A 29 year-old healthy woman attended our outpatient clinic, and was subsequently hospitalized, with polyartritis, myositis, Raynaud phenomenon and dyspnea, that she was experiencing since December 2015. A Mixed Connective Tissue Disease was finally diagnosed according to the clinical presentation and a positive result of antinuclear antibodies (titer 1/2560) with anti-RNP and anti-Ro specificities. During the hospitalization her dyspnea worsened and a severe pulmonary arterial hypertension secondary to MCTD was diagnosed (mPAP 50 mmHg, dilatation of right cavities with flattening of interventricular septum, NT-proBNP >6000 ng/L; other causes of pulmonary arterial hypertension have been ruled out) and dual therapy with epoprostenol and bosentan was initiated, but the response is scarce and the patient is deteriorating. I am wondering about the role of immunosupresion in this patient considering her bad evolution and prognosis.
Thank you very much in advance!
Dear Dr Ivan,
any autoimmune entity in my opinion requires a immunosuppression. so also in your case regardless of patient having PAH or not. having said that PAH does not deteriorate so rapidly in MCTD. Though you have ruled out other causes of PAH, i would like to know whether APLA work up has been done too, as well as whether dermatomyositis (synthetase syndrome) has been ruled out. i would keep searching for an additional cause of PAH.
Regards
Dear Iván, I believe you should treat the patient with immunosupresion and mantain classic therapy against pulmonar hypertension with phosphodiesterase enzyme inhibitor or endothelin receptor antagonist or prostaglandins.
A recente revision published by Eric L Greidinger (http://emedicine.medscape.com/article/335815-overview) consider that:
Major organ involvement may require moderate-to-high divided daily doses of corticosteroids and cytotoxic agents (eg, PO or pulse IV cyclophosphamide). Recent reports suggest that, in contrast to primary or scleroderma-associated pulmonary hypertension, a subset of MCTD patients with pulmonary hypertension may respond well to aggressive immunosuppression with cytotoxic agents.
The timely diagnosis of PAH hopefully lead to its early treatment with the administration of therapies which have shown some promise in this disorder. These therapies include a calcium channel blocker (usually long-acting nifedipine ), anticoagulation, intravenous prostacyclin, prolonged immunosuppression (beginning with glucocorticoids and combining with cyclophosphamide if necessary), and angiotensin converting enzyme inhibitors.
The PAH of this patient is secandary of MCTD, so treat MCTD will be the first choice.
I think this young lady should be treated with immunosuppresants since the basis for her current problem is an ongoing immmune-mediated process.