Pharmacotherapy for pulmonary sarcoidosis: A delphi consensus study

Schutt, Amanda C.; Bullington, Wendy; Judson, Marc A.

doi:10.1016/j.rmed.2009.12.009

Cited by 180 publications

(154 citation statements)

References 15 publications

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“…In a patient with pSS, the differential diagnosis of dyspnea and cough includes xerotrachea, pulmonary vascular disease, and interstitial lung diseases that may present with sicca symptoms or salivary gland involvement (e.g., sarcoidosis, IgG4-related systemic disease) [9][10][11]. Sarcoidosis may also present with lacrimal and salivary gland involvement, as well as diffuse parenchymal lung disease.…”

Section: Discussionmentioning

confidence: 99%

Coexisting primary Sjögren’s syndrome and sarcoidosis: coincidence, mutually exclusive conditions or syndrome?

Santiago

Rovisco

et al. 2014

Rheumatol Int

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the initial laboratory evaluation revealed an erythrocyte sedimentation rate (ESR) of 61 mm/h (normal value <10) and a serum C-reactive protein (CRP) level of 12 mg/L (normal value <5). Rheumatoid factor was 184 uI/mL (normal range <20), and serum angiotensin-converting enzyme (SaCE) was 65 u/I (normal value 8-52 u/L). Full blood count and blood chemistry were all within the normal range. Immunoglobulin G levels were 21.3 g/L (normal values 7.0-16.0 g/L); kappa light chain 18.58 g/L (6.66-14.65 g/L); and lambda light chain 7.43 g/L (2.99-6.99 g/L), with no detection of monoclonal component. Purified protein derivative test result was negative (an induration <5 mm).her chest radiograph (Fig. 1a) and high-resolution computed tomography (hRCt) (Fig. 2a) showed hilar and mediastinal lymphadenopathy and multiple small pulmonary nodules. Lung function tests and carbon monoxide diffusing capacity were normal. Bronchoalveolar lavage demonstrated an increase in CD4+/CD8+ t cell ratio (12.4). mycobacterial and fungal cultures were negative. to investigate the pulmonary nodules, a mediastinoscopydirected biopsy was performed. the histological exam revealed multiple noncaseating granulomas.the diagnosis of sarcoidosis (stage II) was established based on the clinical, imaging, and pathological findings. the patient was diagnosed with coexisting pSS and sarcoidosis.Because the respiratory symptoms and fatigue worsened over time, the patient was empirically treated with prednisolone 0.3 mg/kg/day for 6 weeks, with a slow tapering over 6 months, associated with calcium supplementation and alendronic acid/colecalciferol (70 mg/5,600 uI, weekly) [1].Gradually, all the symptoms improved, and 1 year later, the patient denied any respiratory or systemic symptoms.Abstract herein, we describe a 44-year-old female diagnosed with histologically proven coexistence of primary Sjögren's syndrome and sarcoidosis with pulmonary and muscular involvement. the differential diagnosis may be difficult, but this is not an exceptional case, which highlights the need to critically revise the consideration of sarcoidosis as an exclusion for primary Sjögren's syndrome, as established in current classification criteria.Keywords Primary Sjögren's syndrome · Sarcoidosis · Lung · muscle Case reporta 44-year-old Caucasian female was referred to our Department due to a 2-week history of dyspnea, persistent dry cough, arthralgia, and fatigue. Four years previously, primary Sjögren syndrome (pSS) had been diagnosed on the presence of sicca symptoms associated with SSa and SSB auto-antibodies, and a salivary gland biopsy demonstrating lymphocytic infiltrates (Chisholm-mason classification grade IV). She had been successfully treated with hydroxychloroquine 400 mg/day and an ophthalmic emulsion. her family and occupational and environmental histories were not contributory.Physical examination showed normal vital signs, and cardiovascular, pulmonary, and musculoskeletal clinical examinations were unremarkable. no peripheral adenopathies were noted.

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Section: Discussionmentioning

confidence: 99%

Coexisting primary Sjögren’s syndrome and sarcoidosis: coincidence, mutually exclusive conditions or syndrome?

Santiago

Rovisco

et al. 2014

Rheumatol Int

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“…However, it has been shown in one study that when remission is achieved with corticosteroids, relapse rates are much higher than spontaneous remission [Iannuzzi et al 2007]. Low-dose corticosteroids and basic drugs such as MTX, cyclosporine A and AZT are used for chronic arthropathy [Schutt et al 2010]. New biological drugs have recently been tried to treat sarcoidosis.…”

Section: Discussionmentioning

confidence: 99%

Sarcoidois: is it only a mimicker of primary rheumatic disease? A single center experience

Kobak

Sever

Sivrikoz

et al. 2013

Therapeutic Advances in Musculoskeletal

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Background: Sarcoidosis is known as a T helper 1 lymphocyte (Th1-Ly) mediated disease which can imitate or sometimes accompany many primary rheumatic diseases. The purpose of this study is to share the clinical, demographic and laboratory data of patients presenting with rheumatologic manifestations and diagnosed with sarcoidosis. Methods: A total of 42 patients (10 men) were included in the study. The patients were admitted to the rheumatology outpatient clinic for the first time with different rheumatic complaints between November 2011 and May 2013 and were diagnosed with sarcoidosis after relevant tests. Clinical, demographic, laboratory, radiological and histological data of these patients were collected during the 18-month follow-up period and then analyzed. Results: Mean patient age was 45.2 years (20-70 years) and mean duration of disease was 3.5 years (1 month-25 years). Evaluation of system and organ involvement revealed that 20 (47.6%) patients had erythema nodosum, 3 (7.1%) had uveitis, 1 (2.3%) had myositis, 1 (2.3%) had neurosarcoidosis, 32 (76.2%) had arthritis and 40 (95.2%) had arthralgia. Of the 32 patients with arthritis, 28 (87.5%) had involvement of the ankle, 3 (9.4%) had involvement of the knee and 1 (3.2%) had involvement of the wrist. No patient had cardiac involvement. Thoracic computed tomography scan showed stage 1, 2, 3 and 4 sarcoidosis in 12 (28.5%), 22 (52.4%), 4 (9.5%) and 4 (9.5%) patients, respectively. Histopathology of sarcoidosis was verified by endobronchial ultrasound, mediastinoscopy and skin and axillary biopsy of lymphadenopathies, which revealed noncaseating granulomas. Laboratory tests showed elevated serum angiotensin-converting enzyme in 15 (35.7%) patients, elevated serum calcium level in 6 (14.2%) patients and elevated serum 1,25-dihydroxyvitamin D concentrations in 2 (4.7%) patients. Serological tests showed antinuclear antibody positivity in 12 (28.5%) patients, rheumatoid factor positivity in 7 (16.6%) patients and anticyclic citrullinated antibody positivity in 2 (4.8%) patients. Conclusion: Sarcoidosis can imitate or accompany many primary rheumatic diseases. Sarcoidosis should be considered not simply as an imitator but as a primary rheumatic pathology mediated by Th1-Ly. New studies are warranted on this subject.

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“…Methotrexate and its less toxic analog leflunomide have been billed as the preferred corticosteroid sparing agents for pulmonary sarcoidosis [103,104]. Methotrexate is an anionic bicarboxylic acid at physiological pH [105].…”

Section: Disease Modifying Anti-rheumatic Drugsmentioning

confidence: 99%

Sarcoidosis: Unknown Etiology and Genetic Predisposition Provides Therapeutic Challenges

Rotsinger

Drake²

2014

J Pulm Respir Med

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Sarcoidosis is an idiopathic multisystem disorder characterized by noncaseating epithelioid granulomas predominately affecting lungs and lymph nodes, but with potential to affect any organ system. Sarcoidosis shares similarities in development to other infectious granulomatous diseases, such as berylliosis and tuberculosis; however, its elusive etiology and non-distinctive histology have provided many diagnostic challenges. Evidence of a transferrable antigen combined with a high incidence rate in the lungs has focused efforts primarily on antigens with airborne transmissibility. While Mycobacterium tuberculosis and Propionibacterium acnes have provided strong associations to implicate each as a contributor to sarcoidosis pathogenesis, detection challenges remain and consensus of a definitive antigen is lacking.

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Pharmacotherapy for pulmonary sarcoidosis: A delphi consensus study

Cited by 180 publications

References 15 publications

Coexisting primary Sjögren’s syndrome and sarcoidosis: coincidence, mutually exclusive conditions or syndrome?

Coexisting primary Sjögren’s syndrome and sarcoidosis: coincidence, mutually exclusive conditions or syndrome?

Sarcoidois: is it only a mimicker of primary rheumatic disease? A single center experience

Sarcoidosis: Unknown Etiology and Genetic Predisposition Provides Therapeutic Challenges

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