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A Curious Case of Acute Respiratory Failure: Is It Antisynthetase Syndrome?

Malhotra G, Ramreddy N, Chua S, Iliescu M, Kaur T - Case Rep Crit Care (2016)

Bottom Line: Interstitial lung disease (ILD) is the main pulmonary manifestation and may be severe thereby determining the prognosis.ADM may be associated with rapidly progressive course of interstitial lung disease (ADM-ILD) which is associated with high mortality.Differentiation between ADM-ILD and AS syndrome may be difficult in the absence of positive serology and clinical presentation may help in clinching the diagnosis.

View Article: PubMed Central - PubMed

Affiliation: University of Illinois at Chicago, Chicago, IL 60612, USA.

ABSTRACT
Antisynthetase (AS) syndrome is a major subgroup of inflammatory myopathies seen in a minority of patients with dermatomyositis and polymyositis. Although it is usually associated with elevated creatine phosphokinase level, some patients may have amyopathic dermatomyositis (ADM) like presentation with predominant skin involvement. Interstitial lung disease (ILD) is the main pulmonary manifestation and may be severe thereby determining the prognosis. It may rarely present with a very aggressive course resulting in acute respiratory distress syndrome (ARDS). We report a case of a 43-year-old male who presented with nonresolving pneumonia who was eventually diagnosed to have ADM through a skin biopsy without any muscle weakness. ADM may be associated with rapidly progressive course of interstitial lung disease (ADM-ILD) which is associated with high mortality. Differentiation between ADM-ILD and AS syndrome may be difficult in the absence of positive serology and clinical presentation may help in clinching the diagnosis.

No MeSH data available.


Related in: MedlinePlus

Skin biopsy shows acanthosis and papillomatosis with focal dermal perivascular lymphocytic infiltrate (10x).
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fig3: Skin biopsy shows acanthosis and papillomatosis with focal dermal perivascular lymphocytic infiltrate (10x).

Mentions: He later developed a fever and chest X-ray (Figure 1) was done which revealed bilateral lower and right middle lobe infiltrates. Respiratory panel, blood culture, sputum culture, fungal culture, Legionella antigen, Histoplasma antigen, QuantiFERON Gold, hepatitis panel, and HIV test were all negative. A computed tomography scan of the chest (Figure 2) showed patchy subpleural bilateral opacities more prominent in the bases with subtle reticulonodular opacities in both lungs along with traction bronchiectasis. No significant pleural effusion, pneumothorax, or lymphadenopathy was seen. He was recently treated for pneumonia at another hospital and was thus empirically started on antibiotics for treatment of suspected health care associated pneumonia. Bronchoscopy with transbronchial biopsy revealed chronic inflammation and fibrosis. Autoimmune workup including anti-cyclic citrullinated peptide, anti-nuclear antibody, double-stranded DNA antibody, anti-proteinase 3, anti-myeloperoxidase, Scl-70, anti-smooth muscle, anti-Jo-1 antibody, and cryoglobulin was unremarkable. Aldolase was elevated at 13.7 (normal < 8.1 U/L), C-reactive protein was 10.4 (normal 0–8 mg/L), creatine phosphokinase was 351 (normal 30–223 IU/L), and rheumatoid factor was 71 (normal < 14 IU/mL). Skin biopsy of lesions over metacarpophalangeal joints revealed mild epidermal acanthosis and papillomatosis with perivascular inflammation suggestive of dermatomyositis (Figure 3).


A Curious Case of Acute Respiratory Failure: Is It Antisynthetase Syndrome?

Malhotra G, Ramreddy N, Chua S, Iliescu M, Kaur T - Case Rep Crit Care (2016)

Skin biopsy shows acanthosis and papillomatosis with focal dermal perivascular lymphocytic infiltrate (10x).
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4940527&req=5

fig3: Skin biopsy shows acanthosis and papillomatosis with focal dermal perivascular lymphocytic infiltrate (10x).
Mentions: He later developed a fever and chest X-ray (Figure 1) was done which revealed bilateral lower and right middle lobe infiltrates. Respiratory panel, blood culture, sputum culture, fungal culture, Legionella antigen, Histoplasma antigen, QuantiFERON Gold, hepatitis panel, and HIV test were all negative. A computed tomography scan of the chest (Figure 2) showed patchy subpleural bilateral opacities more prominent in the bases with subtle reticulonodular opacities in both lungs along with traction bronchiectasis. No significant pleural effusion, pneumothorax, or lymphadenopathy was seen. He was recently treated for pneumonia at another hospital and was thus empirically started on antibiotics for treatment of suspected health care associated pneumonia. Bronchoscopy with transbronchial biopsy revealed chronic inflammation and fibrosis. Autoimmune workup including anti-cyclic citrullinated peptide, anti-nuclear antibody, double-stranded DNA antibody, anti-proteinase 3, anti-myeloperoxidase, Scl-70, anti-smooth muscle, anti-Jo-1 antibody, and cryoglobulin was unremarkable. Aldolase was elevated at 13.7 (normal < 8.1 U/L), C-reactive protein was 10.4 (normal 0–8 mg/L), creatine phosphokinase was 351 (normal 30–223 IU/L), and rheumatoid factor was 71 (normal < 14 IU/mL). Skin biopsy of lesions over metacarpophalangeal joints revealed mild epidermal acanthosis and papillomatosis with perivascular inflammation suggestive of dermatomyositis (Figure 3).

Bottom Line: Interstitial lung disease (ILD) is the main pulmonary manifestation and may be severe thereby determining the prognosis.ADM may be associated with rapidly progressive course of interstitial lung disease (ADM-ILD) which is associated with high mortality.Differentiation between ADM-ILD and AS syndrome may be difficult in the absence of positive serology and clinical presentation may help in clinching the diagnosis.

View Article: PubMed Central - PubMed

Affiliation: University of Illinois at Chicago, Chicago, IL 60612, USA.

ABSTRACT
Antisynthetase (AS) syndrome is a major subgroup of inflammatory myopathies seen in a minority of patients with dermatomyositis and polymyositis. Although it is usually associated with elevated creatine phosphokinase level, some patients may have amyopathic dermatomyositis (ADM) like presentation with predominant skin involvement. Interstitial lung disease (ILD) is the main pulmonary manifestation and may be severe thereby determining the prognosis. It may rarely present with a very aggressive course resulting in acute respiratory distress syndrome (ARDS). We report a case of a 43-year-old male who presented with nonresolving pneumonia who was eventually diagnosed to have ADM through a skin biopsy without any muscle weakness. ADM may be associated with rapidly progressive course of interstitial lung disease (ADM-ILD) which is associated with high mortality. Differentiation between ADM-ILD and AS syndrome may be difficult in the absence of positive serology and clinical presentation may help in clinching the diagnosis.

No MeSH data available.


Related in: MedlinePlus