Iatrogenic flexor tendon rupture caused by misdiagnosing sarcoidosis-related flexor tendon contracture as tenosynovitis: A case report

BACKGROUND Sarcoidosis is a multisystem disease characterized by granuloma formation in various organs.Sarcoidosis-related flexor tendon contractures are uncommon in clinical settings.This contracture is similar to stenosing tenosynovitis and po-tentially leads to misdiagnosis and mistreatment.Herein,we report a rare case of sarcoidosis-related finger flexor tendon contracture that was misdiagnosed as tenosynovitis.A 44-year-old woman presented to our department with flexion contracture of the right ring and middle fingers.The patient was misdiagnosed with tenosynovitis and underwent acupotomy release of the A1 pulley of the middle finger in an-other hospital that resulted in iatrogenic rupture of both the superficial and profundus flexors.Radiological presentation showed multiple sarcoid involve-ments in the pulmonary locations and ipsilateral forearm.A diagnosis of sar-coidosis was made based on the presence of non-caseating granulomas with tubercles consisting of Langhans giant cells with lymphocyte infiltration on biopsy,and the patient underwent surgical repair for the contracture.After 2 mo,the patient experienced another spontaneous rupture of the repaired middle finger tendon and underwent surgical re-repair.Satisfactory results were achieved at the 10 mo follow-up after reoperation.CONCLUSION Sarcoidosis-related finger contractures are rare;thus,caution should be exercised when dealing with such patients to avoid incorrect treatment.

Renal Involvement in Sarcoidosis

Introduction: Sarcoidosis is a granulomatous systemic disease. Renal damage is rare but it can evolve to chronic renal failure (CRF). The aim of our work is to describe the clinical, paraclinical, histological and evolutionary profile of renal involvement during sarcoidosis and to identify the progression factors leading to CRF. Materials and Methods: This is a retrospective descriptive study from January 2009 to December 2022. We collected the medical records of patients with sarcoidosis and renal involvement. To study the progression factors leading to CRF we identified two groups of patients: the group with normal renal function and the group that developed CRF. Results: We included in our study 17 patients with renal sarcoidosis. Their mean age was 45 ± 14.7 years and the sex ratio was 0.4. Renal involvement is revealing of sarcoidosis in 76% of cases. Renal failure was diagnosed in 88% of patients. Proteinuria was positive in 76.5% of cases, leukocyturia in 59% of patients and hematuria in 41% of patients. Renal biopsy was performed in 15 patients showing tubulointerstitial nephropathy in all biopsies. Epithelioid and gigan-to-cellular granuloma without caseous necrosis was found in 46% of cases. Associated glomerular involvement such as segmental and focal hyalinosis was found in 2 patients. Corticosteroid therapy was initiated in 88% of patients for a median duration of 15 months. Normalization of renal function was achieved in 41% of patients, while 59%, i.e. 10 patients, retained a CRF, including 2 who were on dialysis. We showed a statistically significant relationship between the evolution towards CRF and the presence of interstitial fibrosis > 25%. Conclusion: Despite its rarity, renal involvement can be revealing of sarcoidosis, which can condition the prognosis and lead to CRF. Its detection allows an early diagnosis and treatment.

Exercise-induced torsades de pointes as an unusual presentation of cardiac sarcoidosis: A case report and review of literature

BACKGROUND Sarcoidosis is a rare multisystem disease characterized histologically by noncaseating granuloma formation in the affected organ.While cardiac sarcoidosis is found on autopsy in up to 25%of sarcoidosis cases,it is still underdiagnosed and is associated with a poor prognosis.Although the etiology of sarcoidosis remains unclear,an antigen triggered exaggerated immune response has been hypothesized.Early detection and prompt management of cardiac sarcoidosis remains pivotal.CASE SUMMARY A 60-year-old female,with pulmonary sarcoidosis in remission,presented to the cardiology outpatient clinic for evaluation of weeks-long dyspnea on moderate exertion(New York Heart Association class II)that was relieved by rest.Submaximal exercise stress test showed multifocal ventricular extrasystoles,followed by a self-limiting torsades de pointes.Cardiac magnetic resonance imaging showed nondilated and normotrophic left ventricle with basoseptal and mid-septal dyskinesis.The magnetic resonance imaging-derived left ventricular ejection fraction was 45%.Delayed enhancement showed patchy transmural fibrosis of the septum and hyperenhancement of the papillary muscles,all in favor of extensive cardiac involvement of sarcoidosis.A double-chamber implantable cardiac defibrillator was implanted,and methylprednisolone(12 mg/d)and methotrexate(12.5 mg/wk)treatment was initiated.Follow-up and implantable cardiac defibrillator interrogation showed episodes of asymptomatic nonsustained ventricular tachycardia and an asymptomatic episode of nonsustained ventricular tachycardia ending by the first antitachycardia pacing run.CONCLUSION Along an extensive review of the literature,this unusual case report highlights the importance of early detection of cardiac involvement of sarcoidosis,in order to avoid potential complications and increase survival.

Neurosarcoidosis: An Illustrative Case and Review of Perioperative Considerations

Sarcoidosis is a multisystem inflammatory granulomatous disease of unknown etiology. Neurosarcoidosis (NS) is a rare but potentially devastating manifestation of sarcoidosis, with a prevalence of approximately 5% in patients with systemic sarcoidosis. Due to the possible involvement of any part of the nervous system, a myriad of neurological manifestations can occur. Clinical features resulting from involvement of the hypothalmo-pituitary axis and cranial nerves, in particular, cranial nerve VII are the more common presentations of this condition. Medical therapy with corticosteroids is the mainstay of treatment and providing tissue for diagnosis remains the principal indication for surgery. Therapeutically, neurosurgery is indicated only for life-threatening complications. We describe the clinical case of a patient with fatally progressive NS who had multiple anesthetic exposures. This case highlights the perioperative considerations of NS and its anesthetic implications.

The Role of Gallium (Ga-67) Scintigraphy in the Diagnosis of Sarcoidosis

Purpose: To identify and interrogate the role of Gallium-67 scintigraphy in the diagnosis of Sarcoidosis. Methods: A retrospective study of 75 patients with suspected Sarcoidosis in a large teaching hospital with a specialist Sarcoidosis Centre. Results: Ga-67 scintigraphy in patients considered on clinical grounds to have Sarcoidosis is a useful and sensitive test, and furthermore, by relaxing the interpretation of gallium scintigraphy sensitivity may be increased (from 20% to 80%) for a relatively small drop in specificity. Conclusions: The sensitivity of diagnostic power may be increased with a change in diagnostic criteria at the expense of only a small drop in specificity. In the context of Neurosarcoidosis, our results support the theory that Ga-67 scintigraphy has value in identifying potential, asymptomatic biopsy sites outside of the CNS that may not be identified by other forms of imaging.

Cranial neuropathies in sarcoidosis

Sarcoidosis is a multisystem, chronic inflammatory disease that is characterized by the development of non-caseating granulomas in multiple body tissues and organ systems. Neurological complications of systemic sarcoidosis include peripheral and cranial neuropathies, myopathies, seizures, gait dysfunction, and cognitive decline. Because sarcoidosis has a predilection to involve the basilar meninges, cranial neuropathy is the most prevalent neurological deficit seen when the nervous system is involved. Sarcoidosis cranial neuropathy may occur at different stages of the disease and even as the initial clinical manifestation of central nervous system involvement. Attributing a cranial neuropathy to sarcoidosis can be challenging, particularly in the setting of normal imaging studies. In this review, cranial neuropathies in sarcoidosis are discussed in detail.

Gastrointestinal sarcoidosis associated with pneumatosis cystoides intestinalis

A 39-year-old male reported fevers,weight loss,watery loose stools,and decreased visual acuity in his right eye over the prior five years.He was pancytopenic,had an elevated American council on exercise level,total bilirubin,and alkaline phosphatase.Computed tomography revealed massive hepatosplenomegaly and emphysematous lung changes.Liver biopsy showed non caseating granulomas.The patient was diagnosed with extrapulmonary sarcoidosis and was treated with prednisone.The patient symptomatically improved but 5 mo later presented with abdominal pain caused by perforation of the cecum.He underwent a cecectomy and pathology revealed pneumatosis cystoides intestinalis.This represents the first reported association between pneumatosis cystoides intestinalis and sarcoidosis.The etiology of pneumatosis cystoides intestinalis in this case was likely multifactorial and involved both effects of the corticosteroids as well as the advanced nature of the gastrointestinal sarcoidosis.Furthermore this case has the unique features of emphysematous lung changes and pancytopenia which are uncommon with sarcoidosis.

Subcutaneous sarcoidosis of the upper and lower extremities: A case report and review of the literature

BACKGROUND Sarcoidosis is a granulomatous disease of unknown etiology that most often impacts the lungs.Cutaneous manifestations of sarcoidosis are seen among 9%- 37% of patients.Subcutaneous sarcoidosis is a rare presentation of cutaneous sarcoidosis with estimates of frequency ranging from 1.4%-16%.To date,very few articles and case reports have been written about this subject.In this paper,we describe a case of subcutaneous sarcoidosis and perform a review of the literature to determine if there are commonalities among patients who present with subcutaneous sarcoidosis.CASE SUMMARY A 38-year-old female,with a past medical history of arthritis and recurrent nephrolithiasis,presents with an 8-mo history of 4 firm,asymptomatic,skincolored nodules on her left and right upper extremities and neck.Needle biopsy and post-excisional pathology report both revealed well-formed,dense,noncaseating granulomas localized to the subcutaneous tissue.Chest computed tomography revealed mild mediastinal lymphadenopathy.A diagnosis of subcutaneous sarcoidosis was made,and the lesions were surgically removed.CONCLUSION Commonalities among patients presenting with subcutaneous sarcoidosis include: middle-aged female,lesions localizing to the upper or lower limbs,lymphadenopathy or pulmonary infiltration on chest imaging,elevated serum angiotensin-converting enzyme.

Intra-cardiac distribution of late gadolinium enhancement in cardiac sarcoidosis and dilated cardiomyopathy

Cardiac involvement of sarcoid lesions is diagnosed by myocardial biopsy which is frequently false-negative,and patients with cardiac sarcoidosis(CS) who have impaired left ventricular(LV) systolic function are sometimes diagnosed with dilated cardiomyopathy(DCM).Late gadolinium enhancement(LE) in magnetic resonance imaging is now a critical finding in diagnosing CS,and the novel Japanese guideline considers myocardial LE to be a major criterion of CS.This article describes the value of LE in patients with CS who have impaired LV systolic function,particularly the diagnostic and clinical significance of LE distribution in comparison with DCM.LE existed at all LV segments and myocardial layers in patients with CS,whereas it was localized predominantly in the midwall of basal to mid septum in those with DCM.Transmural(nodular),circumferential,and subepicardial and subendocardial LE distribution were highly specific in patients with CS,whereas the prevalence of striated midwall LE were high both in patients with CS and with DCM.Since sarcoidosis patients with LE have higher incidences of heart failure symptoms,ventricular tachyarrhythmia and sudden cardiac death,the analyses of extent and distribution of LE are crucial in early diagnosis and therapeutic approach for patients with CS.

Ultrasound imaging of abdominal sarcoidosis: State of the art

Since it has been recognized that sarcoidosis(SA) is not an exclusive disorder of the lungs but can also affect other organs such as the liver and spleen, efforts have been made to define specific imaging criteria for the diagnosis of the single organ involvement, and the concept has been reinforced that the exclusion of alternative causes is important to achieve the correct diagnosis. Ultrasound(US)is a useful tool to evaluate patients with suspected abdominal SA, such as of the liver, spleen, kidney, pancreas and other organs, showing findings such as organomegaly, focal lesions and lymphadenopathy. While the diagnosis of abdominal SA is more predictable in the case of involvement of other organs(e.g.,lungs), the problem is more complex in the case of isolated abdominal SA. The recent use of contrast-enhanced ultrasound and endoscopic ultrasound elastography has provided additional information about the enhancement patterns and tissue rigidity in abdominal SA. Here we critically review the role of US in abdominal SA, reporting typical findings and limitations of current evidence and by discussing future perspectives of study.

Sarcoidosis imitating breast cancer metastasis:a case report and literature review

Sarcoidosis is a benign systematic granulomatous disorder of unknown etiology and is associated with various malignancies.However, granulomatous and metastatic lymph node lesions are difficult to distinguish even when using precise and modern diagnostic methods, such as positron emission tomography. Thus, histological verification is the only method that can be used to accurately describe the nature of this disease. In this article, we report a case of non-luminal HER-2/neu-positive breast cancer in a patient without history of sarcoidosis and suspected to have metastatic disease.

Ductopenia related liver sarcoidosis

Sarcoidosis is a systemic granulomatous disease which may involve many organs.In approximately 95% of patients there is liver involvement,with noncaseating hepatic granulomas occurring in 21 to 99% of patients with sarcoidosis.Liver involvement is usually asymptomatic and limited to mild to moderate abnormalities in liver biochemistry.The occurrence of jaundice in sarcoidosis is rare;extensive imaging procedures and the examination of liver biopsies permit a precise diagnostic.Ductopenia associated with sarcoidosis has been reported in less than 20 cases and can lead to biliary cirrhosis and liver-related death.We report here on a case of ductopenia-related sarcoidosis in which primary biliary cirrhosis and extrahepatic cholestasis have been carefully excluded.The patient follow up was 8 years.Although ursodesoxycholic acid appears to improve liver biochemistry it does not preclude the rapid occurrence of extensive fibrosis.A review of the literature of reported cases of ductopenia related to sarcoidosis is provided.

Historical transition of management of sarcoidosis

Sarcoidosis is a systemic granulomatous disease of undetermined etiology, and it primarily affects the lungs and lymphatic system but may involve other organs. Recently, there have been several new insights in Japanese patients. The frequency of cardiac, ocular, and cutaneous sarcoidosis has increased in Japan, whereas, the proportion of patients with bilateral hilar lymphadenopathy decreased from 1960 to 2004.Propionibacterium acnes(P. acnes) has been studied extensively as one of the causative microorganism for granuloma formation, particularly in Japan. P. acnes-specific monoclonal antibodies are useful for diagnosing sarcoidosis. The potential association between smoking and sarcoidosis has been evaluated in a Japanese study, which found a higher prevalence of sarcoidosis among young smokers than that in previous reports. Recently, 18F-fluorodeoxyglucose positron-emission tomography, which permits visualization of activated inflammation, and endobronchial ultrasonography-guided transbronchial needle aspiration have been increasingly used to diagnose sarcoidosis. Cardiac sarcoidosis is found to be the main cause of death in Japan. The 2006 revised Japanese guidelines for diagnosing cardiac sarcoidosis are useful, particularly for subclinical cardiac sarcoidosis patients. Further studies and international communication and evaluation are needed to determine the causes of sarcoidosis, identify the risk factors for progressive disease, and develop new and effective treatments.

Systemic Sarcoidosis Associated to IgA Nephropathy

The sarcoidosis was rarely associated to IgA nephropathy. We report a 38-year-old man presented decreased visual acuity and xerostomia. He had two axillary lymphadenopathies and pitting edema of legs in physical examination. The ophthalmological examination revealed a right posterior uveitis. Biological investigations showed a mild renal insufficiency and elevated serum level of angiotensin-converting enzyme, β2 microglobulin and IgA. He had a proteinuria and a microscopic hematuria. The kidney echography was without abnormalities. Histological study of the renal biopsy found results in favor to IgA nephropathy. Biopsies performed in accessory salivary gland and lymph nodes revealed non-necrotising epitheloid and gigantocellular granulomatous inflammation suggesting a sarcoidosis. The diagnosis of a sarcoidosis associated to IgA nephropathy was posed. The treatment was based on oral prednisolone with gradual tapering doses. He regained normal vision. The renal function had not worsened. No relapse of sarcoidosis was noted during our follow up.

Colonic sarcoidosis:Unusual onset of a systemic disease

Sarcoidosis is a multisystem chronic inflammatory condition of unknown etiology that has the potential to involve every tissue in the body.Sarcoidosis in the gastrointestinal system,and particularly the colon,is very rare.Here,we report the case of a 57-yearold man with no previous diagnosis of sarcoidosis who presented with new onset of abdominal pain and constipation.A colonoscopy revealed that the abdominal pain was caused by an obstructing lesion in the cecum-ascending colon and lacked a clear histologic diagnosis.Radiologic investigation revealed concentric wall thickening of the cecum-ascending colon with multiple satellite lymphadenopathies,highly suggestive of a malignancy.The patient underwent a laparotomy and a right hemicolectomy was performed.A diagnosis of colonic sarcoidosis was made after the resected specimen was examined.Additionally,a chest computed tomography scan revealed lung involvement with atypical radiologic features in the absence of respiratory symptoms.Only histologic examination of the surgical specimen can yield a diagnosis of gastrointestinal sarcoidosis due to the non-specificity of endoscopic and radiologic findings.

A case of sarcoidosis associated with chronic eosinophilic pneumonia

A 78-year-old woman was referred to our university hospital due to dry cough, fever, and weight loss. Laboratory revealed blood eosinophilia, high ESR, CRP and LDH. Rheumatologic markers were negative. Chest X-ray showed ground glass opacities in upper lobes. Uveitis developed in the left eye 1 week after admission. Thorax CT revealed ground glass opacities, reticular appearence in the upper lobes, enlarged axillary and mediastinal lymph nodes. Bronchoalveolar lavage CD4/CD8 was 2.1 and had 48% eosinophils. Histopathologic examination revealed eosinophilic infiltration and non-caseating epitheloid granulomas in the lung and mediastinal lymph nodes without vasculitis. Symptoms, blood eosinophilia and initial thorax CT findings improved after two months of methylprednisolone treatment. Chronic eosinophilic pneumonia is a rare disease characterized by systemic and pulmonary manifestations. It may be associated with other diseases.

Fulminant isolated cardiac sarcoidosis with pericardial effusion and acute heart failure: Challenging aspects of diagnosis and treatment

This case report illustrates challenging aspects of diagnosis and treatment of isolated sarcoid heart disease(SHD) and the role of cardiovascular magnetic resonance(CMR) imaging. Here, we present a previously healthy 45-year-old man, who was admitted with pericardial effusion and symptoms of acute heart failure. CMR followed by targeted left ventricular endomyocardial biopsy(EMB) revealed the diagnosis of isolated SHD. The combined use of CMR and EMB was crucial in diagnosing SHD. Furthermore, this case report demonstrates the value of CMR for monitoring response to therapy and lesion healing.

A histologically proven case of progressive liver sarcoidosis with variceal rupture

Sarcoidosis is a chronic multi-systemic granulomatous disease,and liver involvement frequently occurs.in most cases,no evidence of liver dysfunction is ob-served,and portal hypertension due to sarcoid liver diseases is a rareoccurrence.Moreover,no case of liver sarcoidosis has ever been reported with confirma-tion of the disease progression.Herein we describe a patient having hepatic sarcoidosis with severe portal hypertension and liver dysfunction.The diagnosis was histologically confirmed from granulomatous status to established liver cirrhosis over 10 years.A 46-year-old woman developed massive hematemesis due to the rupture of gastric cardial varices.She underwent emer-gency endoscopic injection sclerotherapy,and clear evi-dence of chronic hepatic failure.Twelve years ago,she was diagnosed as having sarcoidosis with respiratoryclinicalsymptoms.Liver biopsy revealed asymptomatic incidental granulomas without fibrosis development.After a couple of years,features of liver dysfunction were manifest and progressed.Ten years after the first biopsy,a second liver biopsy was performed,and well established dense fibrosis was revealed.Although significant liver dysfunction with portal hypertension is rarely seen in sarcoidosis,this case indicates that we have to consider the possibility that sarcoidosis may cause end-stage liver cirrhosis.

Cardiac Sarcoidosis:Sorting Fact from Fiction in This Rare Cardiomyopathy

Sarcoidosis is a rare condition of granulomatous infi ltration of many tissues of the body,including the heart.Cardiac sarcoidosis has been challenging to study,as it is often asymptomatic,although the initial presentation can be sudden cardiac death.The incidence and prevalence rates have been diffi cult to establish and no expert agreed upon guidelines for diagnosis and management of cardiac sarcoidosis exist,and clinical manifestations are varied.The pathophysiology of granuloma formation in the myocardium as well as other tissues is governed by immune response to some environmental antigen.Genetics is also thought to play a role,although gene alterations have not been extensively studied,and no specifi c set of genetic mutations has been identifi ed to aid in identifi cation of individuals at risk of developing disease.Epigenetic factors likely play a signifi cant role in modulation of gene expression with respect to immune response.There is no standardized screening tool for the identifi cation of cardiac sarcoidosis.The presence of systemic sarcoidosis and new-onset third-degree heart block or ventricular arrhythmias warrants further investigation for cardiac sarcoidosis.MRI and PET are useful in helping to identify cardiac sarcoidosis but are not stand-alone tests.Endomyocardial biopsy is the gold standard but has a low yield owing to the patchy nature of granuloma formation in the myocardium.Therapy should be instituted early and involves immunosuppressive therapy with predominant use of corticosteroids.Arrhythmias,either ventricular or high-grade heart blocks,are managed with device therapy.Clinical presentation may warrant use of antiarrhythmic agents and/or catheter ablation.Survival and disease prognosis are dependent on early diagnosis and treatment.This review details the current understanding of cardiac sarcoidosis and highlights diagnostic strategies and treatment with the aim of guiding the clinician to early identifi cation of patients and implementation of appropriate management in this rare disease entity.

Diagnostic value of elevated serum carbohydrate antigen 125 level in sarcoidosis

BACKGROUND Sarcoidosis is a multisystem disorder with unknown etiology, and it predominantly affects the lungs and intrathoracic lymph nodes. For patients with atypical clinical manifestations, the diagnosis of sarcoidosis is difficult and specific biomarkers may play an important role in assisting diagnosis. Previous research has demonstrated a correlation between sarcoidosis and increased carbohydrate antigen 125(CA125), but remains a lack of large cohort studies to validate this observation.AIM To compare serum CA125 levels in sarcoidosis patients and healthy controls, and explore whether CA125 can be used as a biomarker for the diagnosis of sarcoidosis.METHODS In this study, the serum CA125 levels were measured by enzyme-linked immunosorbent assay in 108 consecutive sarcoidosis patients between June 2016 and December 2020(31 males, 77 females;age at diagnosis 49.69 ± 9.10 years) and 112 healthy subjects. Data on the C-reactive protein, erythrocyte sedimentation rate, and angiotensin-converting enzyme were also collected. The association of serum CA125 levels with clinical, radiological, and respiratory functional characteristics was analyzed between patient groups with CA125 ≤ 35 U/mL or CA125 >35 U/mL.RESULTS We found that serum CA125 levels were higher in sarcoidosis patients compared to healthy controls(median: 44.78 vs 19.11 U/mL, P < 0.001). The area under the receiver operator characteristic was 0.9833(95%CI: 0.9717-0.9949), and the best cutoff point was 32.33 U/mL. The elevated serum CA125 was notably associated with the percentage of predicted forced vital capacity(FVC%) and neutrophil-to-lymphocyte ratio(P =0.043 and P = 0.038, respectively) in sarcoidosis patients. Multivariate analysis revealed that FVC%was a statistically notable predictor of elevated serum CA125(P = 0.029). Also, our research revealed that compared to patients with Stage I of radiology classification, patients with Stage Ⅱ and Ⅲ showed a higher concentration of serum CA125(46.16 ± 8.32 vs 41.00 ± 6.04 U/mL, P =0.005, and 47.92 ± 10.10 vs 41.00 ± 6.04 U/mL, P = 0.002, respectively).CONCLUSION Serum CA125 was highly increased in sarcoidosis patients and showed high efficiency for noninvasive diagnosis of the disease. In addition, abnormally elevated serum CA125 was correlated with pulmonary function and radiological Scadding’s classification of sarcoidosis.