Table.

Acute Fibrinous and Organizing Pneumonia Associated with Rheumatic Diseases.

	Rheumatic diseases	Age Sex	Symptoms	Autoantibodies	Radiographic findings	Therapy	Outcome	References
1	Polymyositis	78 F	Dyspnea	Not described	Bilateral reticulonodular	CS	Death unrelated cause	1
2	Ankylosing spondylitis	55 M	Cough, dyspnea, hemoptysis	Not described	Bilateral perihilar air-space	Antibiotics	Improved
3	Possible fibromyalgia	58 F	Fever, arthralgia	Not described	Consistent with atypical pneumonia	Antibiotics	Improved
4	Dermatomyositis	14 F	Dyspnea	ANA	Extensive parenchymal process with patchy densities prominent in both lower lobes	Antibiotics, CS, cyclosporine, IV CYC, ventilation	Death respiratoly failure	2
5	Undifferentiated connective tissue disease	39 F	Cough, fever, dyspnea Raynaud’s phenomenon, sicca syndrome (normal salivary gland biopsy), boot and gloves neuropathic pain	p-ANCA, anti-TPO, anti-TBG	Diffuse GGA, with foci of parenchymal densification	CS, IV CYC, later IV MP ventilation	Death respiratoly failure, pulmonary hemorrhage	5
6	Collagen vascular disease-like condition	47 M	Joint pain and stiffness, myalgia, dry cough, and dyspnea	ANA	Diffuse reticular abnormalities in both lower lobes, associated with a GGA	CS, AZA, ventilation	Improved	6
7	Primary Sjögren's syndrome	75 F	Dyspnea, wheezing, productive cough dry skin, arthritis, rash and pulmonary hypertension	anti-SS-A anti-SS-B	Bilateral multi-lobar areas of consolidation	CS	Improved	7
8	Primary Sjögren's syndrome	60 F	Fever, dyspnea, dry cough	ANA anti-SS-A	Bilateral diffuse multiple solitary nodules admixed with patchy areas of GGA	CS	Improved	8
9	Antisynthetase syndrome	34 M	Dry cough, dyspnea on exertion necrotizing myopathy	anti-EJ	Bilateral patchy infiltrates predominantly in the lower lobes	CS, IVIG, MMF	Improved	9
10	Antisynthetase syndrome	66 F	Pruritic rash, myalgia, productive cough,	Atypical pANCA, anti-EJ	Pathy peripheral airspase consolidation including foci of central lucency	CS (only initial use of MMF and AZA by intolerance)	Improved	10
11	Systemic lupus erythematosus, anti-phospholipid syndrome	47 M	Dyspnea, productive cough, hemoptysis	ANA, anti-dsDNA, anti-Sm anti-phospholipid	Peripheral and peribronchiolar areas of consolidation, maximal in the superior segments of the lower lobes with subpleural sparing in several areas	CS, oral CYC	Improved	11
12	Systemic lupus erythematosus	25 F	Fever, cough, erythema, nephritis, pancytopenia hemophagocytosis	ANA, anti-dsDNA, anti-Sm anti-U1RNP, anti-SS-A, PAIgG	Diffuse granular shadows	IV MP, CS, PE, IVIG, IV CYC	Improved	Present case

ANA: anti-nuclear antibody, AZA: azathioprine, CS: corticosteroid, CYC: cyclophosphamide, GGA: ground-glass attenuation, IV CYC: intravenous cyclophosphamide, IVIG: intravenous immunoglobulin, IV MP; intravenous methyl-prednisolone pulse, MMF: mycophenolate mofetil, PAIgG: platelet-associated immunoglobulin-G, PE: plasma exchange, TGB: thyroxin binding globulin, TPO: thyroid peroxidase