Pneumonitis lupus

Matthay RA, Schwarz MI, Petty TL, et al. Pulmonary manifestations of systemic lupus erythematosus review of twelve cases of acute lupus pneumonitis. Medicine (Baltimore) 1975 54 397 09. 

ILD in SLE may occur as a residual feature of acute lupus pneumonitis (278,288) or may present insidiously, often with mild flares of pulmonary activity (277,293,309). The symptoms are nonspecific with persistent dyspnea on exertion, occasional pleuritic chest pain, and non-productive cough. Physical examination may reveal fever, cyanosis, or bibasilar crackles. Clubbing is less common in SLE-ILD than in IPE (310). The pattern and severity of PFT impairment do not correlate with systemic disease activity, as judged by anti-DNA antibodies and serum complement levels (293,311,312). ELD can occur in the absence of active disease in other organs (282,312). Interestingly, the presence of scleroderma-like traits in SLE is associated with a higher prevalence of restrictive defects or reduced DLco (313). Progressive severe ELD rarely complicates SLE but is seen in some patients with SLE features as part of an overlap syndrome (314). 

Boulware DW, Hedgpeth MT. Lupus pneumonitis and anti-SSA(Ro) antibodies. J Rheumatol 1989 16 479 81. 

SLE is a common CTD with frequent chest manifestations (Table 3). The most common chest manifestation is pleurisy that can occur with or without a pleural effusion. Although ILD has been described in SLE, more common is a presentation of lung parenchymal injury called lupus pneumonitis. SLE may also cause alveolar hemorrhage, a particularly serious form of lung involvement. This review will discuss what is known about these disease states. 

Pleurisy with effusions Exudative pleural effusion Pericarditis with or without effusion Parenchymal lung disease Interstitial lung disease Acute lupus pneumonitis Organizing pneumonia Lymphocytic interstitial pneumonia Diffuse alveolar hemorrhage Airway disease... 

Less acute presentations can occur. A case report of lupus pneumonitis with normal computed tomography scans has been described (28). Migratory infiltrates can also occur. 

The diagnostic evaluation requires samples from the lower respiratory tract. While some individuals have advocated a surgical lung biopsy, most practitioners will perform bronchoscopy with bronchoalveolar lavage (BAL). The BAL is sent for cultures for bacteria, mycobacteria, and fungi. A ceU count in acute lupus pneumonitis is typically neutrophil predominant. Exclusion of pulmonary emboli is usually performed, particularly if the patient has circulating antiphospholipid antibodies. 

The resolution of acute lupus pneumonitis often occurs quickly once immunosuppressives have been initiated. Corticosteroids in high dose are indicated, although cases of relapse on corticosteroid monotherapy have been seen (29). Therefore, the usual patient will be started on adjunctive immunosuppressives dictated by the extent of multiorgan involvement and the confidence that infection has been excluded or adequately treated. Experience with aza-thioprine, methotrexate, cyclophosphamide, cyclosporine, and mycophenalate mofetil has been reported. 

Following episodes of lupus pneumonitis, spirometry is useful to define the resolution and to taper immunosuppressive therapy. Death appears to be rare however, gas exchange abnormalities persist in the majority of patients (30), and a rare patient will have persistent interstitial infiltrates. 

Susanto I, Peters JI. Acute lupus pneumonitis with normal chest radiograph. Chest 1997 111(6) 1781-1783. 

Matthay RA, Hudson LD, Petty TL. Acute lupus pneumonitis response to aza-thioprine therapy. Chest 1973 63(1) 117-120. 

Erickson RW, Frankhn WA, Emlen W. Treatment of hemorrhagic lupus pneumonitis with plasmapheresis. Semin Arthritis Rheum 1994 24(2) 114-123. 

Hypersensitivity Anaphylactoid purpura, anaphylaxis, angioneurotic edema, myocarditis, pericarditis, polyarthralgia, pulmonary infiltrates with eosinophilia, systemic lupus erythematous exacerbation, urticaria hypersensitivity syndrome (cutaneous reaction, eosinophilia, and one or more of the following Hepatitis, pneumonitis, nephritis, myocarditis, pericarditis, fever, lymphadenopathy). Muscuioskeietai - ArVr ra g a, arthritis, bone discoloration, joint stiffness and swelling, myalgia, polyarthralgia. 

T e III reactions are mimicked by nitrofurantoin (pneumonitis) and penicillamine (nephropathy). Lupus erythematosus due to drugs (procainamide, isoniazid, phenytoin) may be pseudoallergic. 

Some drugs such as hydralazine or procainamide may induce lupus like diseases with antinuclear antibodies and proteinuria. D-penicillamine not only causes glomerulopathies, but also myasthenia, polymyositis or lupus, suggesting that this compound provokes immune disregulation. Gold salts also are capable of inducing various immunopathological disorders such as pneumonitis, anemia, thrombocytopenia and hepatitis. 

Systemic lupus erythematosus deficiency Pulmonary Diseases Aspiration pneumonitis Bronchoconstrictive diseases Acute asthma... 

Examples include anemia, leukopenia, thrombocytopenia, pneumonitis, vasculitis, lupus-like reactions, glomerulonephritis. 

Other target organs, such as the skin with lupus, the joints with rheumatoid arthritis, and the lungs with pneumonitis, may be affected. The deposition of antigen-antibody complexes through the circulatory system results in a syndrome referred to as serum sickness, which was... 

The lymphoplasmacytic interstitial infiltrates seen in UIP are generally sparse in comparison to other entities such as nonspecific interstitial pneumonia (NSIP) or hypersensitivity pneumonitis (HP). Extensive cellular infiltrates or follicular lymphoid hyperplasia should lead to consideration of other entities that may cause a UIP pattern of fibrosis such as CVD or HP. Pleural inflammation is not a feature of idiopathic UIP, and its presence indicates a second process or secondary form of UIP with pleuritis, most likely a CVD such as rheumatoid arthritis (RA) or systemic lupus erythematosus (SUE) (14). 

Yood RA, Steigman DM, Gill LR. Lymphocytic interstitial pneumonitis in a patient with systemic lupus erythematosus. Lupus 1995 4 161-163. 

Benisch B, Peison B. The association of lymphocytic interstitial pneumonitis and systemic lupus erythematosus. Mount Sinai J Med 1979 46 398-401. 

Hedgpeth MT, Boulware DW. Interstitial pneumonitis in antinuclear antibodynegative systemic lupus erythematosus a new clinical manifestation and possible association with anti-Ro (SS-A) antibodies. Arthritis Rheum 1988 31 545-548. 

Systemic lupus erythematosus (SLE) is a systemic auto-immune disease of unknown cause in which tissues and cells are damaged by pathogenic autoantibodies and immune complexes. Ninety per cent of cases are in women, usually of childbearing age. Pleurisy and pleural effusions are common manifestations of SLE. Interstitial pneumonitis leading to fibrosis occurs occasionally. Pulmonary hypertension is a serious, uncommon manifestation of SLE. Many autoantibodies could be detected in SLE patients the most frequent are antinuclear antibodies (Keren) (90% of patients) the most specific antibodies are anti-DNA (70% of patients) and anti-Sm (30% of patients). 

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