SARS-CoV-2 lung disease in a patient with pulmonary sarcoidosis - case report.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is the recently identified cause of the current pandemic. In patients with chronic respiratory lung diseases, SARS-CoV-2 may result in significant morbidity and increased mortality. We present a case of a 69-year-old male with stage II pulmonary sarcoidosis who had been under observation for 30 months without immunosuppressive treatment. He then developed severe SARS-CoV-2 disease with typical radiological and laboratory findings. Therapy with oxygen, antibiotics, low-molecular-weight heparin in a prophylactic dose, and dexamethasone resulted in marked clinical improvement. We will discuss the rationale for corticosteroid use in both SARS-CoV-2 disease and in SARS-CoV-2 disease that is complicating comorbid sarcoidosis.

Effectiveness and tolerability of methotrexate in pulmonary sarcoidosis: A single center real-world study.

Background: Pulmonary sarcoidosis patients who get disease progression despite corticosteroid treatment or can't tolerate corticosteroid required second-line drug. Methotrexate (MTX) is the most widely used in our clinical practice. Data on its safety and efficacy at different doses are still limited, especially for those without folic acid supplements. Objective: To report effectiveness of different MTX dosages and tolerability of MTX in pulmonary sarcoidosis without folic acid supplements. Methods: A retrospective study on pulmonary sarcoidosis patients receiving MTX therapy with various dose ≥3 months was conducted. The primary outcome was change in high-resolution computed tomography (HRCT) before and after MTX therapy. Other efficacy parameters included SGRQ score, prednisone dose change, discontinuation and relapse-free survival. Response-linked factors and safety outcomes were also analyzed. Results: Overall, 49 patients (81.7%) were assessed as MTX responders by HRCT and there was no significant difference in clinical response rate among three groups with different doses. The health-related quality of life (HRQL) of the responders improved obviously, which was evidenced by SGRQ score declining from 16.7(IQR: 7.9-26.4) to 10.7(IQR: 4.8-19.3) (P=0.029). The corticosteroids sparing effect was confirmed in "responders" group (P<0.001). When MTX was discontinued in 11 responders with complete improvement, 2 patients experienced relapses within 15.5 (range: 1-30) months (mean follow-up time of these 11 responders: 13.5±13.0 months). No clinical characteristics were found related to MTX effectiveness. Adverse events occurred in 31.7% of the patients, with gastrointestinal-related being the commonest. Drug discontinuation owing to adverse events occupied 6.7% of the subjects. Conclusions: Nearly 80% of the sarcoidosis subjects had well response to MTX. Its effectiveness was irrelevant to the treatment dosages and baseline characteristics. A quite low relapse rate was witnessed in those complete responders discontinuing MTX therapies. The steroid-sparing effect, well drug tolerability and low drug withdrawal rate were observed in these patients even without folic acid supplements in clinical practice.

A Rare Complication of Sarcoidosis.

BACKGROUND: Cerebral vasculitis is a serious, but uncommon inflammatory condition of the blood vessel walls, with an annual incidence of 1-2 per million. A variety of disorders including encephalopathy, stroke, seizure, acute or subacute focal deficits should be considered as a differential diagnosis. CLINICAL CASE: A 56-year old male with a past history of pulmonary sarcoid presented with a unilateral facial numbness and loss of balance arising during sleep. His computed tomography scan of the brain was normal but brain magnetic resonance imaging with gadolinium demonstrated scattered infarcts in mixture of stroke topography not purely in keeping with embolism nor intrinsic small vessel disease. Further investigations including carotid ultrasound, transthoracic echo and 24-hour electrocardiogram were within normal limits. However, computed tomography angiography showed evidence of a widespread intracranial vasculopathy, as well as evidence of dissection of the left common carotid artery. His elevated calcium was consistence with a sarcoidosis relapse and cerebrospinal fluid analysis was in keeping with a central nervous system inflammatory process. Treatment was commenced with high dose steroids with additional pulsed intravenous cyclophosphamide together with antiplatelet therapy and a statin. CONCLUSIONS: This case illustrates the intracranial vasculopathy as a rare complication of sarcoidosis. Although sarcoid is well recognized to affect the central nervous system, it is unusual in the form of cerebral vasculitis.

Efficacy and safety of infliximab biosimilar Inflectra® in severe sarcoidosis.

BACKGROUND: Infliximab, a monoclonal antibody against tumor necrosis factor alpha (TNF-α) is effective third-line therapy in severe sarcoidosis. The originator product of Infliximab, Remicade®, is expensive, limiting universal access. Recently, a less expensive biosimilar of infliximab, Inflectra®, has become available, but the efficacy and tolerability has not been studied in sarcoidosis. METHODS: In this retrospective cohort study, 29 patients treated with the infliximab biosimilar Inflectra®, were analysed. Patients received Inflectra® intravenously monthly at a dose of 5 mg/kg. We measured trough levels before every infusion. Before and after 6 months of induction therapy pulmonary function and disease activity were evaluated using Standardised Uptake Value (SUV) of the 18F-fluorodeoxyglucose by positron emission tomography (18F-FDG PET), soluble interleukin-2 receptor (sIL-2R), angiotensin converting enzyme (ACE) and health-related quality of life (HRQOL). RESULTS: In patients with pulmonary sarcoidosis as main treatment indication (n = 15) the predicted FVC improved with 8.1%, p < 0.05. Furthermore, in the whole group HRQoL improved significantly (p < 0.001), whereas SUVmax and sIL-2R significantly reduced (p < 0.001 and p = 0.001 respectively). Hospitalisation due to infections occurred in four patients. None of the patients discontinued Inflectra® due to side-effects. Furthermore, all patients had detectable trough levels indicating development of neutralizing antibodies. CONCLUSION: Infliximab biosimilar Inflectra® seems effective in the treatment of refractory sarcoidosis with a comparable safety profile to the reference product Remicade®. Inflectra® can be considered as an alternative and less expensive option for patients with refractory sarcoidosis.

The effect of corticosteroids on quality of life in a sarcoidosis clinic: the results of a propensity analysis.

BACKGROUND: Both sarcoidosis and its treatment may worsen health related quality of life (HRQoL). We performed a propensity analysis of sarcoidosis-specific HRQoL patient reported outcome measures (PRO) to disentangle the effects of sarcoidosis and corticosteroid therapy on HRQoL in sarcoidosis outpatients. METHODS: Consecutive outpatient sarcoidosis patients were administered modules from two sarcoidosis-specific HRQoL PROs: the Sarcoidosis Health Questionnaire (SHQ) and the Sarcoidosis Assessment Tool (SAT). Patients were divided into those that received ≤500 mg of prednisone (PRED-LOW) versus >500 mg of prednisone (PRED-HIGH) over the previous year. SAT and SHQ scores were initially compared in the two corticosteroid groups. Then a multivariate analysis was performed using a propensity score analysis adjusted for race, age, gender and the severity of illness. RESULTS: In the unadjusted analysis, the PRED-HIGH group demonstrated the following worse HRQoL scores compared to the LOW-PRED group: SHQ Daily (p = 0.02), SAT satisfaction (p = 0.03), SAT daily activities (p = 0.03). In the propensity analysis, the following domains demonstrated worse HRQoL in the PRED-HIGH group than the PRED-LOW group: SAT fatigue (p < 0.0001), SAT daily activities (p = 0.03), SAT satisfaction (p = 0.03). All these differences exceeded the established minimum important difference for these SAT domains. The SHQ Physical score appeared to demonstrate a borderline improved HRQoL in the PRED-HIGH versus the PRED-LOW group (p = 0.05).). In a post-hoc exploratory analysis, the presence of cardiac sarcoidosis may have explained the quality of life differences between the two corticosteroid groups. CONCLUSIONS: Our cohort of sarcoidosis clinic patients who received ≤500 mg of prednisone in the previous year had an improved HRQoL compared to patients receiving >500 mg on the basis of two sarcoidosis-specific PROs after adjusting for severity of illness. These data support the need to measure HRQoL in sarcoidosis trials, and suggest that the search should continue for effective alternative medications to corticosteroids.

Hot tub lung: presenting features and clinical course of 21 patients.

BACKGROUND: Hot tub lung is an emerging lung disorder associated with exposure to Mycobacterium avium complex organisms contaminating hot tub water. OBJECTIVES: To define the clinical characteristics and outcome of patients with hot tub lung. METHODS: Retrospective review of 21 patients diagnosed with hot tub lung at a tertiary medical center over a 7-year period. RESULTS: The mean (+/-sd) age at presentation was 46 (+/- 15) years; 9 patients were men (43%). All patients described ongoing exposure to hot tubs. The most common referral diagnoses were sarcoidosis, bronchitis, and asthma. Dyspnea and cough were present in all patients, hypoxemia was noted in 10 patients (48%). High-resolution computed tomography of the chest had been performed in 20 patients and demonstrated diffuse centrilobular nodules and/or ground-glass opacities in all patients. M. avium complex was isolated from the hot tub water, respiratory secretions and/or lung tissue in all patients. Bronchoscopic or surgical lung biopsy was obtained in 18 patients and demonstrated bronchiolocentric granulomatous inflammation. With avoidance of exposure, clinical and radiologic improvement was observed in all patients. Additionally, 13 patients (62%) received corticosteroid therapy, 1 (5%) antimycobacterial therapy, 2 (10%) received both, and 5 patients (24%) received no pharmacologic therapy. CONCLUSIONS: Hot tub lung likely represents hypersensitivity pneumonitis due to inhalational exposure to M. avium complex. Antimycobacterial therapy does not appear to be required in the management of this disease. Although corticosteroids may be helpful in the treatment of severely affected patients, others can be managed by avoidance of additional exposure alone.

[Likopid in the complex immunomodulating treatment of patients with sarcoidosis of the lung and intrathoracic lymph nodes]. / Likopid v kompleksnom immunokorrigiruiushchem lechenii bol'nykh sarkoidozom legkikh i vnutrigrudnykh limfaticheskikh uzlov.

AIM: To examine the functional status of the immune system in patients with lung and intrathoracic lymph nodes sarcoidosis and to evaluate the efficiency of immunomodulation alone and in its inclusion in combined treatment of the disease. MATERIALS AND METHODS: 58 patients with the disease of varying severity were followed up. Comprehensive examination, involving clinical, immunological, X-ray, and physical studies, in patients treated with combined immunotherapy was performed. Initial examination revealed mixed immunodefficiency with impaired T- and phagocytic activity. According to the degree of immunological changes, the patients were given immunotherapy, including polyoxidonium, T-activin (or immunophan) injections, a complex of multivitamins and trace elements, and total adaptogens. After partial or complete normalization of an immunogram, all the patients received licopid (two-three 10-day courses, 10 mg/day). RESULTS: The optimal result (as long as 3-year remission) was achieved in the first time diagnosed sarcoidosis who have not taken glucocorticoidal hormones. CONCLUSION: The follow-up shows that addition of licopid is a compulsory component of immunotherapy in this disease; the efficiency of treatment is determined by its multimodality. The courses of therapy should be repeated when immunological indices deteriorated.

Mortality of intrathoracic sarcoidosis in referral vs population-based settings: influence of stage, ethnicity, and corticosteroid therapy.

STUDY OBJECTIVES: To compare the sarcoidosis mortality in referral settings (RS) and population-based settings (PS), and to identify the contribution of stage, ethnicity, and corticosteroid therapy (CST) to their disparate outcomes. DESIGN: All observational studies identified in a MEDLINE search and bibliographic review published in the English language since 1960 dealing with the course and prognosis of sarcoidosis in large, unsorted, adult, ambulatory RS and PS providing long-term follow-up were reviewed and subjected to meta-analysis. MEASUREMENTS AND RESULTS: Sarcoidosis mortality in RS (4.8%), in which 17% of patients had the most unfavorable prognosis as judged by stage (stage III), was 10-fold that reported in PS (0.5%), in which 11% of patients were identified at this stage. The magnitude of this disparity could not be accounted for solely by adverse selection, as indicated by stage or by ethnicity. Patients in RS received CST with sevenfold the frequency of PS, and its provision was highly correlated with stage-normalized mortality. CONCLUSION: The prognosis of patients with intrathoracic sarcoidosis in PS is far more favorable than that obtained in RS. Sarcoidosis mortality is largely independent of ethnicity. The possibility cannot be excluded that excessive employment of CST may unfavorably influence the long-term course of the disease in some individuals.

Sarcoidosis associated with psoriasis vulgaris.

We report a 70-year-old patient with sarcoidosis associated with psoriasis vulgaris. He had a nodule on the medial lower lid of his right eye. Oral corticosteroid for the sarcoid lesions and oral PUVA for psoriasis were employed. The cutaneous lesion disappeared within two months after starting the therapy. No relapse of sarcoidosis has been seen for eight years. The association of sarcoidosis with psoriasis has been previously reported; however, it is still unclear whether this association coincidental or meaningful.

No effect of high-dose inhaled steroids in pulmonary sarcoidosis: a double-blind, placebo-controlled study.

OBJECTIVE: To evaluate whether inhaled steroids in high doses might be of therapeutic value in pulmonary sarcoidosis. DESIGN: Randomized, double blind and placebo controlled parallel study. SETTING: The out-patient clinic of the Department of Pulmonary Medicine, Gentofte Hospital, Copenhagen, Denmark. SUBJECTS: Twenty-one untreated patients (17 males, 4 females, median age 33 years, range 21-65) and eight patients treated with systemic prednisolone. All patients had biopsy proven pulmonary sarcoidosis radiological stage I-III. INTERVENTIONS: Treatment with either inhaled budesonide 1.2 mg day-1-2.0 mg day-1 (n = 9) or placebo (n = 12) for 12 months. MAIN OUTCOME MEASURES: Clinical (cough, chest pain, dyspnoea) and paraclinical variables (chest X-ray, gallium scintigraphy, pulmonary function tests, and biochemical markers of disease activity: blood leukocytes, lymphocytes, serum (S-) angiotensin converting enzyme (ACE), S-1,25-OH-cholecalciferol, plasma (P-) calcium, P-immunoglobulins) were recorded before treatment, every three months during treatment, and 6 months after treatment had been discontinued. RESULTS: There were no significant differences between the recorded variables in the budesonide and placebo groups. In general, a regression of disease activity was observed in both groups. Two patients in the treatment group, treated with 2.0 mg budesonide/day, and two in the placebo group had progression in disease and were put on systemic steroids. CONCLUSION: Inhaled budesonide in doses of 1.2-2.0 mg day-1 had no recognizable therapeutic effect on pulmonary sarcoidosis.