to induce angiogenesis in vivo.

Both allogeneic immunocompetent CD4+ lymphocytes and activated macrophages of mice can induce neovascularization when inoculated intradermally into host animals. Because sarcoidosis is associated with an increase in both activated macrophages and CD4+ effector lymphocytes in the lung, we carried out experiments in which cells obtained by bronchoalveolar lavage (BAL) of patients with pulmonary sarcoidosis were tested in a murine intradermal angiogenesis assay. BAL cells from patients with pulmonary sarcoidosis induced a significantly greater degree of angiogenesis than those from normal volunteers or from patients with other lung diseases. Moreover, the degree of angiogenesis induced by BAL cells from patients with sarcoidosis correlated positively with the severity of the disease. When BAL cells were separated into macrophage and lymphocyte subpopulations by flow cytometric techniques, the observed angiogenic activity was restricted primarily or exclusively to macrophages; lymphocytes were unable to induce angiogenesis in this xenogeneic assay system. These experiments suggest that pulmonary macrophages may play a role in the pathogenesis of sarcoidosis by inducing changes in the pulmonary microvasculature. Moreover, we hypothesize that these vascular changes may be induced not only in the lung but also in other organ systems such as skin, muscle, and eye in which microangiopathies are associated with sarcoid disease.

Animals↗
PubMed1986

[Coincidence of silicosis and sarcoidosis. 2. Relations between silicosis and sarcoidosis as well as forensic consequences].

The observation of a coincidence of silicosis and sarcoidosis (see the 1st Communication) raises many problems on the pathogenesis of both diseases, their possible causal connections and not last the medical judgement. The mutual influence of both diseases is not discernible in the particular case. Pathogenetic connections derived from mentioned examples of coincidence of silicosis and sarcoidosis stay hypothetical, because its experimental confirmation cannot be realized. Within the medical judgement of the coincidence of silicosis and sarcoidosis the differentiation of injury caused only by silicosis must be tried. If that is not possible, the whole bodily injury must be imposed to silicosis to protect the patient from a disadvantageous expert opinion. The professional activity of patients with sarcoidosis in an atmosphere of quartz dust is not permissible, because the momentary injury of tissues of lung and lymph nodes as well as the prognosis of sarcoidosis are not definable with the actual available methods, but it is safe to say, that silicosis is an additional danger to the patient.

Dust↗
PubMed1995

[Cardiac sarcoidosis with myopathy and advanced A-V nodal block in a woman with a previous diagnosis of sarcoidosis].

In 1992, a 49-year-old woman was admitted to the hospital because of exertional dyspnea. Three years earlier sarcoidosis had been diagnosed, and the patient was found to have bilateral hilar lymphadenopathy. The eye, skin, and knee joint were also involved. During the second hospital stay, atrial flutter with advanced A-V nodal block, scattered defects on a 201T1 scintigram, and marked cardiomegaly on chest roentgenogram led to the diagnosis of cardiac sarcoidosis. Signs and symptoms of cardiac failure subsided after placement of an artificial cardiac pacemaker, but the patient still complained of mild muscle weakness in the lower extremities on exertion. 67Ga scintigraphy revealed marked accumulation in the lower extremities, and muscle biopsy of the left gastrocnemius revealed numerous epithelioid cell granulomas with muscle fiber degeneration. Oral corticosteroid therapy was effective. A review of the 24 cases of sarcoid myopathy reported in Japan indicated that the male-to-female ratio is 1:3.8. As compared to patients in whom myopathy led to the diagnosis of sarcoidosis, those in whom myopathy developed after sarcoidosis was diagnosed were (1) relatively older, (2) more likely to have multiple organ involvement, and (3) more likely to have cardiac sarcoidosis. Corticosteroids were beneficial in about three quarters of these 16 cases, who received corticosteroid therapy.

Anti-Inflammatory Agents↗
PubMed1989

Coexistence of sarcoidosis and myeloproliferative disease: a case of sarcoidosis preceding polycythaemia vera with a literature review.

A patient with the chronic active type of sarcoidosis developed polycythaemia vera 20 years later. A review of the literature shows that sarcoidosis preceding myeloproliferative disease tends to be of the chronic active variety. The same pattern is observed in associations of sarcoidosis with malignant lymphoproliferative disease and solid tumours, in which sarcoidosis appears to be the underlying cause of the subsequent malignancy.

Aged↗
PubMed1989

Studies of bronchoalveolar lavage cells and fluids in pulmonary sarcoidosis. II. Enhanced capacity of bronchoalveolar lavage fluids from patients with pulmonary sarcoidosis to induce cell movement in vitro.

The ability to increase the motility of endothelial cells in vitro is a property common to most if not all angiogenesis-inducing factors. Because bronchoalveolar lavage (BAL) cells from patients with pulmonary sarcoidosis have an enhanced capacity to induce neovascularization, the BAL fluids from these patients were assessed for their effect on human and murine endothelial cells and fibroblasts obtained from a variety of tissue sources. A recently developed computer-assisted image analysis system was used to determine the extent and pattern of cell migration in a microwell screening assay. Data were obtained for BAL fluids from 10 patients with pulmonary sarcoidosis and from five normal volunteers. BAL supernatants from patients with active sarcoidosis showed an enhanced (2- to 8-fold) capacity to induce chemokinesis of both endothelial cells and fibroblasts, as measured by increased area of migration and polarized cell movement. There was a marked heterogeneity in the motility of cells from different organ origins, but enhanced cell movement was observed with both endothelial cells and fibroblasts. In contrast, BAL fluids from normal and sarcoid patients were similar in their effect on muscle cells and urothelial cells, whereas pericytes, which responded to BAL fluids from normal subjects or patients with nongranulomatous pulmonary disease, were inhibited by BAL fluids from patients with pulmonary sarcoidosis. The induction of endothelial cell movement in vitro induced by individual supernatants generally correlated with the capacity of BAL cells from these patients to induce angiogenesis in vivo.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed1993

HLA class I, II, and III polymorphism in Italian patients with sarcoidosis. The Pavia-Padova Sarcoidosis Study Group.

We studied the HLA polymorphisms (class I, II, and III) in 107 Italian patients with biopsy specimen-proven sarcoidosis in order to investigate the immunogenetic background of this disease. The mean age of onset of the disease was 36.08 +/- 12.4 years. Four patients (3.73 percent) were in radiologic stage 0, 38 patients (35.51 percent) were in radiologic stage I, 40 patients (37.38 percent) were in stage II, and 25 (23.36 percent) were in stage III. Thirty-eight patients (35.51 percent) had one or more extrapulmonary localization(s) of the disease. Positive association between sarcoidosis and HLA-B8 (chi 2 = 6.07, p = 0.0127, RR = 1.91) was confirmed. Regarding the age of onset of the disease, HLA-B35 was more frequent (chi 2 = 7.34, p = 0.0056, pc < 0.05, RR = 4.62) in patients with early onset of symptoms and/or signs, before the mean age of 36 years. With reference to the radiologic stage of the disease, HLA class II marker DR3 was more frequent in patients with stage I (chi 2 = 7.22, p = 0.0061, pc < 0.05, RR = 7.08). No significant relationship was found between sarcoidosis and HLA class III markers. These results seem to confirm an association of sarcoidosis with HLA classic genes and can sustain the hypothesis of a genetic heterogeneity of this disease.

Adult↗