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TitleAutoimmune Diseases: Acute and Complex Situations
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Table of Contents
                            Autoimmune Diseases
Copyright Page
1: Assessment and Management of the Rheumatological Patient in the Critical Care Unit
2: Life-Threatening Complications of Systemic Lupus Erythematosus
3: Catastrophic Antiphospholipid Syndrome
4: Complex Situations in Rheumatoid Arthritis
5: Sjögren’s Syndrome: Beyond Sicca Involvement
6: Systemic Sclerosis: Severe Involvement of Internal Organs
7: Vasculitic Emergencies in Patients with Large-Vessel Vasculitis
8: Life-Threatening Presentations of ANCA-Associated Vasculitis
9: Severe Polyarteritis Nodosa
10: Life-Threatening Cryoglobulinemia
11: Behçet’s Syndrome: Clinical Presentations Affecting Prognosis and Survival
12: Myositis: When Weakness Can Kill
13: Complicated Sarcoidosis: Challenges in Dealing with Severe Manifestations
14: Complex Situations in Patients with Adult-Onset Still’s Disease
15: Managing Acute and Complex Dermatological Situations
16: Life-Threatening Autoimmune Hematological Disorders
17: Autoimmune Ear, Nose, and Throat Emergencies
18: Ophthalmological Emergencies in Rheumatic and Autoimmune Diseases
19: Emergencies for the Vascular Surgeon
20: Complicated Pregnancies in Patients with Autoimmune Systemic Diseases
21: The Complex Management of Viral-Related Autoimmune Diseases
22: The Role of Intravenous Immunoglobulins in the Management of Acute Complex Autoimmune Conditions
23: Life-Threatening Complications of Biological Therapies
Document Text Contents
Page 2

Autoimmune Diseases

Page 208

202 R.P. Baughman and H. Nunes

Respiratory Failure

While pulmonary disease is the most common manifestation of sarcoidosis, respiratory
failure is relatively unusual. However, pulmonary disease is the most common cause of
death from sarcoidosis.2 Table 13.1 lists the possible reasons for increasing respiratory
distress in a sarcoidosis patient. The multiple causes of dyspnea highlight the multiorgan
nature of sarcoidosis.

Parenchymal Lung Disease

In the assessment of parenchymal lung disease as the cause of respiratory disease, the cli-
nician has two major tools: chest imaging and pulmonary function testing. While both
provide information in identifying advanced lung disease, neither is the “gold standard” in
defining severe respiratory disease.

Thoracic involvement is usually classified using the Scadding staging system based on
chest roentgenogram.3 Patients with fibrosis of the lung are classified as stage 4. This pattern
is usually associated with worsening lung function and more severe symptoms.4 While not all
patients with stage four chest roentgenograms have severe respiratory disease, most patients
who die from pulmonary sarcoidosis have a stage 4 pattern on their chest roentgenogram.2

One of the difficulties of using the Scadding system is the only modest agreement
between readers in staging the chest roentgenogram.5 Another difficulty is that a patient
may have only limited areas of fibrosis and no symptoms. There is also the problem with
mixed disease. This is often more apparent on CT scan. Patients who have areas of honey-
combing and traction bronchiectasis representing fibrosis may have other areas of nodular
infiltrates which may still be reversible with treatment (Fig. 13.1).6

Pulmonary function testing includes spirometry and lung diffusion of carbon monoxide
(DLCO). Spirometry provides both the forced expiratory volume in one second (FEV-1)
and the forced vital capacity (FVC). The FVC is a reasonable measure of lung volume, and

Table 13.1 Causes of respiratory distress in a sarcoidosis patient
• Directly from sarcoidosis organ involvement
– Parenchymal airway disease
– Pulmonary hypertension
– Upper airway
– Compression of airway/vasculature by lymph nodes
– Cardiac
– Skeletal/diaphragmatic muscle weakness
• Consequence of treatment
– Infections
• Other causes
– Pulmonary embolism

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20313 Complicated Sarcoidosis: Challenges in Dealing with Severe Manifestations

a reduced FVC implies restrictive disease. The FEV-1/FVC ratio detects airway obstruc-
tion. While sarcoidosis is often considered a restrictive disease, a significant proportion of
patients have airway obstruction with a reduced FEV-1/FVC.4,7

There are several causes of restrictive disease and reduced FVC. Pulmonary fibrosis is
associated with a reduced FVC. One can also see reduced FVC due to phrenic nerve dis-
ease8 and/or skeletal muscle weakness.9 In one study, muscle weakness was a better predic-
tor of the level of dyspnea than other physiologic measures of lung function.9 Obesity will
also lead to reduced FVC. The DLCO should be reduced to a greater degree than the FVC
in patients with pulmonary fibrosis, while the ratio is normal to increased in patients with
obesity or muscle weakness. Marked reduction of the DLCO compared to the FVC sug-
gests pulmonary hypertension.10,11 This reduction of DLCO out of proportion to FVC has
also been used to detect pulmonary hypertension in scleroderma patients.12

In one prospective study, patients with a FVC of less than 1.5 l (about 30% of predicted
value) had increased mortality over those with higher FVC values.2 However, once patients
are identified with advanced lung disease and referred for lung transplant, the FVC was not
predictive of mortality.13

All patients with significant respiratory symptoms and evidence of parenchymal lung
disease should be considered for systemic therapy.14 A stepwise approach to systemic ther-
apy has been proposed.15 Figure 13.2 shows this approach as applied to a patient with signifi-
cant respiratory disease. Table 13.2 summarizes the initial and maintenance doses of several
of the drugs used to manage complex sarcoidosis. The standard treatment is corticosteroids.
In the United States, this is usually prednisone. Various starting doses of prednisone have
been used. In one study, it was found that prednisone at 20 mg daily was sufficient to treat
pulmonary disease and higher doses were not associated with any greater response.16

Fig. 13.1 Example of CT with
fibrosis and reversible

Page 415

Index 415

management of, 85
screening and prevention, 85

gastrointestinal tract
anorectal disease, 82
colonoscopy/sigmoidoscopy, 83
constipation/volvulus, 82
cyclical antibiotics, 83
diarrhea, 82
endoscopy and barium swallow, 82
gastroesophageal reflux and stricture,

GAVE, 81
malabsorption, 82
morbidity and mortality, 83
octreotide therapy, 84
pancreatic insufficiency, 82
proton pump inhibitors, 83
pseudo-obstruction, 81–82
screening and prevention, 84

immunosuppressive treatments, 68
key features of, 68

pulmonary fibrosis (see Pulmonary

pulmonary hypertension (see Pulmonary

management of, 68–69
severe skin disease, 85–86
SRC (see Scleroderma renal crisis)

Takayasu arteritis (TAK)

bilateral subclavian stenosis, 91
cerebral reperfusion syndrome., 92
CHF, 92
diagnosis, 323
diagnosis of, 2
hypertension, 91–92
mesenteric artery, 92
renal artery stenosis, 90
signs and symptoms, 322–323
TIA, 91
treatment, 323–324
vascular flow restoration, 93
vascular stenosis, 90

Thromboangitis obliterans, 319–320
Thrombocytopenia, 10–11, 58
Thrombocytopenic purpura (TTP), 22
Thrombolysis, 317
Thrombotic thrombocytopenic purpera (TTP),

Toxic epidermal necrolysis (TEN), 238–239
Trans-Atlantic Inter-Society Consensus

(TASC), 316

Transient ischemic attack (TIA), 91

incidence, 385
prevention and management, 388
risk of, 387
TNF-a inhibitors, 385–388

Tumor necrosis factor (TNF), 206

Upper airway disease

endobronchial stenosis, 209
endoscopy, 209–210
lupus pernio, 208–209
stridor, 209
subglottic stenosis, 209
therapy, 210
tonsil, 208

Urticarial vasculitis, 241–242
Uveitis. See also HLA B27 spondyloarthropa-

thy-associated uveitis
etanercept, 310
infectious (see Infectious uveitis)

Vascular disease

abdominal pain, 173
bilateral lower extremity swelling, 173
bilateral pulmonary artery aneurysms, 175
Budd-Chiari syndrome, 175
DVT, 172
hemoptysis, 174–175
PAA, 174
venous stasis ulcer, 172
venous thrombosis, 176

Vasculitis, 49–50. See also Cryoglobulinemic
vasculitis; Gastrointestinal vasculitis

giant cell arteritis (see Giant cell arteritis)
immunosuppressive therapy

cardiovascular, 97
central nervous system, 97
gastrointestinal, 97
infectious, 96

Takayasu arteritis (see Takayasu arteritis)
Venous thrombosis

diagnosis, 327
risk, 326–327
superficial thrombophlebitis, 329
symptoms and signs, 327
treatment, 327–328

Viral-related autoimmune diseases
hepatitis B virus (see Hepatitis B virus

hepatitis C virus (see Hepatitis C virus


Page 416

416 Index

human immunodeficiency virus, 355
pathogenesis, 345

Voltage-gated potassium channel
antibody (VGKCab)–associated
encephalitis, 367

Wegener granulomatosis, 293–294. See also

Antineutrophil cytoplasm antibody
(ANCA)-associated vasculitides

ANCA level, 281
hearing loss and tinitus, 279–280
prevalence, 279
saddle nose deformity, 279, 280
stenosis, 280–281
symptoms, 280
treatment, 281

West Nile encephalitis, 365–366

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