Clinical features and outcomes of asymptomatic pulmonary sarcoidosis. A comparative cohort study.

OBJECTIVE: To evaluate the clinical characteristics and outcomes of patients with asymptomatic pulmonary sarcoidosis (APS) detected incidentally and compare them with symptomatic non-Löfgren sarcoidosis (SnLS) patients. METHODS: Patients diagnosed as having APS at a University hospital in Barcelona, Spain, followed prospectively from 1976 to 2018. APS was defined as the presence of bilateral hilar lymphadenopathy (BHL) with or without lung parenchymal involvement discovered incidentally on chest radiograph or CT scan. APS was compared with SnLS. RESULTS: APS was diagnosed in 50 (13.6%) and SnLS in 317 (86.4%) patients. At diagnosis, stage I chest radiograph was significantly more frequent in APS than in SnLS (p < 0.001) and there were no asymptomatic patients with stages III and IV. SnLS showed more severe impairment in FVC (p = 0.009) and forced expiratory volume in 1st second (FEV1) (p = 0.003) than APS, while DLco was similar in both groups. Extrathoracic involvement at diagnosis and during the follow up was less frequent in APS than in SnLS patients (p < 0.005). Endobronchial ultrasonography-guided transbronchial needle aspiration (EBUS) was the most used diagnostic tool. Treatment was more frequently required in the SnLS than in APS (p < 0.001). At five years, APS patients showed less presence of active disease than SnLS (p = 0.054). CONCLUSIONS: APS showed earlier radiological stages, lesser impairment in lung function, extrapulmonary organ involvement and need for treatment than SnLS. EBUS was the most useful diagnostic tool. In spite of its benign presentation, around one third of patients evolved to persistent disease but usually with mild clinical and functional impairment.

Histopathological Features of Subcutaneous Sarcoidosis.

Only a few series of patients with systemic sarcoidosis and specific subcutaneous lesions have been reported. We reviewed our patients with systemic sarcoidosis with specific subcutaneous lesions to analyze their histopathological features and their relationship with clinical features of the systemic disease. Patients with systemic sarcoidosis with predominantly subcutaneous sarcoid granulomas diagnosed between 1980 and 2016 in Bellvitge University Hospital were enrolled. We also analyzed patients with clinically and histopathologically identical lesions in whom a diagnosis of systemic sarcoidosis could not be made during follow-up. Twenty-eight patients with systemic sarcoidosis presented specific subcutaneous lesions (23 women and 5 men, mean age 55.64 SD 12.26 years). Dermal involvement was observed in 10 cases, always discrete and limited to deep reticular dermis. The distribution of the granulomatous infiltrate was lobular in 7 cases and lobular and septal in 21. Fibrosis was observed in 21 cases. There were no significant differences in persistence of lesions or persistence of systemic disease activity when comparing patients with and without fibrosis. In conclusion, fibrosis is a frequent finding in subcutaneous sarcoidosis, and although it may be intense, it is not associated with pulmonary fibrosis or with >2 years of persistence of systemic sarcoidosis activity.

Elderly sarcoidosis: A comparative study from a 42-year single-centre experience.

OBJECTIVES: To describe the clinical features and outcomes in elderly patients with sarcoidosis and to compare them with younger patients. MATERIAL AND METHODS: Retrospective study of a large cohort of 668 consecutive patients with sarcoidosis prospectively collected during 42 years at the Bellvitge University Hospital, a tertiary care single-centre in Barcelona, Spain. Elderly sarcoidosis was defined as sarcoidosis diagnosed in patients ≥65 years-old. RESULTS: Elderly sarcoidosis was diagnosed in 47 (7%) patients. In younger patients, Löfgren's syndrome was the predominant mode of onset (8.5% vs. 42.2%, p < 0.001). At diagnosis, elderly patients more frequently demonstrated radiographic stage III and IV sarcoidosis (21.3% vs. 7.6%, p = 0.001), isolated extrapulmonary involvement (21.3% vs. 8.2%, p = 0.003), subcutaneous nodules (17% vs. 3.4%, p < 0.001) and intraabdominal/retroperitoneal lymph nodes (23.4% vs. 9.5%, p = 0.003). Furthermore, patients with elderly sarcoidosis achieved remission (spontaneous and under treatment) less frequently during the follow-up period (14 patients, 35% vs. 305 patients, 53%, p = 0.027) and had a higher incidence of pulmonary fibrosis (15% vs. 6.1%, p = 0.029). Death related to sarcoidosis was more prevalent in elderly patients (6.4% vs. 1.3%, p = 0.036). CONCLUSIONS: Sarcoidosis in elderly patients requires a high index of suspicion. Aged pulmonary sarcoidosis patients presented with more severe disease at presentation and worse outcomes compared to younger patients. Isolated extrapulmonary involvement at diagnosis and certain particular extrapulmonary organ involvement were more frequent in elderly sarcoidosis. Remission was less frequent in elderly sarcoidosis.

Specific Skin Lesions of Sarcoidosis Located at Venipuncture Points for Blood Sample Collection.

It has been suggested that the predilection of sarcoidosis to affect scars is due to the presence of antigens or foreign bodies that can serve as a stimulus for granuloma formation. Several patients with sarcoidosis-specific skin lesions in venous puncture sites have been reported. However, in these patients the pathogenesis of the cutaneous lesions is not clear because the presence of foreign bodies is not to be expected. Our objective was to describe 3 patients who developed specific lesions of sarcoidosis in areas of venipuncture and to discuss their possible pathogenesis. The database of the Sarcoid Clinic of Bellvitge Hospital (an 800-bed university referral center providing tertiary care to approximately 1 million people in Barcelona, Spain) was reviewed to detect those patients with specific cutaneous lesions of systemic sarcoidosis in areas of venipuncture. Three patients with biopsy-proven specific cutaneous lesions of systemic sarcoidosis in areas of venipuncture for blood collection were detected (3 women, mean age 56 years). In one case, the histopathological image shows the hypothetical path of a needle through the skin. In 2 cases, an amorphous birefringent material was detected under polarized light. This material was consistent with silicone. In patients who are developing sarcoidosis, the smallest amount of oil used as lubricant in the needle for sample blood collection may induce the formation of granulomas. In addition to exploring scars, it is advisable to explore the cubital folds to detect specific cutaneous lesions of sarcoidosis.

Multidisciplinary approach and long-term follow-up in a series of 640 consecutive patients with sarcoidosis: Cohort study of a 40-year clinical experience at a tertiary referral center in Barcelona, Spain.

Cohort studies of large series of patients with sarcoidosis over a long period of time are scarce. The aim of this study is to report a 40-year clinical experience of a large series of patients at Bellvitge University Hospital, a tertiary university hospital in Barcelona, Spain. Diagnosis of sarcoidosis required histological confirmation except in certain specific situations. All patients underwent a prospective study protocol. Clinical assessment and follow-up of patients were performed by a multidisciplinary team.From 1976 to 2015, 640 patients were diagnosed with sarcoidosis, 438 of them (68.4%) were female (sex ratio F/M 2:1). The mean age at diagnosis was 43.3 ±â€Š13.8 years (range, 14-86 years), and 613 patients (95.8%) were Caucasian. At diagnosis, 584 patients (91.2%) showed intrathoracic involvement at chest radiograph, and most of the patients had normal pulmonary function. Erythema nodosum (39.8%) and specific cutaneous lesions (20.8%) were the most frequent extrapulmonary manifestations, but there was a wide range of organ involvement. A total of 492 patients (76.8%) had positive histology. Follow-up was carried out in 587 patients (91.7%), over a mean of 112.4 ±â€Š98.3 months (range, 6.4-475 months). Corticosteroid treatment was administered in 255 patients (43.4%), and steroid-sparing agents in 49 patients (7.7%). Outcomes were as follows: 111 patients (18.9%) showed active disease at the time of closing this study, 250 (42.6%) presented spontaneous remission, 61 (10.4%) had remission under treatment, and 165 (28.1%) evolved to chronic sarcoidosis; among them, 115 (19.6%) with mild disease and 50 (8.5%) with moderate to severe organ damage. A multivariate analysis showed that at diagnosis, age more than 40 years, the presence of pulmonary involvement on chest radiograph, splenic involvement, and the need of treatment, was associated with chronic sarcoidosis, whereas Löfgren syndrome and mediastinal lymphadenopathy on chest radiograph were indicators of good outcome.Sarcoidosis is a multisystem disease with protean clinical-radiographic manifestations. Although almost half of patients follow a spontaneous resolution or under treatment, a significant number of them may have several degrees of organ damage. This study emphasizes the value of a multidisciplinary approach and long-term follow-up by specialized teams in sarcoidosis.

Aspectos actuales de la sarcoidosis / Current aspects of sarcoidosis

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Papular sarcoidosis of the knees. A frequent form of presentation of systemic sarcoidosis.

BACKGROUND: In recent years we have observed with increasing frequency granulomatous papular lesions involving the knees, for which we proposed the term papular sarcoidosis of knees. OBJECTIVES: To evaluate the clinicopathological features of papular sarcoidosis of the knees. METHODS: Patients with papular lesions of the knees and histopathologically sarcoid granulomas were included in the study. Systemic sarcoidosis was investigated in all cases. Clinical charts were retrospectively retrieved. Biopsy specimens were evaluated under polarized light to detect foreign bodies. RESULTS: Fifty-three patients fulfilled inclusion criteria. In 36 cases systemic sarcoidosis was diagnosed and these cases were considered as papular sarcoidosis of the knees. Foreign particles were observed in 21 of these 36 patients. In only 9/36 patients did the activity of systemic disease persist over two years. In 17 cases sarcoidosis could not be demonstrated during follow-up. CONCLUSION: Papular sarcoidosis of the knees can be considered a relatively frequent form of cutaneous sarcoidosis usually present at the beginning of the disease that can be useful for the diagnosis of sarcoidosis. It is mainly observed in acute forms of sarcoidosis and can be considered a sign of good prognosis.

Consideraciones sobre el síndrome de Löfgren / Considerations about Löfgren syndrome

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Granulomatous cutaneous sarcoidosis: diagnosis, relationship to systemic disease, prognosis and treatment.

Sarcoidosis is an antigen-mediated disease of unknown aetiology, characterized by the presence of non-caseating epithelioid cell granulomas in multiple organs. Cutaneous involvement in sarcoidosis is classified as specific, when biopsy reveals non-caseating granulomas, and non-specific, typically erythema nodosum. Granulomatous skin lesions occur in 9% to 37% of patients. The skin is the second most commonly involved organ after the lung. A skin biopsy is easy to perform and enables an early diagnosis with a minor invasive procedure. Some types of specific lesions have prognostic significance and may help to predict the outcome of the systemic disease. Maculopapules, subcutaneous nodules and scar sarcoidosis are usually transient or tend to follow the course of the systemic disease. Skin plaques and lupus pernio are associated with chronic sarcoidosis. Although most cutaneous lesions of sarcoidosis do not cause significant morbidity and do not require treatment, some have cosmetic importance because they may be disfiguring and can have a strong psychosocial impact. Treatment of these lesions is a challenge since they do not respond well to conventional treatments. This manuscript reviews the clinical characteristics of the more frequent types of specific cutaneous lesions of sarcoidosis, the relationship between cutaneous involvement and systemic disease, the prognostic significance of lesions and the present state of treatment of difficult cases of cutaneous sarcoidosis.

Skin manifestations of sarcoidosis.

The skin manifestations of sarcoidosis are classified as specific, where biopsy reveals non-caseating granulomas, and non-specific, typically erythema nodosum. The most frequent specific (granulomatous) skin lesions are maculopapules, subcutaneous nodules, scar sarcoidosis, plaques and lupus pernio. Skin biopsy allows early diagnosis of sarcoidosis through a non-aggressive procedure. In sarcoidosis, erythema nodosum is usually associated with bilateral hilar lymphadenopathy on the chest radiograph, this being known as Löfgren's syndrome. Cutaneous lesions have prognostic significance. Löfgren's syndrome is usually associated with good prognosis and spontaneous resolution. Maculopapular lesions and subcutaneous nodules are more often associated with remission of the systemic disease at two years, while plaques and, mainly, lupus pernio are hallmarks of chronic disease. Most cutaneous lesions of sarcoidosis are only mildly symptomatic and do not require treatment. However, chronic skin lesions, particularly lupus pernio, are disfiguring and can have a strong psychological and social impact. Treatment of these lesions is a challenge since they do not respond well to conventional treatments. The introduction of biological agents has been an important although not definitive advance in the treatment of cutaneous sarcoidosis.

Molecular imaging in sarcoidosis.

PURPOSE OF REVIEW: In recent years molecular imaging techniques have made important advances as regards the study of sarcoidosis. This paper reviews new developments in these techniques as well as their present role and limitations in the assessment of patients with sarcoidosis. RECENT FINDINGS: PET with (18)F-fluorodeoxyglucose ((18)F-FDG PET) has proved to be more sensitive than (67)gallium ((67)Ga) scan for assessing the inflammatory activity of sarcoidosis. Integrated (18)F-FDG PET/computed tomography (CT) scanners have improved diagnostic accuracy, and an emerging role for (18)F-FDG PET/CT in monitoring therapy has been described. The use of MRI is well established in neurosarcoidosis and musculoskeletal sarcoidosis. MRI is also the test of choice in suspected cardiac sarcoidosis. It provides anatomical information and quantification of ventricular function, and reveals very early changes in the signal of the myocardium in delayed enhancement. SUMMARY: (18)F-FDG PET/CT is useful in the detection of occult granuloma sites and residual activity in patients with fibrotic pulmonary sarcoidosis. It has an emerging role in the therapeutic management of patients with multisystemic sarcoidosis. MRI is indicated when neurosarcoidosis or cardiac or musculoskeletal involvement is suspected. Although most lesions detected are nonspecific in appearance, some patterns may be present in the early stages.

Subcutaneous sarcoidosis.

Subcutaneous sarcoidosis has been reported to occur in 1.4% to 6% of patients with systemic sarcoidosis. Most reported cases are in women, most often in their fifth and sixth decades, and appear as multiple, asymptomatic, hardly indurated subcutaneous nodules without changes in the overlying epidermis. The lesions are characteristically located in the upper extremities, mainly in the forearms, and usually are bilateral and asymmetric. In most cases the lesions appear at the beginning of systemic sarcoidosis and are not associated with chronic fibrotic disease. Histopathologically, sarcoidosis is characterized by noncaseating naked granulomas involving fat lobules, with minimal to no septal involvement.

Erythema nodosum.

Erythema nodosum (EN) is the most common form of panniculitis. It is characterized by the presence of rounded or oval, slightly raised, nonulcerative painful red nodules in the skin and subcutaneous fatty tissue, 1 to 6 cm in diameter, sometimes coalescing. They tend to be symmetrical in distribution and are usually located bilaterally on the lower extremities, particularly on the anterior tibial surface, although they may also involve the ankles, the lower parts of the thighs, and the forearms.

Sarcoidosis and autoimmune thyroid disease. A case series of ten patients.

BACKGROUND: Sarcoidosis coexisting with autoimmune disorders, especially with autoimmune thyroid disease (ATD), has been previously described and a common immunopathogenesis has been proposed. We report a series of ten new cases of this association from a large series of patients with sarcoidosis. METHODS: The clinical records of patients diagnosed with sarcoidosis between 1984 and 2006 in the Bellvitge University Hospital were reviewed, and those who were also diagnosed as having ATD were selected. A review of the literature was performed as well. RESULTS: Ten out of 348 (2.9%) patients with sarcoidosis were identified as having ATD. Sarcoidosis presented as Löfgren's syndrome in 8 patients. Three patients developed Graves' disease, 6 Hashimoto's thyroiditis with hypothyroidism and one had postpartum thyroiditis. In one case, ATD had developed 15 years before sarcoidosis. In the remaining nine cases, sarcoidosis preceded between 4 months to 17 years the development of ATD. In 3 of these cases, sarcoidosis was active when ATD was diagnosed. In one patient, Graves' disease developed immediately after the administration of potassium iodide to treat erythema nodosum. CONCLUSIONS: Sarcoidosis may be associated with ATD at some time of its evolution, either as hyperthyroidism or hypothyroidism. Usually, ATD does not develop during the period of activity of sarcoidosis. We suggest considering personal and family past history of thyroid disease before administering potassium iodide for erythema nodosum in patients with sarcoidosis, as it could trigger hyperthyroidism, especially in patients with iodine deficiency.

Abscesos cutáneos y masa mediastínica en varón joven / Skin abscesses and a mediastinal mass in a young male

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