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*For medical professionals to read and reference Quick Collection, avoid misdiagnosis and mistreatment of sarcoidosis is not uncommon in clinical practice, but it is easy to be misdiagnosed and mistreated, because the clinical manifestations of sarcoidosis patients are very heterogeneous
.
This article combines the diagnosis and treatment guidelines of sarcoidosis to sort out the main points of diagnosis and differential diagnosis, in order to improve the clinical understanding of sarcoidosis
.
Differential diagnosis of sarcoidosis Before the diagnosis of sarcoidosis, granulomatous diseases of other etiologies must be excluded, especially in patients with sarcoidosis at the first diagnosis and in patients with atypical clinical symptoms (eg, after reduction of immunosuppressive therapy).
secondary relapsers)
.
The differential diagnosis of sarcoidosis includes: necrotizing versus non-necrotizing granulomatous disease (Table 1), as well as specific differential diagnoses based on the organ/system involved in sarcoidosis (Table 2)
.
Table 1 Differential diagnosis points of infectious and non-infectious granulomatous lesions at common biopsy sites Note: ACE: angiotensin-converting enzyme; EIA: enzyme-linked immunosorbent assay; ANCA: anti-neutrophil cytoplasmic antibody; CCP: cyclic Citrulline antibody; GLILD: granulomatous-lymphocytic interstitial lung disease; GLUS: granulomatous lesion syndrome of undetermined significance; GPA: granulomatosis with polyangiitis; LIP: lymphocytic interstitial pneumonia ; MAC: Mycobacterium avium-intracellular complex; M.
kansasii: Mycobacterium kansasii; MPA: Microscopic polyvascular; MPO: myeloperoxidase; p‐ANCA: perinuclear ANCA; PR3: PR3‐ ANCA; PET: positron tomography; TNF: tumor necrosis factor; a is a common differential diagnosis related to granulomatous diseases in the United States, and individualized differential diagnosis is recommended based on the clinical manifestations and medical history of patients; ×: no lesion distribution report table 2 Differential diagnosis points of sarcoidosis in different organs/systems Diagnostic flow chart: Figure 1 Diagnostic flow chart of sarcoidosis 8 aspects of sarcoid treatment recommendations The European Respiratory Society (ERS) working group committee has launched the clinical treatment of sarcoidosis The practice guideline, developed a total of eight Patient, Intervention, Comparison, Outcome (PICO) questions for which specific evidence-based recommendations were made
.
Table 3 ERS Task Force Recommendations PICO Question Recommendation 1) For patients with pulmonary sarcoidosis, should glucocorticoid therapy be added or immunosuppressive therapy not administered? For untreated patients primarily affected by pulmonary sarcoidosis, considered to be at higher risk for future death or permanent disability due to sarcoidosis, we suggest the introduction of glucocorticoid therapy to improve and/or maintain FVC and QoL
.
(Strong recommendation, low-quality evidence
.
) 2) For patients with pulmonary sarcoidosis, should immunosuppressive combination therapy be added or glucocorticoid therapy continued? 1.
For patients with symptomatic pulmonary sarcoidosis who have been treated with glucocorticoids and who have persistent disease or unacceptable side effects caused by glucocorticoids who are considered to be at higher risk of future death or permanent disability from sarcoidosis, we recommend adding Methotrexate to improve and/or preserve FVC and QoL
.
(Conditional recommendation, very low quality of evidence
.
) 2.
For patients with symptomatic pulmonary sarcoidosis who are treated with glucocorticoids or other immunosuppressive agents and whose disease persists, are considered to have a higher risk of future death or permanent disability, we recommend the addition of infliximab to improve and/or maintain FVC and QoL
.
(Conditional recommendation, low quality of evidence
.
) 3) For patients with cutaneous sarcoidosis, should glucocorticoid therapy be added or immunosuppressive therapy not administered? For patients with uncontrolled cutaneous sarcoidosis and significant active lesions with topical therapy, we suggest considering oral corticosteroids to reduce lesions
.
(Conditional recommendation, very low quality of evidence
.
) 4) Should glucocorticoid therapy be ineffective in patients with cutaneous sarcoidosis, should other immunosuppressive therapy be added? For patients with cutaneous sarcoidosis who have been treated with glucocorticoids and/or other immunosuppressive agents and have persistent significant active skin disease, we suggest the addition of infliximab to reduce cutaneous reduction compared to no additional treatment disease
.
(Conditional recommendation, low quality of evidence
.
) 5) For patients with clinically relevant cardiac sarcoidosis, should glucocorticoids be used with or without other immunosuppressive agents or without immunosuppressive therapy? For patients with evidence of functional cardiac abnormalities, including heart block, arrhythmias, or cardiomyopathy, we suggest glucocorticoids (with or without other immunosuppressants)
.
(Strong recommendation, very low quality of evidence
.
) 6) For patients with neurosarcoidosis, should immunosuppressive therapy be used or not? 1.
For patients with clinically significant neurosarcoidosis, we recommend glucocorticoid therapy
.
(Strong recommendation, very low-quality evidence
.
) 2.
For patients with neurosarcoidosis who have been treated with glucocorticoids and have persistent disease, we suggest adding methotrexate
.
(Conditional recommendation, very low quality of evidence
.
) 3.
For patients with neurosarcoidosis who have been treated with glucocorticoids and second-line drugs (methotrexate, azathioprine, mycophenolate mofetil) and have persistent disease, We recommend adding infliximab
.
(Conditional recommendation, very low quality of evidence
.
) 7) Should immunosuppressants, neurostimulants, exercise, or other treatments be used, or no treatment for fatigue, in patients with sarcoidosis-related fatigue? 1.
For patients with sarcoidosis producing uncomfortable fatigue, we recommend a 6-12 week pulmonary rehabilitation program and/or inspiratory muscle strength training to improve fatigue
.
(Conditional recommendation, low quality of evidence
.
) 2.
For patients with sarcoidosis who have severe fatigue unrelated to disease activity, we recommend d-methylphenidate or adrenalin after considering a lung exercise or rehabilitation program Modafinil for 8 weeks to test its effect on fatigue and tolerance
.
(Conditional recommendation, low quality of evidence
.
) 8) Should patients with sarcoidosis and small fiber neuropathy be treated with immunosuppressants or intravenous immunoglobulin or not? Due to a lack of sufficient evidence, no recommendation is made on this PICO issue
.
PICO: patient, intervention, comparison, outcome; FVC: forced vital capacity; QoL: quality of life
.
In short, sarcoidosis is not uncommon in clinical practice, but it is easy to be misdiagnosed and mistreated, and it needs the attention of clinicians
.
With the understanding of sarcoidosis, the continuous emergence of new sarcoid treatment methods and the continuous confirmation of their efficacy, the problem of sarcoidosis will hopefully be solved in the future
.
In order to better provide you with interesting, useful and attitudinal content, the Rheumatism and Immunity Channel in the medical community welcomes you to complete the following survey with your fingers, in just five seconds!
.
This article combines the diagnosis and treatment guidelines of sarcoidosis to sort out the main points of diagnosis and differential diagnosis, in order to improve the clinical understanding of sarcoidosis
.
Differential diagnosis of sarcoidosis Before the diagnosis of sarcoidosis, granulomatous diseases of other etiologies must be excluded, especially in patients with sarcoidosis at the first diagnosis and in patients with atypical clinical symptoms (eg, after reduction of immunosuppressive therapy).
secondary relapsers)
.
The differential diagnosis of sarcoidosis includes: necrotizing versus non-necrotizing granulomatous disease (Table 1), as well as specific differential diagnoses based on the organ/system involved in sarcoidosis (Table 2)
.
Table 1 Differential diagnosis points of infectious and non-infectious granulomatous lesions at common biopsy sites Note: ACE: angiotensin-converting enzyme; EIA: enzyme-linked immunosorbent assay; ANCA: anti-neutrophil cytoplasmic antibody; CCP: cyclic Citrulline antibody; GLILD: granulomatous-lymphocytic interstitial lung disease; GLUS: granulomatous lesion syndrome of undetermined significance; GPA: granulomatosis with polyangiitis; LIP: lymphocytic interstitial pneumonia ; MAC: Mycobacterium avium-intracellular complex; M.
kansasii: Mycobacterium kansasii; MPA: Microscopic polyvascular; MPO: myeloperoxidase; p‐ANCA: perinuclear ANCA; PR3: PR3‐ ANCA; PET: positron tomography; TNF: tumor necrosis factor; a is a common differential diagnosis related to granulomatous diseases in the United States, and individualized differential diagnosis is recommended based on the clinical manifestations and medical history of patients; ×: no lesion distribution report table 2 Differential diagnosis points of sarcoidosis in different organs/systems Diagnostic flow chart: Figure 1 Diagnostic flow chart of sarcoidosis 8 aspects of sarcoid treatment recommendations The European Respiratory Society (ERS) working group committee has launched the clinical treatment of sarcoidosis The practice guideline, developed a total of eight Patient, Intervention, Comparison, Outcome (PICO) questions for which specific evidence-based recommendations were made
.
Table 3 ERS Task Force Recommendations PICO Question Recommendation 1) For patients with pulmonary sarcoidosis, should glucocorticoid therapy be added or immunosuppressive therapy not administered? For untreated patients primarily affected by pulmonary sarcoidosis, considered to be at higher risk for future death or permanent disability due to sarcoidosis, we suggest the introduction of glucocorticoid therapy to improve and/or maintain FVC and QoL
.
(Strong recommendation, low-quality evidence
.
) 2) For patients with pulmonary sarcoidosis, should immunosuppressive combination therapy be added or glucocorticoid therapy continued? 1.
For patients with symptomatic pulmonary sarcoidosis who have been treated with glucocorticoids and who have persistent disease or unacceptable side effects caused by glucocorticoids who are considered to be at higher risk of future death or permanent disability from sarcoidosis, we recommend adding Methotrexate to improve and/or preserve FVC and QoL
.
(Conditional recommendation, very low quality of evidence
.
) 2.
For patients with symptomatic pulmonary sarcoidosis who are treated with glucocorticoids or other immunosuppressive agents and whose disease persists, are considered to have a higher risk of future death or permanent disability, we recommend the addition of infliximab to improve and/or maintain FVC and QoL
.
(Conditional recommendation, low quality of evidence
.
) 3) For patients with cutaneous sarcoidosis, should glucocorticoid therapy be added or immunosuppressive therapy not administered? For patients with uncontrolled cutaneous sarcoidosis and significant active lesions with topical therapy, we suggest considering oral corticosteroids to reduce lesions
.
(Conditional recommendation, very low quality of evidence
.
) 4) Should glucocorticoid therapy be ineffective in patients with cutaneous sarcoidosis, should other immunosuppressive therapy be added? For patients with cutaneous sarcoidosis who have been treated with glucocorticoids and/or other immunosuppressive agents and have persistent significant active skin disease, we suggest the addition of infliximab to reduce cutaneous reduction compared to no additional treatment disease
.
(Conditional recommendation, low quality of evidence
.
) 5) For patients with clinically relevant cardiac sarcoidosis, should glucocorticoids be used with or without other immunosuppressive agents or without immunosuppressive therapy? For patients with evidence of functional cardiac abnormalities, including heart block, arrhythmias, or cardiomyopathy, we suggest glucocorticoids (with or without other immunosuppressants)
.
(Strong recommendation, very low quality of evidence
.
) 6) For patients with neurosarcoidosis, should immunosuppressive therapy be used or not? 1.
For patients with clinically significant neurosarcoidosis, we recommend glucocorticoid therapy
.
(Strong recommendation, very low-quality evidence
.
) 2.
For patients with neurosarcoidosis who have been treated with glucocorticoids and have persistent disease, we suggest adding methotrexate
.
(Conditional recommendation, very low quality of evidence
.
) 3.
For patients with neurosarcoidosis who have been treated with glucocorticoids and second-line drugs (methotrexate, azathioprine, mycophenolate mofetil) and have persistent disease, We recommend adding infliximab
.
(Conditional recommendation, very low quality of evidence
.
) 7) Should immunosuppressants, neurostimulants, exercise, or other treatments be used, or no treatment for fatigue, in patients with sarcoidosis-related fatigue? 1.
For patients with sarcoidosis producing uncomfortable fatigue, we recommend a 6-12 week pulmonary rehabilitation program and/or inspiratory muscle strength training to improve fatigue
.
(Conditional recommendation, low quality of evidence
.
) 2.
For patients with sarcoidosis who have severe fatigue unrelated to disease activity, we recommend d-methylphenidate or adrenalin after considering a lung exercise or rehabilitation program Modafinil for 8 weeks to test its effect on fatigue and tolerance
.
(Conditional recommendation, low quality of evidence
.
) 8) Should patients with sarcoidosis and small fiber neuropathy be treated with immunosuppressants or intravenous immunoglobulin or not? Due to a lack of sufficient evidence, no recommendation is made on this PICO issue
.
PICO: patient, intervention, comparison, outcome; FVC: forced vital capacity; QoL: quality of life
.
In short, sarcoidosis is not uncommon in clinical practice, but it is easy to be misdiagnosed and mistreated, and it needs the attention of clinicians
.
With the understanding of sarcoidosis, the continuous emergence of new sarcoid treatment methods and the continuous confirmation of their efficacy, the problem of sarcoidosis will hopefully be solved in the future
.
In order to better provide you with interesting, useful and attitudinal content, the Rheumatism and Immunity Channel in the medical community welcomes you to complete the following survey with your fingers, in just five seconds!
