Symptomatic Sarcoidosis Research Articles

2363 Symptomatic Hepatic Sarcoidosis in a Hispanic Patient With Severely Elevated Liver Biochemical Tests

INTRODUCTION: Sarcoidosis is a systemic granulomatous disease of unknown etiology that typically predominates as a respiratory disease process. Hepatic involvement is uncommon and reported in only 11.5% of cases and even rarer is symptomatic hepatic involvement that occurs in approximately 5 to 30% of those cases. Epidemiological studies show substantial variations in extrapulmonary involvement based on race, with few studies on the Hispanic population. This case report presents a Hispanic female who underwent extensive workup and was diagnosed with symptomatic hepatic sarcoidosis. CASE DESCRIPTION/METHODS: A 27-year-old Hispanic woman with history of a mediastinal mass presented to the emergency department with an abrupt onset of right upper quadrant (RUQ) abdominal pain and nausea. Liver biochemical tests were severely elevated in a hepatocellular pattern. A RUQ ultrasound showed a dilated common bile duct (CBD) with a questionable stone in the CBD. Next, a magnetic resonance cholangiopancreatography was performed and demonstrated a normal CBD without biliary ductal dilatation or choledocholithiasis. Workup for chronic liver disease was negative, as well as extensive workup for other causes (Table 1). Then a liver biopsy was performed with histology demonstrating scattered non-necrotizing granulomas (Figure 1) with negative stains for mycobacterial and fungal organisms. Review of past medical records revealed a biopsy of her mediastinal mass with histology showing a non-necrotizing granulomas (Figure 2). A diagnosis of sarcoidosis was established and she was started on oral prednisone and referred to pulmonology. DISCUSSION: A diagnosis of sarcoidosis involves a suggestive history, granulomas in two organs, negative staining for acid fast bacilli, and a lack of drug-induced disease. The majority of patients with hepatic granulomas have primary biliary cholangitis (PBC). Screening for anti-mitochondrial antibodies and elevated IgM levels is important as these are present in >90% of those with PBC. Infectious diseases should be ruled out including tuberculosis, AIDS-related diseases, Q fever, and Brucellosis. Race is the most important risk factor for developing extrapulmonary sarcoidosis, possibly due to involvement of the reticuloendothelial system. Hispanics account for 18.1% of the U.S population. It is possible that Hispanics are at increased risk for extrapulmonary sarcoidosis compared to whites, but more Hispanic epidemiology studies are warranted.

1460 An Unusual Histopathology of Colonic Polyps

INTRODUCTION: Sarcoidosis is a multi-system disorder most commonly known to involve lungs, eyes, skin and joints. Colonic involvement either in isolation or as a part of systemic disease has been reported in less than 20 cases. Here, we describe a patient who presented with abdominal pain, constipation, and weight loss and was found to have colonic sarcoidosis. CASE DESCRIPTION/METHODS: A 38-year-old woman presented with chronic left lower quadrant abdominal pain, constipation, and unintentional weight loss for a few months. Her past medical history significant for treated latent tuberculosis and uterine fibroids. She denied fever, diarrhea, melena or hematochezia. Physical exam revealed tenderness in the left lower quadrant and periumbilical region. Laboratory work up showed hemoglobin of 9.6 (11.7-14.9 g/dL). Her ACE levels were elevated at 120 (8-52 U/L). Chest xray showed scattered pulmonary nodules and bilateral hilar lymphadenopathy. CT scan demonstrated extensive porta hepatis, mesenteric, portocaval, retroperitoneal, and inguinal lymphadenopathy. Biopsy of inguinal lymph node showed granulomatous lymphadenitis but no malignancy. Acid fast bacilli and fungal stains were both negative. Further, colonoscopy revealed multiple sessile polyps in descending colon ranging between 2-8 mm (Figure 1), removed with excisional biopsy. Biopsy from the colon polyps revealed numerous non-caseating granulomas (Figure 2). She was diagnosed with multi-system symptomatic sarcoidosis and was started on prednisone with improvement of her symptoms. DISCUSSION: Gastrointestinal tract involvement by sarcoidosis is seen in less than 1% of cases, with stomach being the most commonly affected organ. Colonic sarcoidosis is extremely rare and has variable clinical presentation but abdominal pain is most commonly described. Endoscopic appearance is very nonspecific and includes polyps, mass like lesions, erosions, ulcers and focal nodularity. Diagnosis requires demonstration of non necrotizing granulomas in the involved organ, evidence of sarcoidosis in at least one other organ system and exclusion of other granulomatous disorders such as tuberculosis, fungal infections, inflammatory bowel disease and malignancy. While no therapy is warranted for inactive disease, steroids are the initial treatment of choice in symptomatic patients.

Repository corticotropin injection in patients with advanced symptomatic sarcoidosis: retrospective analysis of medical records.

Background:Repository corticotropin injection (RCI) has regulatory approval for many indications, including symptomatic sarcoidosis. This large case series of patients with advanced symptomatic sarcoidosis treated with RCI describes patient characteristics, RCI utilization patterns, concomitant therapies, and physicians’ assessments of treatment response.Methods:Patients ⩾18 years with symptomatic sarcoidosis, treated with RCI in the previous 36 months, who had completed a course of RCI or received RCI for ⩾6 months at the time of data collection were included.Results:The study included 302 patients (mean age, 51 years; 52%, women) with a mean 4.8 years since initial diagnosis of sarcoidosis. Most patients (76%) had extrapulmonary involvement, primarily in the skin (28%), joints (25%), heart (22%), and eyes (22%); 34% had multiple (⩾2) organ involvement. The mean duration of RCI treatment was 32.5 weeks, with 61.6% of patients continuing RCI therapy for ⩾6 months. The RCI utilization pattern indicated an individualized approach to therapy, with a higher starting dose associated with a shorter duration of therapy compared with a lower starting dose. The percentage of patients who used corticosteroids decreased from 61.3% during the 3 months before initiation of RCI to 12.9% 3 months after RCI therapy; the mean daily dose of corticosteroid decreased from 18.2 mg to 9.9 mg. The proportion of patients given <10 mg/day of prednisone increased from 21% before RCI use to 47% 3 months after RCI use. According to physicians’ assessments of change in patients’ health status after RCI therapy, overall status improved in 95% of patients, overall symptoms in 73%, lung function in 38%, and inflammation in 33%.Conclusions:The findings suggest that RCI is a viable treatment option for patients with advanced symptomatic sarcoidosis and provide insights on patient characteristics and practice patterns to help clinicians determine appropriate use. The reviews of this paper are available via the supplemental material section.

Corrigendum to "Spontaneous Resolution of Symptomatic Hepatic Sarcoidosis".

[This corrects the article DOI: 10.1155/2018/1535049.].

Gastric Sarcoidosis: A Rare Manifestation of the Disease

Sarcoidosis is a chronic systemic inflammatory disease of unclear etiology characterized by non-caseating granulomas. It can affect nearly every organ system. Alimentary tract sarcoidosis is atypical within which gastric sarcoidosis is the most common form. Involvement of the alimentary tract is usually as a part of systemic disease or rarely as an isolated finding. Symptomatic gastric sarcoidosis is further more distinct. We present a rare case of sarcoidosis initially presenting with gastrointestinal symptoms and without pulmonary symptoms. A 57 years old morbidly obese African American female with PMH of HTN and DM type II presented with epigastric abdominal pain, nausea, vomiting, and a six month history of unintentional 60lb weight loss secondary to early satiety and dysgeusia. She was found to have hypercalcemia of 13.4 mg/dL, AKI and lactic acidosis. A non-contrast CT and subsequent CTA of the abdomen and pelvis showed small bowel wall thickening, abdominal lymphadenopathy, patent celiac axis and mesenteric vessels, and numerous ˜1mm lung base nodules. EGD revealed erythematous mucosa of gastric antrum with erosions and shallow duodenal erosions (Image 1). Gastric biopsy revealed granulomatous inflammation. Duodenal biopsy showed mild villous blunting without granulomas. Stains for fungal elements and AFB was negative. No intestinal metaplasia, dysplasia or malignancy was noted. PTH, PTHrP, and TSH were normal. CT chest revealed centrilobular nodular interstitial opacities with extensive bilateral mediastinal and hilar lymphadenopathy (Image 2). FNA of right hilar lymphadenopathy under EBUS revealed epithelioid histiocytes and lymphocytes suggestive of granulomatous inflammation. No malignant cells were seen. Fungal and AFB stains and AFB culture were negative. PPD skin test was negative. Patient's GI symptoms improved with supportive care. Given the lack of pulmonary symptoms, corticosteroids were not started. Patient has continued to do well during subsequent clinic follow up. Gastric sarcoidosis is among the rarer subsets of the disease. Most cases have been described as posthumous discoveries on autopsy of known sarcoidosis patients as patients are usually asymptomatic. Biopsy proven pulmonary and gastric sarcoidosis presenting with GI symptoms and an anomalous lack of pulmonary symptoms is exceptionally rare and questions a broader understanding for consideration of sarcoidosis diagnosis.2704_A Figure 1. Erythematous mucosa of gastric antrum with erosions on EGD. H. pylori negative.2704_B Figure 2. CT Chest: Centrilobular nodular interstitial opacities throughout both lungs with moderate mediastinal, hilar, and upper abdominal lymphadenopathy

Sarcoidosis-related Uveitis: Clinical Presentation, Disease Course, and Rates of Systemic Disease Progression After Uveitis Diagnosis

To document the clinical presentation, treatment, and visual outcome of sarcoid uveitis and to determine the timing and potential risk factors of sarcoidosis progression to symptomatic systemic disease from the time of sarcoid uveitis diagnosis. Retrospective, interventional case series. Subjects: Patients with dual diagnoses of uveitis and presumed/biopsy-proven sarcoidosis. Retrospective review of 143 patient records from the Royal Victorian Eye and Ear Hospital and Eye Surgery Associates in Melbourne, Australia, between October 1990 and April 2014 coded with the dual diagnoses of uveitis and sarcoidosis. Only patients with uveitis and presumed or biopsy-proven sarcoidosis (N= 113) were included. Ascertainment of rate and time (months) to the development of symptomatic systemic sarcoidosis from uveitis onset; comparison of the patient demographics, characteristics of uveitis, treatment, and visual outcome between those who developed systemic sarcoidosis and those who remained systemically asymptomatic. Uveitis was the initial presenting complaint of sarcoidosis in 78.8% (n= 89). Twenty-three patients had concurrent undiagnosed systemic disease at presentation and 29 subsequently developed symptomatic sarcoidosis in an organ uninvolved at uveitis onset. The median time to the development of symptomatic systemic sarcoidosis was 12months. No statistically significant association was ascertained between any particular uveitis characteristic and extraocular sarcoidosis progression. Uveitis was the initial presentation of sarcoidosis in the vast majority of our subjects. Concurrent undiagnosed systemic sarcoidosis was common at the time of uveitis onset. A high index of suspicion for subsequent systemic progression should also be maintained, especially within the first 5 years of the uveitis diagnosis.

Symptomatic muscular sarcoidosis: Lessons from a nationwide multicenter study.

ObjectivesTo describe clinicopathologic features of muscular sarcoidosis and the associated sarcoidosis phenotype through a nationwide multicenter study.MethodsPatients were included if they had histologically proven sarcoidosis and symptomatic muscular involvement confirmed by biological, imaging, or histologic examinations.ResultsForty-eight patients (20 males) were studied, with a median age at muscular symptoms onset of 45 years (range 18–71). Four patterns were identified: a nodular pattern (27%); smoldering phenotype (29%); acute, subacute, or progressive myopathic type (35%); and combined myopathic and neurogenic pattern (10%). In all patterns, sarcoidosis was multivisceral with a median of 3 extramuscular organs involved (mostly lungs, lymph nodes, eyes, and skin) and a prolonged course with long-term use of corticosteroids and immunosuppressive drugs. Muscular patterns differed according to clinical presentation (myalgia, nodules, or weakness), electromyographic findings, muscular MRI, and response to sarcoidosis treatment. The myopathic and neuromuscular patterns were more severe.ConclusionThis nationwide study of muscular sarcoidosis allowed the identification of 4 patterns of granulomatous myositis, which differed by phenotypes and the clinical course.

Severe opportunistic infections in extrapulmonary sarcoidosis

Introduction: Sarcoidosis is a chronic multisystemic disease of unknown etiology characterized by noncaseating granulomas that most frequently affect the lungs, but frequently has extrapulmonary manifestations. Treatment of symptomatic sarcoidosis, glucocorticoids and immunosuppressants, may be associated with an increased risk of infection.

Severe infections in sarcoidosis: Incidence, predictors and long-term outcome in a cohort of 585 patients.

Sarcoidosis is associated with cell-mediated immunodeficiency and treatment of symptomatic sarcoidosis usually includes systemic immunosuppressants. Data relative to incidence, prognosis factors, and outcome of infections are scarce.Retrospective cohort study of 585 patients with biopsy proven sarcoidosis in a tertiary referral specialist clinic, with a nested case-control analysis. Twenty nine patients (4.9%) with severe infections were compared to 116 controls subjects with sarcoidosis, matched according to their gender, ethnicity, age at diagnosis, and treatment with corticosteroids.After a median follow-up of 8 years [range; 1–46], 38 severe infections [mycobacterial infections (n = 14), fungal infections (n = 10), bacterial (n = 8), viral (n = 3) and parasitic (n = 1)] were observed in 30 patients. The incidence of severe infections was 0.71% persons-year (CI 95% 0.5–0.98) and 0.43% persons-year (CI 95% 0.27–0.66). Patients with severe infection were more frequently of male gender (60% vs 46%) and were more likely treated by ≥ 3 immunosuppressive agents (OR = 3.8, IC 95% [1.5–9.64], P = .005) and by cyclophosphamide (OR = 5.55, IC 95% [1.9–16.1], P = .002), and with neurological (OR = 3.36 CI 95% [1.37–8.25], P = .008), or cardiac (OR = 2.65 CI 95% [1.09–6.43], P = .031) involvement of the sarcoidosis, compared to the controls. Two patients died within the 6 months following infection, due to progressive multifocal leucoencephalopathy (n = 1), and of peritonitis (n = 1).Severe infections are observed in 5.1% of our patients with sarcoidosis after a median follow-up of 8 years. Risk factors for severe infections included neurological or cardiac involvement of sarcoidosis, the use of immunosuppressive agents and mainly cyclophosphamide.

Correlation Between Parenchymal Findings on Computerized Tomography and Need for Symptomatic Treatment in Patients With Stage I Sarcoidosis

SESSION TITLE: Pulmonary Manifestations of Systemic Diseases SESSION TYPE: Original Investigation Slide PRESENTED ON: Wednesday, November 1, 2017 at 07:30 AM - 08:30 AM PURPOSE: Sarcoidosis is a multisystem disease most commonly affecting the lungs. Scadding stage I disease is defined as hilar lymphadenopathy without parenchymal involvement on chest radiography (CXR). Among patients with stage I disease the majority (60-90%) will have spontaneous regression without treatment. Prior studies have shown that the Scadding staging system underestimates the number of patients who have parenchymal involvement on Computerized Tomography (CT) scans. Our study sought to determine treatment rates in patients with stage 1 sarcoidosis by CXR and parenchymal disease on CT scan and to determine if certain parenchymal findings were associated with higher treatment rates. METHODS: This is a retrospective cohort study of 508 patients in a sarcoidosis database at a tertiary care center in the United States. Two thoracic radiologists independently reviewed and identified 19 patients with stage I disease by CXR who also had CT imaging, done within 3 months, with abnormal parenchymal findings. CT scans were analyzed for parenchymal findings including ground glass opacities (GGO), nodules and fibrosis. The electronic medical records of these patients were reviewed to identify those who needed treatment for pulmonary symptoms related to sarcoidosis. RESULTS: In our cohort of 19 patients, 84% were African-American and 68% were female. All but one patient with stage I disease by CXR (95%) had parenchymal findings on CT. On CT imaging, 12 (63%) patients had nodules only, 7 (37%) had GGO in combination with nodules or fibrosis. Of these 19 patients, 8 patients (42%) required treatment for symptomatic pulmonary sarcoidosis. On further characterization 4 (33%) of the 12 patients with nodules were treated and 4 (57%) of the 7 patients with GGO plus nodules or fibrosis were treated. CONCLUSIONS: We found a high treatment rate in our cohort of stage I sarcoidosis patients with parenchymal findings on CT. Those patients with GGO plus nodules/fibrosis on CT had higher treatment rates than those with nodules alone. This suggests that parenchymal findings on CT in stage I sarcoidosis patients may provide additional clinical value. CLINICAL IMPLICATIONS: Chest CT scans in patients with stage I sarcoidosis may have important prognostic implications and may help guide the clinician in making treatment decisions. DISCLOSURE: The following authors have nothing to disclose: Pratyaya Majumder, Anand Pariyadath, Robert Groves, Ennis James, Thomas Iden, Aamer Syed No Product/Research Disclosure Information

Clinical and Economic Evaluation of Repository Corticotropin Injection: A Narrative Literature Review of Treatment Efficacy and Healthcare Resource Utilization for Seven Key Indications.

IntroductionRepository corticotropin injection (RCI; H.P. Acthar® Gel; Mallinckrodt Pharmaceuticals Inc., Hampton, NJ) is a highly purified, prolonged-release porcine preparation of adrenocorticotropic hormone (ACTH) analogue that is FDA-approved for treatment of 19 autoimmune and inflammatory disorders. The diverse physiological actions of RCI at the melanocortin receptors (MCRs) affect processes involved in inflammation, pigmentation, steroidogenesis, and immunomodulation. Although RCI has been approved to treat inflammatory and autoimmune diseases for more than 60 years, recent progress in understanding both MCRs and the effects of RCI in modulating immune responses has led to increased interest in RCI as a therapeutic choice. The objective of this narrative literature review is to summarize key clinical and economic data on RCI treatment of seven disorders: infantile spasms (IS), multiple sclerosis (MS) relapses, proteinuria in nephrotic syndrome, rheumatoid arthritis (RA), dermatomyositis/polymyositis (DM/PM), systemic lupus erythematosus (SLE), and symptomatic sarcoidosis based on published literature and product information. An extended report is available as the Academy of Managed Care Pharmacy (AMCP) Formulary dossier for H.P. Acthar® Gel.MethodsKey studies of clinical efficacy and healthcare utilization and cost from 1956 to 2016 are summarized.ResultsThe evidence supports the efficacy of RCI across the seven indications. RCI is effective as a first-line therapy for IS. For the other six conditions, RCI may improve clinical outcomes during exacerbations or when the condition is resistant to conventional treatments. Use of RCI is associated with reduced use of biologics, corticosteroids, and disease-modifying antirheumatic drugs. Initiation of RCI therapy in patients with IS, MS, RA, SLE, or DM/PM has been associated with lower post-therapy healthcare utilization and medical costs, including decreases in hospitalizations, hospital length of stay, outpatient visits, and emergency department visits.ConclusionThe evidence suggests that RCI may improve inflammatory and autoimmune disease control and patient quality of life, particularly in complex patients, and yield healthcare cost savings that demonstrate the medicine’s value.FundingMallinckrodt Pharmaceuticals Inc.Electronic supplementary materialThe online version of this article (doi:10.1007/s12325-017-0569-9) contains supplementary material, which is available to authorized users.

Symptomatic pancreatic sarcoidosis

Objective: Report the case of female patient with symptomatic pancreatic sarcoidosis. Methods: Case Report. Results: A 65-year-old female was referred for the evaluation of severe fatigability, poor appetite, weight loss over the past two months and abdominal pain. She denied smoking cigarettes or alcohol intake. A review of systems was unremarkable. Her physical exam revealed the abdomen was soft, non-distended and non-tender with normal liver and spleen span. She presented with leukopenia, hypercalcemia, elevated liver enzyme an elevated alkaline phosphatase and an elevated erythrocyte sedimentation rate. An abdominal CT scan demonstrated a pancreatic head mass with retroperitoneal lymph node enlargement Magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERCP) were not performed. A CT scan guided pancreatic mass biopsy was performed with uncertain results. Laparoscopic pancreatic biopsy demonstrated noncaseating granulomas with Langerhan giant cells and fibrosis. Both an acid fast bacilli stain and gram stain for a fungal infection were negative. The patient presented elevated Angiotensin converting enzyme levels. Oral prednisone 40 mg daily for 7 days followed by a 7 week tapering schedule was initiated. The patient's signs and symptoms improved abdominal CT scan 6 weeks after treatment revealed no change in the size of the pancreas and peripancreatic lymph nodes. After 8 week of prednisone she remains clinically stable without any abnormal laboratory parameters. Conclusion: Sarcoidosis is a chronic multiorgan disease that uncommonly involves the pancreas. Pancreatic involvement is usually asymptomatic and discovered on autopsy. However, doctors should be cognizant that sarcoidosis can present with symptomatic pancreatic mass, albeit rarely.

Physician Preference or Clear-Cut Guideline? How to Manage a Case of Symptomatic Sarcoidosis of the Liver

Physician Preference or Clear-Cut Guideline? How to Manage a Case of Symptomatic Sarcoidosis of the Liver

A Rare Case of Abdominal Pain: Gastric Sarcoidosis With Quiescent Pulmonary Disease

A 77-year-old African American female presented with 3-weeks of epigastric abdominal pain, bilious emesis, and dysphagia to solids. Pain was sharp, mid-epigastric, 10/10 in severity, and was exacerbated by oral intake. The patient's past medical history included hypertension, coronary artery disease, and pulmonary sarcoidosis, which was diagnosed by bronchoscopy 21 years prior and treated with corticosteroids for one year. She was followed closely and was in remission for 20 years. On presentation, physical exam was significant for hyperactive bowel sounds. Lab work was within normal limits. The patient underwent endoscopy, which showed a single non-bleeding 6mm ulcer in the stomach body and mucosal erythema and nodularity in the antrum and stomach body. Biopsies revealed non-necrotizing granuloma in the gastric mucosa with mild chronic and focal acute inflammation. Gastric ulcer histology was benign. Stains were negative for H. pylori, acid-fast bacilli, and fungal organisms. The patient was started on oral prednisone 40mg daily for a diagnosis of gastric sarcoidosis. She was symptomatically improved after two days of treatment. Sarcoidosis is a chronic disease; however, about ⅔ of patients have remission within a decade after diagnosis, while only ⅓ have progressive disease that leads to organ damage. Less than 5% of patients have disease recurrence after they are in remission for more than 1 year. Recurrence of active disease in an extrapulmonary location after a prolonged period of latency from pulmonary sarcoid is extremely rare. Most cases of gastric sarcoidosis are asymptomatic and found on incidental biopsy. Symptomatic patients most often present with epigastric pain, vomiting, and weight loss. Endoscopic findings range from mucosal erythema and ulcers to nodular lesions to linitis plastica. Biopsy showing noncaseating granulomas is critical for diagnosis, but not pathognomonic. Granulomas can also be seen in Crohn's disease, tuberculosis, gastric malignancy, lymphoma, and idiopathic granulomatous gastritis. To establish a diagnosis of GI sarcoidosis, in addition to histology, other etiologies must be excluded. There also must be clinical, radiographic or histopathologic evidence of sarcoidosis in another organ system. If the GI tract is the only organ involved, sarcoidosis cannot be confirmed. Oral corticosteroids are the mainstay for treatment of symptomatic GI sarcoidosis. Improvement can be seen quickly, as shown in our patient. Surgery is reserved for refractory or life-threatening cases. This case highlights the importance of having GI sarcoidosis in the differential diagnosis for epigastric pain, despite quiescent pulmonary disease.Figure 1Figure 2Figure 3

Splenomegaly in sarcoidosis: Frequency, treatment, prognosis and long-term follow-up

The splenic involvement is common in sarcoidosis, but its real frequency is still obscure depending doubtless on the method of splenomegaly detection. Splenomegaly may be accompanied with pain or anemia, leucopenia and thrombocytopenia. The aim of this study was to investigate the frequency of splenomegaly related to clinical characteristics of sarcoidosis and to solve the dilemma--whether to introduce medicaments, and when to perform splenectomy. The method of the study is a retrospective and prospective analysis of the patients'material. The study included 540 patients with sarcoidosis in a 20-year period. Of them, 26% had splenomegaly detected by computerized tomography screening. Splenomegaly was more frequently registered in the patients with a longer history of sarcoidosis (38%), as compared to those with a shorter history of the disease (23%) (p < 0.05). Splenomegaly was more frequently registered in the patients with other extrapulmonary lesions detected (33%) than in those who had no extrapulmonary manifestations of sarcoidosis (17%) (p < 0.01). Indications, possible benefits and complications of splenectomy were analysed in 11 sarcoidosis patients undergoing this intervention for various reasons, of which the follow-up period ranged from one to 20 years. Splenomegaly was more frequent in chronic cases or in the patients with established sarcoid lesions of other extrapulmonary organs.The primary treatment of uncomplicated symptomatic splenic sarcoidosis includes medicamentous therapy. Occasionally, splenectomy is required. Prognostically, splenomegaly indicates an unfavorable course of the disease.

A Case of Gastrointestinal Sarcoidosis without Pulmonary Involvement

Sarcoidosis is a multisystemic granulomatous disease of unknown etiology that is based on the presence of non-caseating granulomas. Sarcoidosis commonly involves the lungs, skin, and eyes. Gastrointestinal tract involvement in sarcoidosis is uncommon. Gastrointestinal tract sarcoidosis commonly occurs subclinically, with clinical manifestations present in only 0.1-0.9% of patients with the disease. There are rare case reports of an individual with symptomatic gastrointestinal sarcoidosis. In Korea, gastrointestinal sarcoidosis without pulmonary involvement has not been reported previously. Here, we describe a case of gastrointestinal sarcoidosis in a 37-year-old male with symptoms of weight loss, fever, and abdominal pain. He was diagnosed based on the presence of non-caseating epithelioid granulomas in the stomach, duodenum, and colon by multiple biopsies. (Korean J Med 2015;89:127-132)

Osseous sarcoidosis: Clinical characteristics, treatment, and outcomes—Experience from a large, academic hospital

ObjectiveOsseous sarcoidosis has been infrequently reported. We aimed to characterize the distribution of lesions, clinical presentation, treatment, and outcomes for osseous sarcoidosis. MethodsCases of osseous sarcoidosis were identified by directed inquiry to clinicians and electronic query. Cases were defined as having pathologic evidence of non-caseating granulomas on bone biopsy or evidence of osseous lesions on imaging attributable to sarcoidosis in patients with known sarcoidosis. Detailed characteristics were obtained by medical record review. ResultsWe identified a total of 20 cases of osseous sarcoidosis. Osseous lesions were detected by imaging during the initial sarcoidosis presentation in 60% of cases. In those who had a prior diagnosis of sarcoidosis, the median duration of sarcoidosis before detection of osseous involvement was 4.3 years. Symptoms were present in 50% of cases. All cases had more than one bone involved. The axial skeleton was involved in the majority of cases (90%), primarily the pelvis and the lumbar spine. Most cases required no treatment (55%); a minority of cases (45%) were treated, most often with prednisone, methotrexate, or hydroxychloroquine. Two cases required multiple immunosuppressants, including tumor necrosis factor inhibitors, for refractory symptomatic osseous sarcoidosis. Treated cases were younger than those who were untreated. At last follow-up, most cases (85%) were asymptomatic from osseous lesions. ConclusionsIn this case series of osseous sarcoidosis from a single center, most patients had multiple bones affected and had other systemic manifestations of sarcoidosis. A minority required treatment for relief of symptoms, and most cases were asymptomatic at last follow-up.

Exercise Capacity, Muscle Strength, and Fatigue in Sarcoidosis: A Follow-Up Study

The purpose of this study was to examine changes in the prevalence of exercise intolerance, reduced muscle strength, and fatigue and the changes in these parameters in individual patients during a 2-year follow-up study. Ninety sarcoidosis patients (62 males and 28 females; mean age: 46.0±10.2years) participated in a 2-year follow-up study. At the baseline and follow-up measurements, patients performed a 6-min walk test and elbow flexor muscle strength, quadriceps peak torque, and hamstrings peak torque tests. Maximal inspiratory pressure was recorded. All patients completed the Fatigue Assessment Scale. Both at baseline and follow-up, a substantial proportion of the patients showed a reduced 6-minute walk test (41.6 and 34.8%, respectively), elbow flexor muscle strength (6.7 and 14.6%), quadriceps peak torque (21.3 and 18%), hamstrings peak torque (13.5 and 12.4%), and maximal inspiratory pressure (45.9 and 48.6%). The majority of the patients reported fatigue (86 and 77%). These physical impairments remained stable during the follow-up period. The prevalence of these physical impairments in patients diagnosed with sarcoidosis <2years before inclusion in this study was similar to that in patients with a longer history of the disease. Exercise intolerance, muscle weakness, and fatigue are frequent problems in symptomatic sarcoidosis patients with a stable and persistent character. This study highlights that beyond medical treatment a rehabilitation program should be considered as adjunct therapy in the multidisciplinary management of sarcoidosis patients even though the achieved benefit needs future studies.

A rare case of symptomatic gastric sarcoidosis

### Learning Point for Clinicians Always think about sarcoidosis in patient with multi-organ involvement. Endoscopic appearance of gastric sarcoidosis can be normal. The diagnosis requires biopsy. A 65-year-old Caucasian male presented to our institute with a 3-week history of anorexia, nausea and watery diarrhea accompanied by a 1-week history of fever, dry cough and mild shortness of breath. Physical examination was only remarkable for red subcutaneous nodules at left thigh. Initial laboratory investigations, including complete blood cell count, blood chemistry, liver function test and serum angiotensin converting enzyme, were within normal range. His stool studies including stool culture, stool for Giardia antigen, Cryptosporidium antigen and Clostridium difficile toxins were all negative. His initial chest X-ray was unremarkable but his computed tomography …

Gastric sarcoidosis in children: A case report and review of the literature

Sarcoidosis is a systemic granulomatous disease of unknown etiology that is characterized by the formation of noncaseating granulomas. Gastrointestinal (GI) tract involvement in sarcoidosis is rare. Gastric sarcoidosis, particularly involving the antrum, affects approximately 10% of patients with systemic disease. GI sarcoidosis commonly occurs subclinically, with clinical manifestations present in only 0.1–0.9% of patients with the disease.This is a rare case report of a 8year Saudi girl with symptomatic gastric sarcoidosis. The patient presented with anorexia, postprandial upper abdominal pain and fullness, and weight loss of 3months duration. She was presented acutely after 6months with attack of hematemesis. Endoscopic examination of upper gastrointestinal tract revealed bleeding nodular mucosal irregularities. Mucosal biopsies revealed noncaseating granulomatous inflammation involving the gastric mucosa confirming the diagnosis of sarcoidosis. Corticosteroid therapy was started and the symptoms abated almost immediately. We also offer a review of the literature.