Case Report: Sarcoidosis in a Patient with Diabetes
Robby FerrantePharmacy CandidateWestern New England CoP
Objectives
Evaluate patient presenting to the Big Y Health Clinic with history of sarcoidosis
Discuss the pathology of sarcoidosis
Review treatment options
Determine an ideal regimen for the patient
Patient Presentation
49 Year old AA male
Initial visit
Pleasant and energetic but disheveled
Newly diagnosed with Type 2 DM
Chief Concern: Help with food choices
Social History
Recently started a new job Lost previous job and was evicted due to
hospital stay Living in shelter/housing where he has a
bedroom with a roommate and a shared kitchen for the complex
Drinks a couple beers once or twice a week
Denies tobacco use, but smokes marijuana multiple times daily to stimulate appetite.
Past Medical History
Recent diagnosis with diabetes Coronary artery disease
2 stents placed this year following recent MI
Sleep apnea History of kidney stones Sarcoidosis
Manifestations in lungs, eyes, and skin
Medication List
Metformin XR 500 mg every morning Prasugrel 10 mg daily Metoprolol tartrate 75 mg twice a day Atorvastatin 80 mg daily ASA 81 mg daily Isosorbide mononitrate 30 mg daily Hydroxychloroquine 200 mg twice a
day Unknown ointment/cream for
sarcoidosis lesions
What is sarcoidosis?
Systemic disease of unknown cause characterized by formation of immune granulomas.
Often affects lungs and lymph system
Epidemiology
70 % of cases reported in ages 25-45
Incidence: 14-24 per 100,000 Prevalence: 8-102 per 100,000 Heritability?
Twin study 61,662 pairs of Danish and Finnish
twins 210 pairs had at least 1 probandValeyre D, Prasse A, Nunes H, Uzunhan Y, Brillet PY, Müller-Quernheim J. Sarcoidosis. Lancet.
2014 Mar 29;383(9923):1155-67Rybicki B, Ianuzzi M, Frederick M, et al. Familial aggregation of sarcoidosis. A case-control etiology study of sarcoidosis (ACCESS) Am J Respir Crit Care. 2001 Dec 1;164(11):2085-91
Pathophysiology
Genetic predisposition for exaggerated inflammatory response
Large release of cytokines leads to granuloma formation
Depending upon cytokine release, may be chronic Chronic in about 20% of patients
Human Leukocyte Antigen dependent HLA-DRB1*1101 = Higher incidence HLA-DRB1*03 = better prognosisBaughman R.P., Lower E.E. (2012). Chapter 329. Sarcoidosis. In Longo D.L., Fauci A.S., Kasper D.L., Hauser S.L.,
Jameson J, Loscalzo J (Eds), Harrison's Principles of Internal Medicine, 18e. Retrieved May 06, 2015 from http://0-accesspharmacy.mhmedical.c
Immune Response
APC Helper T cells * Macrophage
cluster Cytokine release
IL-2 IFN gamma IL-10 vs IL-8 + TNF
Diagnosis
Manifestations
Lungs: >90% Skin: 30% Eyes: Vary
>70% in Japan 30% in US (more common in AA
population) Liver: 20-30% Anemia 20%
Chest Manifestations
http://pixgood.com/sarcoidosis-chest-x-ray.html
Chest Manifestations
Skin Manifestations
Acute Disease Management
Patient Case
What medication is our patient not receiving that we might expect him to be on?
Oral Steroids
Prednisone 20-40 mg per day then taper down 5 mg/day biweekly to maintenance dose of 5-10 mg/day
Adverse Effects: Weight gain Hypertension Infection Blood glucose increase (Diabetes) Cushing’s syndrome Glaucoma/Cataracts
Oral Steroids
Cochrane analysis of 3 trials of steroids vs placebo Chest x –ray showed improvement in 70% of
patients with steroids vs. 49% in placebo group
But… No significant difference after 2 years of treatment
Analysis of 2 trials of steroid vs no treatment Significant improvement at 7 months, but
not after 24 monthsParamothayan N, Lasserson T, Jones P. Corticosteroids for pulmonary sarcoidosis. Cochrane Database Syst Rev. 2005; Apr 18;(2)
Chronic Treatment
Methotrexate
10-20 once a week PO or IM MOA: Multiple, but in sarcoidosis, inhibition of
T cell activation and down regulation of B cells Toxicities: hepatic, hematologic, renal,
pulmonary Contraindicated:
Liver/Renal Failure Respiratory Failure Alcohol abuse Pregnant/Lactating women
Pregnancy Category X (cytotoxic)
Methotrexate
SIDE EFFECTS
Hepatic fibrosis Leukopenia Pulmonary fibrosis
Non-productive cough Dyspnea Fever
Nausea Alopecia
EFFICACY
Have been found to reduce steroid dose Not significant because of
drop out Retrospective cohort of
200 patient with MXT or AZA Decrease in prednisone
dose▪ 6 mg/yr
Increase in FEv1 and VC AZA had high rate of
infectionVorselaars A, Wuyts W, Vorselaars V, et al. Methotrexate vs azathioprine in second-line therapy of sarcoidosis. Chest. 2013;144(3):805
Hydroxychloroquine
200-400 mg/day Very limited data, only used when other
treatments have failed More often used for cutaneous sarcoidosis Major Adverse Effects:
Retinopathy/Blindness Hepatic failure Hematologic toxicity Hearing loss Angioedema
Baughman R, Lower E. Evidence-based therapy for cutaneous sarcoidosis. Clin Dermatol: 2007;25(3):334
Infliximab
Chimeric, humanized monoclonal antibody Neutralizes TNFa
Dose: 3-5 mg/kg at weeks 0, 2, 6, and 12, then…?
Study performed of 138 patients with sarcoidosis for over a year
FVC increased 2.8% and 2.2% in 3 mg/kg and 5 mg/kg, respectively.
Small study size, relatively small increase, only 24 week study
Baughman R, Drent M, Kavuru M. Infliximab therapy in patients with chronic sarcoidosis and pulmonary involvement. Am J Respir Crit Care Med. 2006: 1;174(7): 795-802
Patient Review
Recently diagnosed with Type 2 DM Poor diet, inactivity, weight gain, long-
term corticosteroid use History of sarcoidosis (>1 year)
Skin lesions, pulmonary manifestations, visual manifestations, kidney stones
Currently taking hydroxychloroquine 200 mg BID and topical ointment PRN
References
Valeyre D, Prasse A, Nunes H, Uzunhan Y, Brillet PY, Müller-Quernheim J. Sarcoidosis. Lancet. 2014 Mar 29;383(9923):1155-67
Rybicki B, Ianuzzi M, Frederick M, et al. Familial aggregation of sarcoidosis. A case-control etiology study of sarcoidosis (ACCESS) Am J Respir Crit Care. 2001 Dec 1;164(11):2085-91
Baughman R.P., Lower E.E. (2012). Chapter 329. Sarcoidosis. In Longo D.L., Fauci A.S., Kasper D.L., Hauser S.L., Jameson J, Loscalzo J (Eds), Harrison's Principles of Internal Medicine, 18e. Retrieved May 06, 2015 from http://0-accesspharmacy.mhmedical.com
Paramothayan N, Lasserson T, Jones P. Corticosteroids for pulmonary sarcoidosis. Cochrane Database Syst Rev. 2005; Apr 18;(2)
Vorselaars A, Wuyts W, Vorselaars V, et al. Methotrexate vs azathioprine in second-line therapy of sarcoidosis. Chest. 2013;144(3):805
Baughman R, Lower E. Evidence-based therapy for cutaneous sarcoidosis. Clin Dermatol: 2007;25(3):334
Baughman R, Drent M, Kavuru M. Infliximab therapy in patients with chronic sarcoidosis and pulmonary involvement. Am J Respir Crit Care Med. 2006: 1;174(7): 795-802
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