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Guidelines, statements and technical standards for Respiratory Medicine
BTS guidelines for the management of spontaneous pneumothorax. BTS guidelines for the insertion of a chest drain. Measurement of exhaled nitric oxide in children. Paediatric respiratory training in Europe: Consequently many authors advise testing for TPMT levels before starting azathioprine, despite a paucity of evidence to support this practice. Hydroxychloroquine like chloroquine is an anti-malarial agent. Two randomized controlled trials have compared chloroquine and placebo in pulmonary sarcoidois. Chloroquine treatment conferred no benefit and greater side-effects. In the recent, Canadian, study, 23 patients received chloroquine mg daily for 6 months, gradually tapering every two months to mg daily.
Eighteen patients were then randomized to a maintenance group or to an observation group. Patients randomized to the maintenance group had a slower decline in lung function and fewer relapses than those in the observation group. Side-effects were mainly limited to the high dose treatment phase. The authors conclude that chloroquine should be considered in chronic pulmonary sarcoidosis; in practice, hydroxychloroquine is preferred because it has lower ocular toxicity.
Hydroxychloroquine should be used with caution in liver or renal impairment, and regular blood count monitoring to check for agranulocytosis and thrombocytopenia and liver function is needed. The British Royal College of Ophthalmologists advises that patients should be asked about visual impairment before starting treatment, and that visual acuity should be measured and recorded. If eye disease is present, an ophthalmologist should be consulted before starting treatment.
Patients should be asked about visual symptoms during treatment and visual acuity monitored annually. If treatment is required for over 5 years, individual arrangements should be made with the local ophthalmology service. Hydroxychloroquine should be used in caution in glucosephosphate G6PD deficiency as it may precipitate acute hemolytic anemia. As deficiency is highly prevalent in Africans, in whom persistent and severe sarcoidosis is more common, many physicians check G6PD levels before starting hydroxychloquine.
Cyclosporin A is a T cell suppressor which has been reported to improve neurosarcoidosis in two retrospective studies. However, a randomized controlled trial in 37 patients with pulmonary sarcoidosis treated over 18 months showed no benefit in terms of breathlessness or lung function, and side-effects were significantly greater in the treatment group. Cyclophosphamide is an alkylating agent which has been used with apparent benefit in cardiac and neurosarcoidosis, but there are no controlled studies in pulmonary disease.
Routine use cannot therefore be recommended. Mycophenolate mofetil MMF is an antiproliferative immunosuppressant like azathioprine, but is metabolized to mycophenolic acid, which has a more selective action than azathioprine. Several authors report employing MMF successfully as a steroid-sparing agent in extra-pulmonary sarcoidosis, but there are no controlled studies in pulmonary disease, and there are therefore currently insufficient data to recommend its use. Individual case reports and small series support the use of thalidomide in cutaneous sarcoidosis, but there are no studies in pulmonary disease.
Teratogenic concerns strictly limit its drug in women of child-bearing age, and side-effects can be troublesome. Pentoxifylline in high doses has been shown to improve lung function in mild pulmonary sarcoidosis. Since then a series of case reports and small series have reported the effectiveness of infliximab for treating various manifestations, including skin, eye, brain, lung, sinus and muscle involvement.
Two larger studies were reported in The first, by Doty and colleagues, 40 was a retrospective study of ten patients with sarcoidosis refractory to conventional agents. Six patients had lung involvement although the indication for using infliximab in these patients was extra-pulmonary disease. Nine patients reported symptomatic improvement with infliximab treatment, and all demonstrated objective evidence of improvement. In five of six patients taking concomitant corticosteroids the dose was reduced. The authors did not comment on lung function or radiology.
They nevertheless concluded that infliximab appeared safe and effective in refractory sarcoidosis. The second study, conducted by Baughman and colleagues, 41 was a phase II, multi-center, double-blind, placebo-controlled clinical trial in which patients were randomized in a 1: Patients were followed through to week In all cases the indication for entering the study was refractory pulmonary disease. Patients in the infliximab groups combined had a mean increase in forced vital capacity FVC of 2.
Post-hoc exploratory analyses suggested that patients with more severe disease longer disease duration, lower FVC or more symptoms seemed to benefit the most. Although these benefits appear modest, they would be significant in patients with life-threatening fibrotic disease who would potentially also be candidates for transplantation. Even stability in such patients represents a significant treatment response, and improvement would be exceptional. Etanercept has not been found to be as effective in sarcoidosis, possibly because infliximab achieves greater tissue penetration and cell mediated lysis of TNF-secreting cells.
Infliximab may therefore be considered in life-threatening pulmonary sarcoidosis when all other options have been exhausted. It is expensive, and patients need careful assessment beforehand for evidence of tuberculosis. Opportunistic infections are a potential consequence of immunosuppression. Prophylaxis against pneumonia caused by Pneumocystis jiroveci should be given to all patients with a history of the infection, and should be considered for severely immunocompromised patients.
North American guidelines for preventing opportunistic infection in HIV-infected individuals, 42 endorsed by the British Infection Society, provide the basis for current recommendations. Oral co-trimoxazole is the drug of choice for prophylaxis, either mg daily or three times a week. The dose can be reduced to mg daily to improve tolerance. In patients unable to tolerate co-trimoxazole, nebulized pentamidine is effective, as is oral dapsone; atovaquone has also been employed. Co-trimoxazole and dapsone can cause bone marrow suppression and skin rashes among other side-effects.
Neither North American nor British sarcoidosis guidelines recommend pneumocystis prophylaxis routinely in otherwise immuno-competent patients. A small number of patients with severe and progressive pulmonary sarcoidosis, despite exhaustive medical therapy, may be candidates for transplantation. Patients must be assessed for the potential presence of bronchiectasis, right ventricular impairment, infection, and mycetomas before proceeding.
Possible reasons include reduced access to healthcare before and after surgery, increased graft loss due to immunological hyper-responsiveness, and greater major histocompatibility polymorphism. Similar findings are reported in the setting of renal and liver transplantation. The authors concluded that patients with progressive symptomatic disease, or asymptomatic patients with infiltrates on CXR and progressively worsening lung function, should probably be treated.
It suggests that inhaled steroids may be of value in airway disease. In patients who fail to respond to steroids after three months, it suggests considering other reasons for failure such as the presence of irreversible fibrosis, noncompliance, or inadequate dosage. The British Thoracic Society BTS interstitial lung disease ILD 16 guidelines conclude that treatment is not indicated for asymptomatic stage I disease, nor in asymptomatic patients with stage II or III disease with mild lung function abnormalities and stable parameters.
They recommend oral corticosteroids as first-line therapy in patients with disease progression as indicated by radiology or lung function or significant symptoms. The authors recommend an initial dose of 0. Inhaled steroids are not recommended as initial or maintenance treatment, but may be considered for symptom control in patients with troublesome cough. Table 2 summarizes the main points from the two guidelines. Bisphosphonates are advised as appropriate to minimize steroid-induced osteoporosis.
Management strategies for pulmonary sarcoidosis
Alternative immunosuppressants are recommended when corticosteroids are not controlling the disease or when side effects are intolerable, with methotrexate the agent of choice. Since low carbon monoxide transfer factor TLCO measurements predict survival in interstitial lung disease, they are a useful guide to the timing of referral for lung transplantation.
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In summary, there is agreement that oral corticosteroids should be considered in patients with severe, persistent or progressively worsening respiratory symptoms, or declining lung function. Many physicians would monitor lung function VC and TLCO for 6 months before deciding that there is progressive deterioration.
There is agreement that neither oral nor inhaled corticosteroids are indicated in asymptomatic patients in the absence of other organ involvement. There is agreement that, provided the patient responds, the dose should then be reduced gradually to a maintenance dose. BTS recommendations do not advise inhaled steroids as initial or maintenance treatment, but suggest they may have a place in managing selected patients to control cough.
With regard to monitoring for adverse effects, potentially serious side-effects are well recognized and the principles used in other clinical settings generally apply. National evidence-based guidelines for the prevention of steroid-induced osteoporosis provide valuable advice. Physicians may however wish to avoid prescribing calcium and vitamin D supplements in patients with sarcoidosis unless they also monitor serum and urine calcium levels.
The dose of calcium and vitamin D can then be tailored according to these parameters. With bisphosphonates, clinicians should be aware of the risk of osteonecrosis of the mandible and be ready to advise patients to have an initial dental examination and appropriate preventive dentistry. When treating females of child-bearing age, they should also be aware that these agents are not recommended in pregnancy.
Current British ILD guidelines recommend considering other immunosuppressive agents when corticosteroids are not controlling the disease, or when side-effects are unacceptable, with methotrexate the agent of choice. Lung transplantation should be considered in end-stage pulmonary sarcoidosis. Sarcoidosis is the commonest ILD and is often managed by respiratory specialists. Despite experience of over 50 years using oral corticosteroids, their long-term benefits remain unclear, as is the value of inhaled corticosteroids. General management strategies include clear patient information and smoking cessation advice.
Pulmonary rehabilitation, oxygen supplementation and palliative care may be appropriate in patients with severe pulmonary fibrosis. There is broad agreement on the initial dose, subsequent tapering and length of treatment. Monitoring for adverse effects requires particular attention to steroid-induced osteoporosis. Alternative immunosuppressant agents and lung transplantation remain options for patients with severe disease. National Center for Biotechnology Information , U. Ther Clin Risk Manag. Author information Copyright and License information Disclaimer. This is an Open Access article which permits unrestricted noncommercial use, provided the original work is properly cited.
Abstract Sarcoidosis is a systemic inflammatory condition with an unexplained predilection for the lung: Introduction Sarcoidosis is the commonest form of interstitial lung disease ILD. Diagnosis Multiple organ involvement is typical. Radiological appearances Pulmonary sarcoidosis is traditionally classified by chest radiograph CXR appearances, as originally proposed by Scadding. Table 1 Chest radiograph appearances in sarcoidosis. Open in a separate window. The inflammatory response The inflammatory response is characterized by activated macrophages and CD4 helper T lymphocytes, with a pattern of cytokine production most consistent with a Th1-type immune response triggered by antigen.
General management strategies Clear communication to patients about diagnosis and management is vital, given clinical and scientific uncertainties around the cause of the condition, treatment and prognosis, and the abundance of poor quality and sensationalist information available on the internet. Treatment with oral corticosteroids There are no clear criteria for initiating corticosteroid therapy for pulmonary disease. Treatment with inhaled corticosteroids Selroos and colleagues first reported the value of inhaled budesonide in an open study of 20 patients with pulmonary sarcoidosis.
Adverse effects of steroids A number of recent studies indicate that adverse events such as weight gain, skin thinning, sleep disturbance, osteoporosis and neuropsychiatric disorders occur not infrequently in patients taking corticosteroids, even in low dose. Alternative immunosuppressive agents A number of patients with severe or persistent sarcoidosis require treatment with alternative agents, usually in combination with corticosteroids but sometimes alone, either in those for whom corticosteroids are contra-indicated, or in those unable to tolerate the side-effects of steroids.
Methotrexate Methotrexate has been employed as a steroid-sparing agent for many years in the treatment of rheumatoid arthritis, and has recently emerged as one of the preferred second-line drugs for treating sarcoidosis.
Azathioprine Azathioprine is a purine analogue which has been shown in case series to improve chest x-ray appearances and breathlessness. Hydroxychloroquine Hydroxychloroquine like chloroquine is an anti-malarial agent. Cyclosporin A Cyclosporin A is a T cell suppressor which has been reported to improve neurosarcoidosis in two retrospective studies. Cyclophosphamide Cyclophosphamide is an alkylating agent which has been used with apparent benefit in cardiac and neurosarcoidosis, but there are no controlled studies in pulmonary disease. Mycophenolate mofetil Mycophenolate mofetil MMF is an antiproliferative immunosuppressant like azathioprine, but is metabolized to mycophenolic acid, which has a more selective action than azathioprine.
Adverse effects of immunosuppression Opportunistic infections are a potential consequence of immunosuppression. Lung transplantation A small number of patients with severe and progressive pulmonary sarcoidosis, despite exhaustive medical therapy, may be candidates for transplantation. Table 2 Summary of current guidelines for treatment of sarcoidosis. Guidelines Indications for treatment Initiation: Conclusions Sarcoidosis is the commonest ILD and is often managed by respiratory specialists.
Footnotes Disclosure The author declares no conflicts of interest. Drent M, Costabel U, editors. Sarcoidosis in Denmark — A registry-based incidence study comprising patients. Sarcoidosis Vasc Diffuse Lung Dis. Incidence and mortality of idiopathic pulmonary fibrosis and sarcoidosis in the UK. Clinical characteristics of patients in a case control study of sarcoidosis Am J Respir Crit Care Med — Smoking and pulmonary sarcoidosis: Corticosteroid therapy for sarcoidosis.
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