A Misleading Diagnosis of Sarcoidosis in an Older Woman

Allaoui, Abire; Moudatir, M.; Echchilal, Khadija; Alaoui, F.Z.; Elkabli, H.

doi:10.12890/2017_000463

Cited by 5 publications

(4 citation statements)

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“…However, some of these patients may have suffered from GLILD. Indeed, there are several case reports describing patients who were misdiagnosed with sarcoidosis and who were frequently unresponsive to glucocorticoid monotherapy, similarly to the results described in this review (64)(65)(66).…”

Section: Strengths and Limitationssupporting

confidence: 77%

Treatment Strategies for GLILD in Common Variable Immunodeficiency: A Systematic Review

et al. 2021

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IntroductionBesides recurrent infections, a proportion of patients with Common Variable Immunodeficiency Disorders (CVID) may suffer from immune dysregulation such as granulomatous-lymphocytic interstitial lung disease (GLILD). The optimal treatment of this complication is currently unknown. Experienced-based expert opinions have been produced, but a systematic review of published treatment studies is lacking.GoalsTo summarize and synthesize the published literature on the efficacy of treatments for GLILD in CVID.MethodsWe performed a systematic review using the PRISMA guidelines. Papers describing treatment and outcomes in CVID patients with radiographic and/or histologic evidence of GLILD were included. Treatment regimens and outcomes of treatment were summarized.Results6124 papers were identified and 42, reporting information about 233 patients in total, were included for review. These papers described case series or small, uncontrolled studies of monotherapy with glucocorticoids or other immunosuppressants, rituximab monotherapy or rituximab plus azathioprine, abatacept, or hematopoietic stem cell transplantation (HSCT). Treatment response rates varied widely. Cross-study comparisons were complicated because different treatment regimens, follow-up periods, and outcome measures were used. There was a trend towards more frequent GLILD relapses in patients treated with corticosteroid monotherapy when compared to rituximab-containing treatment regimens based on qualitative endpoints. HSCT is a promising alternative to pharmacological treatment of GLILD, because it has the potential to not only contain symptoms, but also to resolve the underlying pathology. However, mortality, especially among immunocompromised patients, is high.ConclusionsWe could not draw definitive conclusions regarding optimal pharmacological treatment for GLILD in CVID from the current literature since quantitative, well-controlled evidence was lacking. While HSCT might be considered a treatment option for GLILD in CVID, the risks related to the procedure are high. Our findings highlight the need for further research with uniform, objective and quantifiable endpoints. This should include international registries with standardized data collection including regular pulmonary function tests (with carbon monoxide-diffusion), uniform high-resolution chest CT radiographic scoring, and uniform treatment regimens, to facilitate comparison of treatment outcomes and ultimately randomized clinical trials.

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Section: Strengths and Limitationssupporting

confidence: 77%

Treatment Strategies for GLILD in Common Variable Immunodeficiency: A Systematic Review

et al. 2021

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“…These patients can present with a clinical picture that mimics sarcoidosis and misdiagnosis can delay the treatment for CVID (IVIG), which in the presence of GLILD portends a poorer prognosis. Steroid therapy can further worsen CVID ( 208 , 210 – 212 ).…”

Section: Common Variable Immunodeficiency Syndrome (Cvid)mentioning

confidence: 99%

Sarcoidosis: Pitfalls and Challenging Mimickers

Narula

Iannuzzi

2021

Front. Med.

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Sarcoidosis, a systemic granulomatous disease of unknown etiology, may mimic other conditions at presentation often resulting in delayed diagnosis. These conditions include infections, neoplasms, autoimmune, cardiovascular, and drug-induced diseases. This review highlights the most common sarcoidosis mimics that often lead to pitfalls in diagnosis and delay in appropriate treatment. Prior to invasive testing and initiating immunosuppressants (commonly corticosteroids), it is important to exclude sarcoid mimickers.

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“…For CVID+EGD, steroid monotherapy was the most frequently reported regimen (21 of 53 treatment courses), with 85.7% of treatment courses scored as effective ( Table 5) (17,19,20,(25)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35)(36)(37)(38)(39)(40). For CVID+PGD, steroid monotherapy also was the most frequently reported treatment regimen (15 of 64 treatment courses); 66.7% of these treatment courses were associated with remission of granulomatous disease (Table 6) (29,(41)(42)(43)(44)(45)(46)(47)(48)(49)(50). Apart from monotherapy, steroids were frequently prescribed as part of a treatment regimen containing one or more other drugs, both in CVID+EGD and CVID+PGD.…”

Section: Administered Treatment Regimens In Granulomatous Disease In mentioning

confidence: 99%

What Works When Treating Granulomatous Disease in Genetically Undefined CVID? A Systematic Review

et al. 2020

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BackgroundGranulomatous disease is reported in at least 8–20% of patients with common variable immunodeficiency (CVID). Granulomatous disease mainly affects the lungs, and is associated with significantly higher morbidity and mortality. In half of patients with granulomatous disease, extrapulmonary manifestations are found, affecting e.g. skin, liver, and lymph nodes. In literature various therapies have been reported, with varying effects on remission of granulomas and related clinical symptoms. However, consensus recommendations for optimal management of extrapulmonary granulomatous disease are lacking.ObjectiveTo present a literature overview of the efficacy of currently described therapies for extrapulmonary granulomatous disease in CVID (CVID+EGD), compared to known treatment regimens for pulmonary granulomatous disease in CVID (CVID+PGD).MethodsThe following databases were searched: Embase, Medline (Ovid), Web-of-Science Core Collection, Cochrane Central, and Google Scholar. Inclusion criteria were 1) CVID patients with granulomatous disease, 2) treatment for granulomatous disease reported, and 3) outcome of treatment reported. Patient characteristics, localization of granuloma, treatment, and association with remission of granulomatous disease were extracted from articles.ResultsWe identified 64 articles presenting 95 CVID patients with granulomatous disease, wherein 117 different treatment courses were described. Steroid monotherapy was most frequently described in CVID+EGD (21 out of 53 treatment courses) and resulted in remission in 85.7% of cases. In CVID+PGD steroid monotherapy was described in 15 out of 64 treatment courses, and was associated with remission in 66.7% of cases. Infliximab was reported in CVID+EGD in six out of 53 treatment courses and was mostly used in granulomatous disease affecting the skin (four out of six cases). All patients (n = 9) treated with anti-TNF-α therapies (infliximab and etanercept) showed remission of extrapulmonary granulomatous disease. Rituximab with or without azathioprine was rarely used for CVID+EGD, but frequently used in CVID+PGD where it was associated with remission of granulomatous disease in 94.4% (17 of 18 treatment courses).ConclusionAlthough the number of CVID+EGD patients was limited, data indicate that steroid monotherapy often results in remission, and that anti-TNF-α treatment is effective for granulomatous disease affecting the skin. Also, rituximab with or without azathioprine was mainly described in CVID+PGD, and only in few cases of CVID+EGD.

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A Misleading Diagnosis of Sarcoidosis in an Older Woman

Cited by 5 publications

References 5 publications

Treatment Strategies for GLILD in Common Variable Immunodeficiency: A Systematic Review

Treatment Strategies for GLILD in Common Variable Immunodeficiency: A Systematic Review

Sarcoidosis: Pitfalls and Challenging Mimickers

What Works When Treating Granulomatous Disease in Genetically Undefined CVID? A Systematic Review

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