Lung Manifestations in the Rheumatic Diseases

Abstract

Lung ailments in rheumatic diseases present unique challenges for diagnosis and management and are a source of significant morbidity and mortality for patients. Unlike the idiopathic interstitial pneumonias, patients with rheumatic diseases experience lung disease in the context of a systemic disease that may make it more difficult to recognize and that may present greater risks with treatment. Despite recent advances in our awareness of these diseases, there is still a significant lack of understanding of natural history to elucidate which patients will have disease that is progressive and thus warrants treatment. What we do know is that a subset of patients with rheumatic disease experience parenchymal lung disease that can prognostically resemble idiopathic pulmonary fibrosis, such as in rheumatoid arthritis, and that others can have aggressive inflammatory lung disease in the context of autoimmune myositis, systemic sclerosis, or an undifferentiated autoimmune process. As we enter into a paradigm shift where we view lung health as a cornerstone of our care of patients with rheumatic diseases, we hopefully will improve our ability to identify those patients at highest risk for pulmonary disease and progression, and offer emerging treatments which will result in better outcomes and a better quality of life.

Keywords: interstitial lung disease; myositis; rheumatoid arthritis; scleroderma.

Figure 1

High-resolution CT images. A, Note the rind of ground-glass opacity surrounded by a denser consolidation, known as the atoll sign, in a patient with dermatomyositis and antisynthetae syndrome (Jo-1 antibody positive) and biopsy proven organizing pneumonia. B, Reverse halo sign, which is characterized by ground-glass attenuation surrounded by a ring of denser consolidation. C, Ruptured lung nodule with pneumothorax in a patient with rheumatoid arthritis (RA) and additional rheumatoid nodule in the left lung. D, Mucosa-associated lymphoid tissue (MALT) lymphoma in a 50-year-old woman with Sjögren syndrome and multiple lung nodules. E, Ruptured nodule on right with pneumothorax in a patient with RA and multiple RA nodules.

Figure 2

Clinical characteristics seen in systemic sclerosis/scleroderma. A, Sclerodactyly; note the lack of vertical skin creases on the extensor surfaces of the fingers from the proximal interphalangeal joint to the distal interphalangeal joint. B, Active Raynaud syndrome with sclerodactyly. C, Sharp demarcation in perfusion of the distal digits in a patient with active Raynaud syndrome. D, Ischemic ulceration in a patient with severe Raynaud syndrome. E, Palmar telangiectasia. F, Facial telangiectasia. G, Telangiectasia on the lip and tongue. H, Limited oral aperture. I, Dilated nail fold capillaries, which indicate a vasculopathy seen in scleroderma and other rheumatic syndromes.

Figure 3

Clinical characteristics seen in the autoimmune myositis. A, Diffuse erythema of the face in a patient with dermatomyositis and interstitial lung disease (ILD). B, Periungual erythema (down arrows) of fingers with ischemic vascular changes (up arrow) in the periungual area in a patient with dermatomyositis. C, Eyelid erythema and scaling seen in dermatomyositis. D, Gottren papules over the extensor surfaces of the fingers with periungual erythema in dermatomyositis. E, Cracking in the distal tips of the fingers of a patient with antisynthetase syndrome: “mechanics hands.” F, Mechanics hands in the antisynthetase syndrome. G, Nodular erythematous lesions on the palmar surface of the hand seen in melanoma differentiation-associated gene 5 (MDA5) antibody-related ILD. H, Healing ulcerating plaques on the dorsal surface of the hand in a patient with MDA-5 antibody-related ILD. I, Pneumomediastinum in dermatomyositis.

Figure 4

Additional clinical findings suggestive of a rheumatic syndrome. A, Nodulosis of the extensor elbow region in rheumatoid arthritis. B, Dilated esophagus with air-fluid level in a 68-year-old man with systemic sclerosis. C, Right parotid swelling in a 58-year-old woman with Sjögren syndrome.