REFRACTORY SARCOIDOSIS
DOI:
https://doi.org/10.55519/JAMC-03-10915Keywords:
Sarcoidosis, Non-caseating Granuloma, Rituximab, Infliximab, Mycophenolate, Methotrexate, AzathioprineAbstract
A multi-organ granulomatous disease with characteristic lung manifestations, sarcoidosis generally responds well to glucocorticoid therapy but 10% of cases are refractory necessitating immunosuppressive therapy. A 58-year-old lady presented with dry cough and progressively worsening shortness of breath for last 12 months. On investigation, her ESR was raised but cultures, malignancy screen and TB quantiferon were negative. HRCT chest demonstrated multiple pulmonary nodules with hilar lymphadenopathy and CT guided biopsy revealed non-caseating granuloma. She was diagnosed with Pulmonary Sarcoidosis and started on oral steroids with minimal improvement. Azathioprine was added but due to gastric intolerance switched to methotrexate. Her disease however continued to worsen and infliximab was started but she developed severe allergic reaction. She was then started on mycophenolate mofetil but her chest imaging continued to worsen. After failing prednisone, azathioprine, methotrexate, infliximab and mycophenolate mofetil, the patient was started on rituximab.References
Sakthivel P, Bruder D. Mechanism of granuloma formation in sarcoidosis. Curr Opin Hematol 2017;24(1):59–65.
Arkema EV, Cozier YC. Epidemiology of sarcoidosis: current findings and future directions. Ther Adv Chronic Dis 2018;9(11):227–40.
Jamilloux Y, Cohen-Aubart F, Chapelon-Abric C, Maucort-Boulch D, Marquet A, Pérard L, et al. Groupe Sarcoïdose Francophone. Efficacy and safety of tumor necrosis factor antagonists in refractory sarcoidosis: A multicenter study of 132 patients. Semin Arthritis Rheum 2017;47(2):288–94.
Korsten P, Strohmayer K, Baughman RP, Sweiss NJ. Refractory pulmonary sarcoidosis - proposal of a definition and recommendations for the diagnostic and therapeutic approach. Clin Pulm Med 2016;23(2):67–75.
Sweiss NJ, Lower EE, Mirsaeidi M, Dudek S, Garcia JG, Perkins D, et al. Rituximab in the treatment of refractory pulmonary sarcoidosis. Eur Respir J 2014;43(5):1525–8.
El Jammal T, Jamilloux Y, Gerfaud-Valentin M, Valeyre D, Sève P. Refractory Sarcoidosis: A Review. Ther Clin Risk Manag 2020;16:323–45.
Spagnolo P, Rossi G, Trisolini R, Sverzellati N, Baughman RP, Wells AU. Pulmonary sarcoidosis. Lancet Respir Med 2018;6(5):389–402.
Baughman RP, Culver DA, Judson MA. A concise review of pulmonary sarcoidosis. Am J Respir Crit Care Med 2011;183(5):573–81.
Baughman RP, Nunes H, Sweiss NJ, Lower EE. Established and experimental medical therapy of pulmonary sarcoidosis. Eur Respir J 2013;41(6):1424–38.
Chapelon-Abric C, Sene D, Saadoun D, Cluzel P, Vignaux O, Costedoat-Chalumeau N, et al. Cardiac sarcoidosis: Diagnosis, therapeutic management and prognostic factors. Arch Cardiovasc Dis 2017;110(8-9):456–65.
Pariser RJ, Paul J, Hirano S, Torosky C, Smith M. A double-blind, randomized, placebo-controlled trial of adalimumab in the treatment of cutaneous sarcoidosis. J Am Acad Dermatol 2013;68(5):765–73.
Maneiro JR, Salgado E, Gomez-Reino JJ, Carmona L. Efficacy and safety of TNF antagonists in sarcoidosis: data from the Spanish registry of biologics BIOBADASER and a systematic review. Semin Arthritis Rheum 2012;42(1):89–103.
Cinetto F, Compagno N, Scarpa R, Malipiero G, Agostini C. Rituximab in refractory sarcoidosis: a single centre experience. Clin Mol Allergy 2015;13(1):19.
Zella S, Kneiphof J, Haghikia A, Gold R, Woitalla D, Thöne J. Successful therapy with rituximab in three patients with probable neurosarcoidosis. Ther Adv Neurol Disord 2018;11:1756286418805732.
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