mycophenolic-acid and Alveolitis--Extrinsic-Allergic

The optimal pharmacological management of chronic hypersensitivity pneumonitis (cHP) is unknown. Corticosteroids are often used as first line therapy but can be associated with side effects. There is a paucity of data examining the role of steroid-sparing agents in cHP. We aimed to determine the effect of mycophenolate mofetil (MMF) and azathioprine (AZA) on lung function and prednisolone dose in cHP patients.. Retrospective analysis of patients initiated on either MMF or AZA following a multidisciplinary team diagnosis of cHP. Changes in lung function and prednisolone dose up to 12 months before and after MMF/AZA initiation were analysed.. Twenty two out of 30 patients remained on treatment at 12 months (18 MMF, 4 AZA). Steroid-sparing therapy resulted in a significant reduction in prednisolone dose from 16.2 ± 9.7 to 8.2 ± 4.2 mg daily (P = 0.002). Treatment with MMF or AZA for 12 months was associated with a significant improvement in carbon monoxide diffusing capacity (TLCO) (-0.55 ± 0.96 vs. +0.31 ± 0.58 mmol/kPa/min, P = 0.02). Although treatment reduced the rate of forced vital capacity decline (-111 ± 295 vs. +2.3 ± 319 mL), it was not significant (P = 0.22).. MMF or AZA therapy in cHP is associated with an improvement in TLCO and reduction in prednisolone dose. There is a need for prospective trials.

Topics: Adrenal Cortex Hormones; Aged; Alveolitis, Extrinsic Allergic; Azathioprine; Carbon Monoxide; Chronic Disease; Drug Therapy, Combination; Enzyme Inhibitors; Female; Humans; Immunosuppressive Agents; Male; Middle Aged; Mycophenolic Acid; Prednisolone; Pulmonary Diffusing Capacity; Respiratory Function Tests; Retrospective Studies; Vital Capacity

The treatment of chronic hypersensitivity pneumonitis (cHP) often includes systemic oral corticosteroids, but the optimal pharmacologic management remains unclear. The morbidity associated with prednisone has motivated the search for alternative therapies. We aimed to determine the effect of treatment with mycophenolate mofetil (MMF) or azathioprine (AZA) on lung function in patients with cHP.. Patients with cHP treated with either MMF or AZA were retrospectively identified from four interstitial lung disease centers. Change in lung function before and after treatment initiation was analyzed using linear mixed-effects modeling (LMM), adjusting for age, sex, smoking history, and prednisone use.. Seventy patients were included: 51 were treated with MMF and 19 with AZA. Median follow-up after treatment initiation was 11 months. Prior to treatment initiation, FVC and diffusion capacity of the lung for carbon monoxide (Dlco) % predicted were declining at a mean rate of 0.12% (P < .001) and 0.10% (P < .001) per month, respectively. Treatment with either MMF or AZA was not associated with improved FVC (0.5% at 1 year; P = .46) but was associated with a statistically significant improvement in Dlco of 4.2% (P < .001) after 1 year of treatment. Results were similar in the subgroup of patients treated with MMF for 1 year; the FVC increased nonsignificantly by 1.3% (P = .103) and Dlco increased by 3.9% (P < .001).. Treatment with MMF or AZA is associated with improvements in Dlco in patients with cHP. Prospective randomized trials are needed to validate their effectiveness for cHP.

Topics: Aged; Alveolitis, Extrinsic Allergic; Azathioprine; Carbon Monoxide; Chronic Disease; Female; Glucocorticoids; Humans; Immunosuppressive Agents; Linear Models; Male; Middle Aged; Mycophenolic Acid; Prednisone; Pulmonary Diffusing Capacity; Retrospective Studies; Treatment Outcome; Vital Capacity

Posttransplant lymphoproliferative disorders (PTLD) are a potentially life-threatening complication of immunosuppression in transplant recipients. The majority of cases are Epstein-Barr virus-associated lesions of B cell origin. T cell PTLD is rare, particularly in pediatric patients. We present an unusual case of monomorphic T cell PTLD with features of angioimmunoblastic T cell lymphoma in an 8-year-old heart transplant patient, presenting with cranial nerve palsy.

Topics: Alveolitis, Extrinsic Allergic; Antineoplastic Combined Chemotherapy Protocols; Bone Marrow; Child; Cyclophosphamide; Cytarabine; Etoposide; Heart Transplantation; Humans; Hydrocortisone; Immunoblastic Lymphadenopathy; Immunocompromised Host; Immunophenotyping; Immunosuppressive Agents; Kartagener Syndrome; Lymphoma, T-Cell; Methotrexate; Mycophenolic Acid; Oculomotor Nerve Diseases; Postoperative Complications; Prednisone; Remission Induction; T-Lymphocyte Subsets; Tacrolimus; Vincristine