Shrinking Lung Syndrome: A Rare Manifestation of Systemic Lupus Erythematosus

Abstract

Shrinking lung syndrome (SLS) is a pulmonary complication of systemic lupus erythematosus (SLE) characterized by dyspnea, pleuritic chest pain, and progressive decrease in lung volumes with no evidence of pleural or interstitial disease on chest CT. We present a 51-year-old female with a 14-year history of SLE with symptoms of progressive shortness of breath, pleuritic chest pains, low grade fevers, and productive cough which was unresponsive to multiple courses of antibiotics. After careful review of her course of SLE and timeline of symptoms, she was diagnosed with SLS. Even though rare, clinicians should have a high suspicion of SLS in patients with a long-term history of SLE and worsening dyspnea. Early treatment can be initiated to help reduce long-term morbidity and mortality and maintain the quality of life.

‘But you don’t look sick’: a qualitative analysis of the LUPUS UK online forum

“…Reports of dismissal and disbelief by clinicians unable to discover a cause for a new symptom were frequent, and their offers of reassurance rarely inspired confidence. Even those with long-standing disease reported misdiagnoses or no explanation-or treatment-for new, rarer [39][40][41] manifestations, particularly from those physicians who were felt to focus only on the more common symptoms affecting joints, skin and kidneys. Early signs of rarer manifestations were often under-investigated and undetected, until they progressed to become obvious on current (often perceived as inadequate) testing, sometimes coinciding with irreversible organ damage, including sight or hearing loss, intestinal failure and brain damage.…”

Respiratory Manifestations in Systemic Lupus Erythematosus

“…Shrinking lung syndrome (SLS) is a rare manifestation of SLE affecting less than 1% of SLE patients [ 131 ], with about 100 cases described to date [ 132 ]. Older papers reported a higher prevalence of 18–27%, while a prevalence of up to 7% has been described among patients with refractory SLE [ 132 , 133 , 134 ].…”

“…Shrinking lung syndrome (SLS) is a rare manifestation of SLE affecting less than 1% of SLE patients [ 131 ], with about 100 cases described to date [ 132 ]. Older papers reported a higher prevalence of 18–27%, while a prevalence of up to 7% has been described among patients with refractory SLE [ 132 , 133 , 134 ]. It was described for the first time in 1965 by Hoffbrand and Beck [ 135 ], and subsequently it has occasionally been described in other autoimmune diseases (e.g., systemic sclerosis, primary Sjögren’s syndrome, RA and undifferentiated arthritis) [ 136 , 137 ].…”

“…No specific association was found between serologic markers and the disease, it was suggested an association with Anti-Ro/SSA. Since there are no specific diagnostic criteria, the diagnosis is one of exclusion [ 131 , 132 , 138 , 139 , 140 , 141 ]. The pathogenesis of this condition is not known, and several mechanisms have been proposed in recent years: micro-atelectasis with surfactant deficiency, phrenic nerve neuropathy, primary respiratory muscle myopathy, diaphragmatic fibrosis, steroid induced myopathy, pleural adhesions, and pleuritic chest pain with reduced chest expansion by an inhibitory reflex [ 135 , 140 , 142 , 143 , 144 , 145 ].…”

“…The majority of patients received high dose of CS, even with iv pulses, with improvement occurring in several weeks, but in some cases even in 48 h [ 140 , 141 ]; anecdotal data support the use of immunosuppressive agents such CYC, azathioprine, methotrexate, MMF after CS failure or as CS-sparing agents [ 132 , 139 , 141 ]. RTX has been shown to improve lung function and pain in some cases [ 146 ].…”