Sarcoidosis Overview. Kenneth S. Knox, MD Assistant professor of medicine Division of Pulmonary & Critical Care Medicine Indiana University. What is Sarcoidosis ?. Nobody knows Systemic inflammatory/immunologic disorder
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Kenneth S. Knox, MD
Assistant professor of medicine
Division of Pulmonary & Critical Care Medicine
** ACCESS trial– ACase Controlled Etiologic Study of Sarcoidosis
1) Rybicki BA, Iannuzzi MC, Frederick MM, Familial aggregation of sarcoidosis. A case-control etiologic study of sarcoidosis (ACCESS). Am J Respir Crit Care Med. 2001 Dec 1;164(11):2085-91.
2) Baughman RP, Teirstein AS, Judson MA, Clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Crit Care Med. 2001 Nov 15;164(10 Pt 1):1885-9.
3) Freemer M, King TE Jr. The ACCESS study: characterization of sarcoidosis in the United States. Am J Respir Crit Care Med. 2001 Nov 15;164(10 Pt 1):1754-5.
4) Rossman M, Thompson B, Frederick M,. Sarcoidosis: association with human leukocyte antigen class II amino acid epitopes and interaction with environmental exposures. Chest. 2002 Mar;121(3)supl.
5) Pandey JP, Frederick M; ACCESS Research Group. A Case Control Etiologic Study of Sarcoidosis. TNF-alpha, IL1-beta, and immunoglobulin (GM and KM) gene polymorphisms in sarcoidosis. Hum Immunol. 2002 Jun;63(6):485-91.
6) Judson MA, Baughman RP, Thompson BW, Two year prognosis of sarcoidosis: the ACCESS experience. Sarcoidosis Vasc Diffuse Lung Dis. 2003 Oct;20(3):204-11.
7) Rossman MD, Thompson B, Frederick M, HLA-DRB1*1101: a significant risk factor for sarcoidosis in blacks and whites. Am J Hum Genet. 2003 Oct;73(4):720-35.
Second most common lung disease in young adults (second only to asthma)
“Clustering”many reports, (ie: navy, Isle of Man)
School teachers- my experience
Geographical(“farther from the equator”)
Seasonal (“cool summer, mild winter”)“springtime dz”
cases reported worldwide.
Likely not associated
Wood dust and pine pollen
Review: Current Opinion in Pulmonary Medicine 2002,8, 413-476
ATS/ERS/WASOG statement on sarcoidosis: Sarc Vasc Diff Lung Dis 1999,16
Notable, recent infectious possibilities
You react to “privileged self-antigens”
** Many exposures, infections, and “injury”
can be associated with “granulomatous inflammation”
Associated with therapy “Immune-modulating”
** Heterogeneous disease!
25% of patients
20% of patients
5% symptomatic, 30% incidental
< 5% have bone involvement; less than 1% have chronic muscle involvement
CNS: headache, memory loss, palsy, weakness, dizziness, visual, stroke
5% symptomatic, 15% overall
IU neurosarcoid data
Lung:cough, short of breath, chest pain
Staging disease by Chest X-ray:
Stage 0 Normal (5%,ACCESS 8%)
Stage 1 Large chest lymph nodes only (50%,40%)
Stage 2 Chest nodes and lung infiltrate (25%,37%)
Stage 3 Lung infiltrates only (15%,10%)
Stage 4 Fibrosis (5%,5%)
IU clinical BAL Lab
Tissue- granuloma shuffle
(Classically, a “Non-necrotizing granuloma without necrosis”)
DDx: TB, Endemic fungal infection,
Malignancy, GLUS, Wegner’s, HP
foreign body reaction
** Many times supportive evidence and clinical context
Endobronchial biopsy for sarcoidosis: a prospective study.Shorr AF, Torrington KG, Hnatiuk OW. Chest. 2001 Jul;120(1):109-14.
Six TBB specimens were obtained, as were six EBB samples. For patients with abnormal-appearing airways, four specimens were obtained from the abnormal-appearing airways and two specimens were obtained from the main carina. In patients with normal-appearing airways, four specimens were obtained from a secondary carina and two specimens were obtained from the main carina. EBB findings were positive in 61.8% of patients, while TBB showed nonnecrotizing granulomas in 58.8% of subjects. The addition of EBB increased the yield of FOB by 20.6%. Although EBB findings were more frequently positive in abnormal-appearing airways (p = 0.014), EBB provided diagnostic tissue in 30.0% of patients with normal-appearing endobronchial mucosa.
CONCLUSION: EBB has a yield comparable to TBB and can safely increase the diagnostic value of FOB. Pulmonologists should consider routinely performing EBB in cases of suspected sarcoidosis.
Diagnostic value of transbronchial needle aspiration by Wang 22-gauge cytology needle in intrathoracic lymphadenopathy.Cetinkaya E, Yildiz P, Altin S, Yilmaz V. Chest. 2004 Feb;125(2):527-31.
Diagnostic material was obtained from 16 of 21 patients with sarcoidosis (76%). In sarcoidosis, TBNA provided the only diagnostic specimen in 13 of 21 patients (62%).
CONCLUSION: TBNA performed with a Wang 22-gauge cytology needle is an effective and safe way of obtaining cytologic specimens from intrathoracic lymph nodes and can rapidly provide diagnosis, both in malignant and benign mediastinal diseases. Hopefully, this technique will reduce further need for more invasive surgical procedures.
CONSIDER NOT TREATING!
Early Treatment of Stage II Sarcoidosis Improves 5-Year Pulmonary Function*
Anne Pietinalho, MD, PhD; et al. the Finnish Pulmonary Sarcoidosis Study Group†
Study objective:To evaluate the 5-year prognosis of patients with stage I and stage II newly
detected (< 3 months) pulmonary sarcoidosis treated immediately after diagnosis with prednisolonefor 3 months followed by inhaled budesonide for 15 months. 79 patients with initial stage I disease and 70 patients with stage II disease.
Treatment:Oral prednisolone for 3 months followed by inhaled budesonide for 15 months (800mg bid), or placebo tablets followed by placebo inhaler therapy. Thereafter, treatment only on anindividual basis in the case of clinical deterioration.
Results: Placebo-treated patients more frequently required treatment with corticosteroids during the 5-year follow-up (p < 0.05). Steroid-treated patients with initial stage II(-III) disease improved more in FVC and DLCO (p < 0.05). No differences in reported adverse events or in SACE, serum calcium, or urinary calcium values were seen.
Conclusion: Immediate treatment of pulmonary stage II(-III) sarcoidosis but not stage I disease improved the 5-year prognosis with regard to lung function variables. (CHEST 2002; 121:24–31)
Topical steroids as primary therapy
Steroids are the mainstay of treatment
If prednisone failure, intolerance, or need another controller agent
Azathioprine(Imuran- 200 max per day)
Eur Respir J. 1999 Nov;14(5):1117-22.
If prednisone failure, intolerance, or need another controller agent
Leflunomide (+/- MTX)
If prednisone failure, intolerance, or need another agent
Baughman, et al. Seminars in Respiratory Infections;1998
Jones and Callen, J Am Acad Derm; 1990
Unclear role, adjunctive, third line
Web-based medicine- risky!
• Carcinosin • Euphrasia • Graphites • Leuticum (Syphilinum) • Bacillinum • Sepia • Phosphorus • Arsenicum album
Quality of life- Psychosocial