This kind of vasculitis delay diagnosis mortality rate is high, diagnostic ideas please put away ...

* For medical professionals to read and reference, it is another case that makes people "stupid and

unclear"

Case profile

In women 60+ years of age, there is no obvious trigger for bilateral lower extremity papulative purpura rash[1].

There are no other vasculitic manifestations
other than the rash.
With a previous history of seropositive rheumatoid arthritis, methotrexate 20 mg once a week and 200 mg hydroxychloroquine per day are used for treatment
.
Skin biopsy shows leukocytic vasculitis with isolated C3 deposition without IgG or IgM
in immunofluorescence.

Figure 1A: Papular purpura rash in patients

B: Skin biopsy results

Elevated inflammatory markers: erythrocyte sedimentation rate is 33 mm/h (normal value <20 mm/h), C-reactive protein (CRP) is 45 mg/L (normal value <5 mg/L), high titer rheumatoid factor (RF) is 447 IU/ml (normal value <20 IU/ml), low C4, <0.
07 g/L (normal value is 0.
13-0.
41 g/L).
<b10>

In addition, antinuclear antibodies, antiprotease 3 and antimyelin peroxidase antibodies, HIV, and hepatitis screening were negative
.

The doctor gives treatment
to rheumatoid vasculitis (a condition in which the skin manifests alone).
After 2 months the rash subsides and the patient begins to tape the prednisolone
used.

The rash has subsided, and what is the sudden fever and lung lesions? The rash subsides, hormones decrease, and just when the patient thinks that the drug has been "cured", new symptoms appear
.
When prednisolone was reduced to 10 mg per day, she began to develop symptoms of fever and drowsiness, which lasted for a full week, reaching a maximum of 39 ° C
.
However, carefully reviewing the various diseases that have occurred, there is no obvious source of infection
.

Doctors then gave the patient a sputum culture and found a species of Klebsiella pneumoniae, but other interferon γ release tests such as tuberculosis and infected serum were negative
.
The patient began to receive a variety of broad-spectrum antibiotics, but the condition did not improve
.
Even its clinical symptoms worsened: persistent fever, elevated inflammatory markers, and CRP up to 197 mg/L
.
However, after 1 week of taking antibiotics, CT scans of the chest, abdomen, and pelvis were normal
.

But this did not "pick out" the "real culprit" of the patient's fever
.
A second CT scan performed after another 1 week shows new hypoleft lobe vacuolar lesions (Figure 2
).

Figure 2: Patient CT result axis map shows new subpleural opacity in the left lower lobe, with central translucency, consistent
with 3.
6cm×3.
1 cm cavitation of thickened peripheral epidermis.

After another 1 week, a third interval CT scan performed showed a new infiltration of ground glass in the upper region on both sides (Figures 3 and 4).


Figure 3: CT view of the chest (A, axial; B, coronal) shows a decrease in the size of the thick-walled cavity lesion, measuring 2.
8 cm× 1.
6 cm, with small pleural effusions
on both sides.

Figure 4: CT view of the chest (A, axial direction; B, coronal) shows new bilateral anterior upper leaf hair glass infiltration
.

At this point, she developed a new purpuric rash in her right upper extremity, and another blood test showed that the RF titer had risen to 1208 IU/ml, with significant hypocomplementemia, C4<0.
07 g/L, C3<0.
43 g/L (normal, 0.
74–1.
57 g/L), suggesting cryoglobulin vasculitis<b120>.
Serum cryoglobulin is detected as IgMκ and polyclonal IgGκ paraproteins, which are consistent with type II cryoglobulinemia
.

The patient started oral prednisolone 1 mg/kg, and within 10 days, her clinical condition improved significantly, with RF titers and CRP dropping to 811 IU/ml and 28 mg/L
, respectively.

Repeated CT of the chest shows cavitary lesions and complete disappearance of the frosted glass-like shadow (Figure 5
).
The final diagnosis is cryoglobulin-like vasculitis
with pulmonary involvement.
She received intravenous rituximab 1 g in the week and 2nd week of definitive diagnosis, with a good clinical response, and methotrexate 25 mg/week with no recurrence of pulmonary symptoms at 4 months of follow-up
.

Figure 5: CT view of the chest shows that the previously seen clandation of the lower left lobe (A, axial direction; B, coronal) and the ingrown glass penetrates into the upper region (C, axial; D, coronal) resolution
.

What may be different from what some readers think, the patient did not have a lung biopsy because it was thought that the patient had a risk factor for a lung biopsy:

• Pulmonary parenchymal infiltrates are most likely to manifest as tissue pneumonia;
  
• Tissue pneumonia can manifest as serous glass infiltration or consolidation;
  
• Focal tissue pneumonia can present as nodules, lumps, or even vacuoles
  .
  Important differences in migratory multifocal peripheral sclerosis include tissue pneumonia, eosinophilic pneumonia, pulmonary hemorrhage, and pulmonary vasculitis
  .
  

How much do you know about cryoglobulinemia?

Cryoglobulinemia is a clinical condition characterized by the presence of cryoglobulin in the blood serum
.
Cryoglobulin is an immunoglobulin that precipitates in vitro at temperatures below normal body temperature (<37 °C) and re-dissolves on rewarming<b11>.
This observation was first reported
in 1933 in a patient with multiple myeloma.
The term "cryoglobulin" was coined in 1947, when cryoglobulins were discovered to be globulins that appear in various diseases [2].


There are three main types of cryoglobulinemia: type I and mixed cryoglobulinemia (type II and type III
).
Cryoglobulinemia can be asymptomatic or can cause disease
by causing vascular obstruction, hyperviscosity syndrome, or cryoglobulin vasculitis.

Table 1: Clinical findings
may suggest evaluation of cryoglobulinemia, which is a small to medium-sized vascular disease usually caused by lymphoproliferative disorders, paraproteinemia, viral infections (especially hepatitis C, HIV, and hepatitis B), and autoimmune diseases: including Sjögren's syndrome, systemic lupus erythematosus, and RA
.
Clinical manifestations can be varied, typically with purpura, arthralgia and weakness, and the Meltz triad
.
The case presents with unexplained fever, pulmonary infiltrates, and cavitation
.

Summary: Cryoglobulinemia
vasculitis is a challenging diagnosis because it requires a high index
of suspicion.
Certain infections can trigger transient cryoglobulinemia, most asymptomatic, e.
g.
, hepatitis B, HIV, cytomegalovirus, Coxley bain, parvovirus B19, tuberculosis, and brucellosis
.

Thus, in patients with cavitary pulmonary infiltrates, the real challenge is to determine whether the lesion is attributable to cryoglobulinemia vasculitis or cavitary lung infection, resulting in temporarily asymptomatic cryoglobulinemia
.
A high index of suspicion is key to making such a diagnosis, as delayed diagnosis may lead to increased
morbidity and mortality.

In this case, despite the use of broad-spectrum antibiotics, the patient's clinical deterioration and the evolution of lung infiltrates in a negative culture environment showed that the patient's infection was not a simple lung infection, and also helped doctors make further diagnoses
.

References:

[1] Adrian Mark Masnammany,Peter Kee Kee Wong et al.
Mixed cryoglobulinaemic vasculitis with pulmonary infiltrates and cavitation.
BMJ Case Rep 2022; 15:e251705.
doi:10.
1136/bcr-2022-251705.

[2] Muchtar E,Magen H,Gertz MA.
How I treat cryoglobulinemia.
Blood.
2017 Jan 19; 129(3):289-298.
doi:10.
1182/blood-2016-09-719773.
Epub 2016 Oct 31.
PMID:27799164.

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Source of this article: Medical Rheumatic Immunization Channel This article author: NaCl This article review: Chen Xinpeng Deputy Chief Physician Responsible Editor: Tangerine
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