Search

Patients with connective tissue diseases may have pulmonary involvement, including interstitial lung disease. Various patterns of interstitial lung disease have been classically described in certain connective tissue diseases. It is now recognized that there is significant overlap between patterns of interstitial lung disease observed in the various connective tissue diseases. Differentiating idiopathic from connective tissue disease-related interstitial lung disease is challenging but of clinical importance. New concepts in the diagnosis of connective tissue disease related interstitial lung disease may prove useful in making the diagnosis., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Raynaud's phenomenon affects most patients who have mixed connective tissue disease (MCTD) and frequently represents the initial manifestation of the disease. It is the cutaneous symptom of a systemic vasculopathy that is characterized by intimal fibrosis and blood vessel obliteration that frequently leads to visceral involvement, particularly pulmonary hypertension. An association between Raynaud's phenomenon and the characteristic autoantibody in MCTD, anti-U1-RNP (ribonucleoprotein), is found across the spectrum of rheumatic diseases, including undifferentiated connective tissue disease, scleroderma, and systemic lupus erythematosus. Capillary nailfold examination represents a valuable tool to identify patients who are at risk for MCTD. The goal in the therapy of Raynaud's phenomenon in MCTD is to decrease the frequency of attacks, to prevent digital ulceration, and to limit progressive vascular damage. Therapeutic regimens include the traditional use of calcium channel blockers and novel vascular therapies.

We report a 74-year-old man suffering from mixed connective tissue disease (MCTD) accompanied by a lymphoid follicle in muscle pathology. He showed proximal muscle weakness and arthritis in finger joints. His laboratory findings were characteristic of MCTD. Prednisolone administration favorably improved his symptoms. A lymphoid follicle is a rare pathological finding in inflammatory myopathies. The immunohistochemical findings suggest that the lymphoid follicle would be involved in the specific role for the pathogenesis of MCTD.

A 60-year-old woman with a diagnosis of poorly differentiated lymphocytic lymphoma presented with nephrotic syndrome. Her clinical picture and serological studies were consistent with the diagnosis of mixed connective tissue disease (MCTD). Renal biopsy demonstrated the changes of membranous nephropathy by light, immunofluorescence and electron microscopy. Treatment with corticosteroids and cyclophosphamide had no effect on the proteinuria, and she demonstrated progression of renal failure. This patient demonstrates that the lymphadenopathy associated with MCTD may be of a separate malignant origin. The treatment of MCTD may not alter the course of renal disease, and renal failure may ensue in patients with MCTD.

A 23-year-old black woman with mixed connective tissue disease developed acute onset of shortness of breath and evidence of pulmonary edema. Cardiac isoenzymes, electrocardiograms and radionuclide myocardial scintigraphy were consistent with focal myocardial necrosis. The patient has had no further myocardial complication since initiation of therapy with steroids.